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 Fourth
Edition

Atlas of
UNCOMMON
PAIN SYNDROMES
Steven D. Waldman, MD, JD
Vice Dean
Chairman and Professor
Department of Humanities and Bioethics
University of Missouri—Kansas City
Kansas City, Missouri
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899

ATLAS OF UNCOMMON PAIN SYNDROMES, FOURTH EDITION ISBN: 978-0-323-64077-0

Copyright © 2020 by Elsevier Inc. All rights reserved.

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Last digit is the print number: 9 8 7 6 5 4 3  2 1

 This book is dedicated to David Mayo
My little boy yesterday, my friend today, my son forever.
SDW
2019

WHAT OCCAM, KISS, ZEBRAS, AND MICKEY
GILLEY ALL HAVE IN COMMON
It has been said that the three most dangerous things in medicine
are: (1) a medical student with a sharp object; (2) a resident armed
with a recently published study from NEJM, and; (3) an attending
physician with an anecdote. One must suspect that #2 was at play
when in the 1940’s, while on rounds at the University of Mary-
land Hospital in Baltimore, Maryland, Theodore Woodward, MD,
stated that “If you hear hoof beats out on Green Street, don’t look for
zebras”! How this admonition to aspiring physicians morphed into
“when you hear hoof beats, look for horses, not zebras” is anybody’s
guess. (My son who was an ophthalmology resident in Baltimore
suggests that this sage piece of advice was most likely accompa-
nied by a long-winded and confusing anecdote—see #3 above).
On the surface, most of us would agree with Dr. Woodward’s
logic that the most common things are the most common. Occam
agreed, when in the 14th century he put forth the philosophical
tenant of parsimony that proposed that simpler explanations are, all
things being equal, almost always better than more complex ones.
He used a razor to “shave away” unnecessary or extraneous data
to get to the simplest solution. When you think about it, a razor
was all the rage as a medical instrument in the 14th century, so it is
not surprising that Occam chose it as his preferred medical device.
Occam’s razor certainly has a nice ring to it—better than Occam’s
MRI—which would no doubt be the name of his maxim if he had
lived in the 21st century, given that the MRI is certainly our most
current popular medical device for “shaving away” extraneous data.
Which brings us to KISS. Not the Gene Simmons’ rock band
KISS, but the admonition “Keep It Simple Stupid.” KISS was set
forth by Lockhead aeronautical engineer Kelly Johnson when he
handed his design team a few simple tools and challenged them
to design military jets that could be easily fixed with the simple
tools that were available in combat situations. It is still not exactly
clear to me who was “stupid,” but I certainly hope it is not the guys
who fix the jets I fly on. KISS makes sense when designing jet
engines, but one has to ask what KISS has to do with the individual
patient—the sick one—the scared one—the one you worry about
in the middle of the night. Unfortunately, very little. Because for
the individual patient with a difficult diagnosis, it turns out that a
guy named Harry Hickam was probably more correct than Occam.
While on teaching rounds at Duke University, Harry
Hickam, MD, admonished his students and residents that
“patients can have as many diseases as they damn well please”!
(See also #3 above.) He correctly posited that when diagnosing
the individual patient, using Occam’s razor often provides the
correct diagnosis. But more often than we would care to admit,
when dealing with a patient presenting with a perplexing con-
stellation of signs and symptoms, it can just as easily provide the
wrong one. In fact, overreliance on Occam’s razor can be down-
right dangerous for both the patient and physician.
Often the simplest or, in the case of medical diagnosis, the
most common illness is exactly what is causing the patient’s
P R E FA C E
symptoms. But sometimes, in our almost obsessive desire to
make the diagnosis, simplicity is our enemy. In our haste to
make the patient fit the diagnosis, we get it wrong. Uncommon
diseases are called uncommon diseases because they are uncom-
mon—they are not called unknown diseases (see below). Since
the beginning of time healers have recognized that the correct
diagnosis is the key to getting the patient well, and as a corol-
lary, they also realized that the wrong diagnosis is not a “practice
builder.” Which brings us to country music legend Mickey Gilley.
In 1976 Mickey Gilley recorded the classic country ballad “The
Girl’s All Get Prettier At Closing Time. (FYI, 12 straight weeks
charted at #1). This song is a plaintive lament about loneliness and
late-night desperation. It explores how one’s perception of things
can change as circumstances change. What turns an unknown and
undiagnosable disease into an uncommon disease is knowledge.
What changes our perception of what a constellation of symp-
toms and physical findings mean when we are confronted with
a sick patient with an elusive diagnosis is knowledge. As we gain
more clinical experience, things that were once unknown become
known—even commonplace. The more we hone our clinical
acumen, the easier it is to put the pieces together—the jumble of
disparate signs and symptoms come into focus—and then all of a
sudden, we have a diagnosis—a diagnosis we will never miss again!
The Atlas of Uncommon Pain Syndromes, Fourth Edition,
seeks to accomplish three things: The first is to familiarize the
clinician with a group of uncommon pain syndromes that occur
with enough frequency that they merit serious study—not rare
or orphan diseases—just uncommon ones that are often mis-
diagnosed. Second, this text is written with the goal of helping
the clinician reinforce his or her knowledge of common pain
syndromes to help in those situations when Occam is relatively
correct—when the pieces of the puzzle don’t quite fit the simple
diagnosis. The third goal is more about the clinician and a little
less about the patient. It is about what attracted many of us to
medicine to begin with. It is the irresistible charm of being pre-
sented with a difficult clinical problem and getting it right. What
a great feeling that is! I hope you enjoy reading Atlas of Uncom-
mon Pain Syndromes, Fourth Edition, as much as I did writing it.
Steven D. Waldman, MD, JD
PS: The following ad recently ran in a local Kansas newspaper.
Perhaps the hoof beats were zebras after all!
TAMED YOUNG MALE AND FEMALE ZEBRAS FOR
SALE
($2,000.00)
I have available young Male and Female zebra babies for sale to good
and lovely homes who knows about the tamed zebras, feel free to send
us emails for more details and pictures, please only serious inquiries.
Category: Pets »Horses
Ad ID: 1564632
Date: May 16, 2018
vii
 SECTION 1 Headache and Facial Pain Syndromes

1
Ice Pick Headache

ICD-10 CODE R51 TESTING

Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps to identify abnormalities that
CLINICAL SYNDROME may put the patient at risk for neurological disasters secondary
Ice pick headache is a constellation of symptoms consisting of to intracranial and brainstem pathological conditions, includ-
paroxysms of stabbing jabs and jolts that occur primarily in the ing tumors and demyelinating disease (Fig. 1.2). Magnetic
first division of the trigeminal nerve. These paroxysms of pain resonance angiography (MRA) also may be useful in helping
may occur as a single jab or a series of jabs that lasts for a frac- identify aneurysms, which may be responsible for the patient’s
tion of a second followed by relatively pain-free episodes. The neurological findings. In patients who cannot undergo MRI,
pain of ice pick headache occurs in irregular intervals of hours such as a patient with a pacemaker, computed tomography (CT)
to days. Similar to cluster headache, ice pick headache is an epi- is a reasonable second choice. Radionuclide bone scanning and
sodic disorder that is characterized by clusters of painful attacks plain radiography are indicated if fracture or bony abnormality,
followed by pain-free periods. Episodes of ice pick headache
usually occur on the same side, but in rare patients the pain may
move to the same anatomical region on the contralateral side.
Ice pick headache occurs more commonly in women and is gen-
erally not seen before the fourth decade of life, but rare reports
of children suffering from ice pick headache sporadically appear
in the literature. Synonyms for ice pick headache include jabs
and jolts headache, primary stabbing headache, ophthalmody-
nia periodica, and idiopathic stabbing headache.

SIGNS AND SYMPTOMS

A patient suffering from ice pick headache complains of jolts
or jabs of pain in the orbit, temple, or parietal region (Fig. 1.1).
Some patients describe the pain of ice pick headache as a sud-
den smack or slap on the side of the head. Similar to patients
suffering from trigeminal neuralgia, a patient suffering from ice
pick headache may exhibit involuntary muscle spasms of the
affected area in response to the paroxysms of pain. In contrast to
trigeminal neuralgia, involving the first division of the trigem-
inal nerve, there are no trigger areas that induce the pain of ice
pick headache. The neurological examination of a patient suf-
fering from ice pick headache is normal. Some patients exhibit
anxiety and depression because the intensity of pain associated
with ice pick headache leads many patients to believe they have Fig. 1.1 Ice pick headache is characterized by jabs or jolts in the orbit,
a brain tumor. temple, or parietal region.

1
2 SECTION 1 Headache and Facial Pain Syndromes
Fig. 1.2 Diffuse Pachymeningeal and Calvarial Metastasis From
Carcinoma of the Breast Axial T1-weighted postgadolinium MRI
shows diffuse nodular and bandlike contrast-enhanced thickening of the
dura over the high right frontoparietal convexity (arrow). (From Haaga
JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole
Body. 4th ed. Philadelphia: Mosby; 2003:198.)
such as metastatic disease, is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry should be performed if the diagnosis of ice pick head-
ache is in question. Intraocular pressure should be measured if
glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Ice pick headache is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic ice pick
headache include trigeminal neuralgia involving the first divi-
sion of the trigeminal nerve, demyelinating disease, and chronic
paroxysmal hemicrania. Trigeminal neuralgia involving the first
division of the trigeminal nerve is uncommon and is charac-
terized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological findings,
including optic neuritis and other motor and sensory abnor-
malities. The pain of chronic paroxysmal hemicrania lasts much
longer than the pain of ice pick headache and is associated with
redness and watering of the ipsilateral eye.
TREATMENT
Ice pick headache uniformly responds to treatment with indo-
methacin. Failure to respond to indomethacin puts the diag-
nosis of ice pick headache in question. A starting dosage of 25
mg daily for 2 days and titrating to 25 mg three times per day
is a reasonable treatment approach. This dose may be carefully
increased to 150 mg per day. Indomethacin must be used care-
fully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to
cyclooxygenase-2 (COX-2) inhibitors in the treatment of ice
pick headache have been noted in the headache literature.
Underlying sleep disturbance and depression are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to correctly diagnose ice pick headache may put the
patient at risk if intracranial pathological conditions or demy-
elinating disease, which may mimic the clinical presentation of
chronic paroxysmal hemicrania, is overlooked. MRI is indicated
in all patients thought to be suffering from ice pick headache.
Failure to diagnose glaucoma, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.
CLINICAL PEARLS The diagnosis of ice pick headache is made by
obtaining a thorough, targeted headache history. Patients suffering from ice
pick headache should have a normal neurological examination. If the results of
the neurological examination are abnormal, the diagnosis of ice pick headache
should be discarded and a careful search for the cause of the neurological
findings should be undertaken.
SUGGESTED READINGS
Cutrer FM, Boes CJ. Cough, exertional, and sex headaches. Neurol
Clin. 2004;22:133–149.
Dafer RM. Neurostimulation in headache disorders. Neurol Clin.
2010;28:835–841.
Loulwah O, Jan MMS. Primary stabbing “ice-pick” headache. Pediat-
ric Neurology. 2011;45(4):268.
Matthew NT. Indomethacin responsive headache syndromes: head-
ache. J Head Face Pain. 1981;21:147–150.
Pascual J. Other primary headaches. Neurol Clin. 2009;27:557–571.
Rampello L, Malaguarnera M, Rampello L, et al. Stabbing headache
in patients with autoimmune disorders. Clin Neurol Neurosurg.
2012;114(6):751–753.
Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exer-
tional and sexual headaches in a Turkish population of headache
patients. J Clin Neurosci. 2008;15:774–777.

ICD-10 CODE G50.0
CLINICAL SYNDROME
The pain of supraorbital neuralgia is characterized as persistent
pain in the supraorbital region and forehead with occasional
sudden, shock-like paresthesias in the distribution of the supra-
orbital nerves. Sinus headache involving the frontal sinuses,
which is much more common than supraorbital neuralgia, can
mimic the pain of supraorbital neuralgia. Supraorbital neuralgia
is the result of compression or trauma of the supraorbital nerves
as the nerves exit the supraorbital foramen. Such trauma can be
in the form of blunt trauma directly to the nerve, such as when
the forehead hits the steering wheel during a motor vehicle acci-
dent, or repetitive microtrauma resulting from wearing welding
or swim goggles that are too tight. This clinical syndrome also is
known as swimmer’s headache.
SIGNS AND SYMPTOMS
The supraorbital nerve arises from fibers of the frontal nerve,
which is the largest branch of the ophthalmic nerve. The fron-
tal nerve enters the orbit via the superior orbital fissure and
passes anteriorly beneath the periosteum of the roof of the
orbit. The frontal nerve gives off a larger lateral branch, the
supraorbital nerve, and a smaller medial branch, the supra-
trochlear nerve. Both exit the orbit anteriorly. The supraorbital
nerve sends fibers all the way to the vertex of the scalp and
provides sensory innervation to the forehead, upper eyelid,
and anterior scalp (Fig. 2.1). The pain of supraorbital neuralgia
is characterized as persistent pain in the supraorbital region
and forehead with occasional sudden, shock-like paresthesias
in the distribution of the supraorbital nerves. Occasionally, a
patient suffering from supraorbital neuralgia complains that
the hair on the front of the head hurts (Fig. 2.2). Supraorbital
nerve block is useful in the diagnosis and treatment of supra-
orbital neuralgia.
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
2
Supraorbital Neuralgia
may put the patient at risk for neurological disasters secondary
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 2.3). Magnetic
resonance angiography (MRA) also may be useful in helping
identify aneurysms, which may be responsible for the patient’s
neurological findings. In patients who cannot undergo MRI,
such as a patient with a pacemaker, computed tomography (CT)
is a reasonable second choice. Radionuclide bone scan, CT, and
plain radiography are indicated if sinus disease, fracture, or
bony abnormality such as metastatic disease is considered in
the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of supra-
orbital neuralgia is in question. Intraocular pressure should be
measured if glaucoma is suspected (Fig. 2.4).
DIFFERENTIAL DIAGNOSIS
Supraorbital neuralgia is a clinical diagnosis supported by a
combination of clinical history, normal physical examination,
radiography, CT, and MRI. Pain syndromes that may mimic
supraorbital neuralgia include ice pick headache, trigeminal
neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania.
Trigeminal neuralgia involving the first division of the trigemi-
nal nerve is uncommon and is characterized by trigger areas and
tic-like movements. Demyelinating disease is generally associ-
ated with other neurological findings, including optic neuritis
and other motor and sensory abnormalities. The pain of chronic
paroxysmal hemicrania lasts much longer than the paroxysmal
pain of supraorbital neuralgia and is associated with redness
and watering of the ipsilateral eye.
TREATMENT
The primary treatment intervention for supraorbital neuralgia is
the identification and removal of anything causing compression
of the supraorbital nerves (e.g., tight welding or swim goggles).
A brief trial of simple analgesics alone or in combination with
gabapentin also should be considered. For patients who do not
respond to these treatments, supraorbital nerve block with local
anesthetic and a steroid is a reasonable next step. Ultrasound
guidance for needle placement may be useful when performing
supraorbital nerve block.
3
4 SECTION 1 Headache and Facial Pain Syndromes
Inflamed
supraorbital n.
Fig. 2.1 The supraorbital nerve sends fibers all the way to the vertex
of the scalp and provides sensory innervation to the forehead, upper
eyelid, and anterior scalp. n., Nerve.
Fig. 2.2 Occasionally a patient with supraorbital neuralgia complains
that the hair on the front of the head hurts. The supraorbital nerve sends
fibers all the way to the vertex of the scalp and provides sensory inner-
vation to the forehead, upper eyelid, and anterior scalp.
To perform supraorbital nerve block, the patient is placed in
the supine position. Using a 10-mL sterile syringe, 3 mL of local
anesthetic is drawn up. When treating supraorbital neuralgia
with supraorbital nerve block, 80 mg of depot steroid is added
to the local anesthetic with the first block, and 40 mg of depot
steroid is added with subsequent blocks.
The supraorbital notch on the affected side is identified by
palpation. The skin overlying the notch is prepared with anti-
septic solution, with care taken to avoid spillage into the eye. A
25-gauge, 1½-inch needle is inserted at the level of the supraor-
bital notch and is advanced medially approximately 15 degrees
off the perpendicular to avoid entering the foramen. The needle
is advanced until it approaches the periosteum of the under-
lying bone (Fig. 2.5). A paresthesia may be elicited, and the
patient should be warned of such. The needle should not enter
the supraorbital foramen; if this occurs, the needle should be
withdrawn and redirected slightly more medially.
Because of the loose alveolar tissue of the eyelid, a gauze
sponge should be used to apply gentle pressure on the upper
eyelid and supraorbital tissues before injection of solution to
prevent the injectate from dissecting inferiorly into these tis-
sues. This pressure should be maintained after the procedure to
avoid periorbital hematoma and ecchymosis.
After gentle aspiration, 3 mL of solution is injected in a fan-
like distribution. If blockade of the supratrochlear nerve also
is desired, the needle is redirected medially and, after careful
aspiration, an additional 3 mL of solution is injected in a fanlike
manner. In rare cases, destruction of the supraorbital nerve by
radiofrequency lesioning or supraorbital nerve stimulation may
be required to provide long-lasting relief (Fig. 2.6).
Underlying sleep disturbance and depression associated
with the pain of supraorbital neuralgia are best treated with a
tricyclic antidepressant compound, such as nortriptyline. The
tricyclic antidepressant can be started at a single bedtime dose
of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose supraorbital neuralgia correctly may put
the patient at risk if an intracranial pathological condition or
demyelinating disease, which may mimic the clinical presenta-
tion of supraorbital neuralgia, is overlooked. MRI is indicated
in all patients thought to have supraorbital neuralgia. Failure to
diagnose glaucoma, which also may cause intermittent ocular
pain, may result in permanent loss of sight.
The forehead and scalp are highly vascular, and when per-
forming supraorbital nerve block the clinician should carefully
calculate the total milligram dosage of local anesthetic that may
be given safely, especially if bilateral nerve blocks are being per-
formed. This vascularity gives rise to an increased incidence of
postblock ecchymosis and hematoma formation. Despite the
vascularity of this anatomical region, this technique can be per-
formed safely in the presence of anticoagulation by using a 25-
or 27-gauge needle, albeit at increased risk for hematoma, if the
clinical situation dictates a favorable risk-to-benefit ratio. These
complications can be decreased if manual pressure is applied to
 CHAPTER 2 Supraorbital Neuralgia 5

A B

C
Fig. 2.3 Subdural Empyema in a Patient With Sinusitis (A) T2-weighted MRI shows high-signal-intensity
extraaxial fluid collection in the right frontal convexity and along the falx on the right side. (B and C) Gado-
linium-enhanced MRI shows extraaxial fluid collections in the right frontal convexity and along the falx with
intense peripheral enhancement. The signal intensity of the fluid collection is slightly higher than that of
cerebrospinal fluid. (From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body.
4th ed. Philadelphia: Mosby; 2003:209.)
6 SECTION 1 Headache and Facial Pain Syndromes

Fixed
semidilated
Hazy corneal reflex pupil
signifying edema
Cataractous
lens

Opaque thickened
edematous cornea
Cataractous lens
Shallow anterior
chamber

Fig. 2.4 Acute Angle Closure Resulting From an Intumescent Cataractous Lens The eye is red with a
hazy view of the anterior segment from corneal edema, with a fixed, irregular, semidilated pupil from iris
infarction. The slit image shows the corneal edema and a very shallow anterior chamber. Some uveitis may be
present because of ischemia, and this must be differentiated from the larger accumulations of lens material
and macrophages seen with phacolytic glaucoma. (From Spalton DJ, Hitchings RA, Hunter P. Atlas of Clinical
Ophthalmology. 3rd ed. London: Mosby; 2005:225.)

Supraorbital n.

Supraorbital notch

Fig. 2.6 A three-dimensional CT reconstruction, taken prior to the pro-

cedure, showing the position of the supraorbital foramen (arrow). (From
Huibin Q, Jianxing L, Guangyu H, et al. The treatment of first division
Fig. 2.5 Injection Technique for Relieving the Pain of Supraorbital
idiopathic trigeminal neuralgia with radiofrequency thermocoagulation
Neuralgia n., Nerve. (From Waldman SD. Atlas of Pain Management
of the peripheral branches compared to conventional radiofrequency.
Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007.)
J Clin Neurosci. 2009;16(11):1425–1429. ISSN 0967-5868, https://doi.
org/10.1016/j.jocn.2009.01.021.)

the area of the block immediately after injection. Application of
cold packs for 20-minute periods after the block also decreases
the amount of postprocedure pain and bleeding.
CLINICAL PEARLS Supraorbital nerve block is especially useful in the
diagnosis and palliation of pain secondary to supraorbital neuralgia. The first
step in the management of this unusual cause of headache is the correct fit-
ting of swimming goggles that do not compress the supraorbital nerves. Coex-
istent frontal sinusitis should be ruled out in patients who do not respond
rapidly to a change in swim goggles and a series of the previously mentioned
nerve blocks. Any patient with headaches severe enough to require neural
blockade as part of the treatment plan should undergo MRI of the head to rule
out unsuspected intracranial pathological conditions.
CHAPTER 2 Supraorbital Neuralgia 7
SUGGESTED READINGS
Hillerup S, Jensen RH, Ersbøll BK. Trigeminal nerve injury associated
with injection of local anesthetics: needle lesion or nneurotoxicity?
J Am Dental Assoc. 2011;142(5):531–539.
Levin M. Nerve blocks and nerve stimulation in headache disorders.
Tech Reg Anesth Pain Manage. 2009;13:42–49.
Levin M. Nerve blocks in the treatment of headache. Neurotherapeu-
tics. 2010;7:197–203.
Waldman SD. Swimmer’s headache. In: Waldman SD, ed. Atlas of
Pain Management Injection Techniques. Philadelphia: Saunders;
2007:7–10.
Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
view. Philadelphia: Saunders; 2009:15–17.
 3
Chronic Paroxysmal Hemicrania

may put the patient at risk for neurological disasters secondary

ICD-10 CODE R51
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 3.2). Magnetic res-
onance angiography (MRA) also may be useful in identifying
aneurysms, which may be responsible for the patient’s neuro-
CLINICAL SYNDROME logical findings. In patients who cannot undergo MRI, such
Chronic paroxysmal hemicrania, which is also known as as a patient with a pacemaker, computed tomography (CT) is
Sjaastad syndrome, shares many characteristics of its more a reasonable second choice. Radionuclide bone scanning and
common analogue, cluster headache, but has several important
differences (Table 3.1). Similar to cluster headache, chronic par-
oxysmal hemicrania is a severe, episodic, unilateral headache TABLE 3.1 Comparison of Cluster Headache
that affects the periorbital and retroorbital regions. In contrast and Chronic Paroxysmal Hemicrania
to cluster headache, which occurs 10 times more commonly
Chronic
in men, chronic paroxysmal hemicrania occurs primarily in
Paroxysmal
women (Fig. 3.1). The duration of pain associated with chronic
Comparison Factors Cluster Headache Hemicrania
paroxysmal hemicrania is shorter than that of cluster headache,
Gender predominance Male Female
lasting 5 to 45 minutes. This pain does not follow the chronobi-
Response to indomethacin Negative Positive
ological pattern seen in patients with cluster headache. Patients
Chronobiological pattern Positive Negative
with chronic paroxysmal hemicrania usually experience more Alcohol trigger Positive Negative
than five attacks per day. Chronic paroxysmal hemicrania uni- Length of attacks Longer Shorter
formly responds to indomethacin, whereas cluster headache Horner syndrome Present Present
does not.

SIGNS AND SYMPTOMS

During attacks of chronic paroxysmal hemicrania, patients
exhibit the following physical findings suggestive of Horner
syndrome on the ipsilateral side of the pain:
• Conjunctival and scleral injection
• Lacrimation
• Nasal congestion
• Rhinorrhea
• Ptosis of the eyelid
As in cluster headache, the patient may become agitated during
attacks, rather than seeking dark and quiet as does the patient
with migraine. In contrast to cluster headache, alcohol consump-
tion does not seem to trigger attacks of chronic paroxysmal hemi-
crania. Between attacks, the neurological examination of a patient
with chronic paroxysmal hemicrania should be normal.

TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents. Fig. 3.1 In contrast to cluster headache, which occurs primarily in men,
MRI is highly accurate and helps to identify abnormalities that chronic paroxysmal hemicrania occurs primarily in women.

8
 CHAPTER 3 Chronic Paroxysmal Hemicrania 9

plain radiography are indicated if fracture or bony abnormal-

ity such as metastatic disease is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
chronic paroxysmal hemicrania is in question. Intraocular pres-
sure should be measured if glaucoma is suspected.

DIFFERENTIAL DIAGNOSIS
Chronic paroxysmal hemicrania is a clinical diagnosis sup-
ported by a combination of clinical history, abnormal physi-
cal examination during attacks, radiography, and MRI. Pain
syndromes that may mimic chronic paroxysmal hemicrania
include cluster headache, trigeminal neuralgia involving the
A
first division of the trigeminal nerve, demyelinating disease,
and ice pick headache. Trigeminal neuralgia involving the first
division of the trigeminal nerve is uncommon and is charac-
terized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological find-
ings, including optic neuritis and other motor and sensory
abnormalities. The pain of cluster headache lasts much longer
than the pain of chronic paroxysmal hemicrania, and cluster
headache has a male predominance, a chronobiological pat-
tern of attacks, and a lack of response to treatment with indo-
methacin (Fig. 3.3).

B oxygenase-2 (COX-2) inhibitors in the treatment of chronic
Fig. 3.2 Sagittal (A) and semiaxial (B) T2-weighted images of a massive
prolactinoma in a 41-year-old man with chronic daily headache. (From
Benitez-Rosario MA, McDarby G, Doyle R, et al. Chronic cluster-like
headache secondary to prolactinoma: uncommon cephalalgia in asso-
ciation with brain tumors. J Pain Symptom Manage. 2009;37:271–276.)
TREATMENT
Chronic paroxysmal hemicrania uniformly responds to
treatment with indomethacin. Failure to respond to indo-
methacin puts the diagnosis of chronic paroxysmal hemicra-
nia in question. A starting dose of 25 mg daily for 2 days
and titrating to 25 mg three times per day is a reasonable
treatment approach. This dose may be carefully increased up
to 150 mg per day. Indomethacin must be used carefully, if
at all, in patients with peptic ulcer disease or impaired renal
function. Anecdotal reports of a positive response to cyclo-
paroxysmal hemicrania have been noted in the headache
literature. Underlying sleep disturbance and depression are
best treated with a tricyclic antidepressant compound, such
as nortriptyline, which can be started at a single bedtime
dose of 25 mg.

SUNCT Paroxysmal hemicrania Cluster headache

5 s-4 min 2-30 min 15-180 min Time

Overlap between duration Overlap between duration

Fig. 3.3 Overlap between attack duration in trigeminal autonomic cephalalgias. The duration of each trigeminal
autonomic cephalalgia is specified by the International Classification of Headache Disorders. (From Silber-
stein SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am.
2013;97(2):321–328.)
10 SECTION 1 Headache and Facial Pain Syndromes

TABLE 3.2 Characteristics of the Trigeminal SUGGESTED READINGS

Autonomic Cephalgias Benitez-Rosario MA, McDarby G, Doyle R, Fabby C. Chronic ­cluster-
• Unilateral like headache secondary to prolactinoma: uncommon cephalalgia in
• Short duration association with brain tumors. J Pain Symptom Manage. 2009;37:
• High frequency 271–276.
• Orbital, periorbital, or temporal
Benoliel R, Sharav Y. Paroxysmal hemicrania: case studies and review
• Associated autonomic symptoms
of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol
• Lacrimation
Endodontol. 1998;85:285–292.
• Conjunctival injection
Camarda C, Camarda R, Monastero R. Chronic paroxysmal hemicra-
• Nasal congestion
• Ptosis nia and hemicrania continua responding to topiramate: two case
• Eyelid edema reports. Clin Neurol Neurosurg. 2008;110:88–91.
Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalalgias.
II. Paroxysmal hemicrania. Oral Surg Oral Med Oral Pathol Oral
Radiol Endodontol. 2007;104:640–646.
COMPLICATIONS AND PITFALLS Schytz HW, Hargreaves R, Ashina M. Challenges in developing drugs
for primary headaches. Prog Neurobiol. 2017;152:70–88.
Failure to diagnose chronic paroxysmal hemicrania correctly
Sjaastad O. Chronic paroxysmal hemicrania: clinical aspects and con-
may put the patient at risk if intracranial pathological condi- troversies. In: Blau JN, ed. Migraine: Clinical, Therapeutic, Conceptu-
tions or demyelinating disease that may mimic the clinical pre- al and Research Aspects. London: Chapman & Hall; 1987:135–152.
sentation of chronic paroxysmal hemicrania is overlooked. MRI Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias
is indicated in all patients thought to have chronic paroxysmal other than cluster headache. Med Clin North Am. 2013;97(2):
hemicrania. Failure to diagnose glaucoma, which may cause 321–328.
intermittent ocular pain, may result in permanent loss of sight. Talvik I, Koch K, Kolk A, Talvik T. Chronic paroxysmal hemicrania in
a 3-year, 10-month-old female. Pediatr Neurol. 2006;34:225–227.
CLINICAL PEARLS Chronic paroxysmal hemicrania is classified as a
trigeminal autonomic cephalgia. The trigeminal autonomic cephalgias are a
group of distinct headache syndromes that share a number of common and
often overlapping clinical characteristics (Table 3.2). The diagnosis of chronic
paroxysmal hemicrania is made by obtaining a thorough, targeted headache
history. Between attacks, patients with chronic paroxysmal hemicrania should
have a normal neurological examination. If the neurological examination is
abnormal between attacks, the diagnosis of chronic paroxysmal hemicrania
should be discarded and a careful search for the cause of the patient’s neuro-
logical findings should be undertaken.
 4
Hemicrania Continua

ICD-10 CODE G44.51 TESTING

Magnetic resonance imaging (MRI) of the brain provides
the best information regarding the cranial vault and its con-
CLINICAL SYNDROME tents. MRI is highly accurate and helps identify abnormali-
Hemicrania continua is a primary headache disorder that is ties that may put the patient at risk for neurological disasters
classified as a trigeminal autonomic cephalgia that shares char- secondary to intracranial and brainstem pathological con-
acteristics of both cluster headache and migraine headache ditions, including tumors and demyelinating disease (Fig.
(Table 4.1). Similar to cluster headache, hemicrania continua 4.2). Magnetic resonance angiography (MRA) also may be
is a severe, unilateral headache with associated signs of auto- useful in identifying aneurysms, which may be responsi-
nomic dysfunction, including lacrimation, scleral injection, ble for the patient’s neurological findings. In patients who
eyelid ptosis, and nasal stuffiness syndrome (Fig. 4.1). In con- cannot undergo MRI, such as a patient with a pacemaker,
trast to cluster headache, which occurs 10 times more com- computed tomography (CT) is a reasonable second choice.
monly in men, hemicrania continua occurs more commonly in Radionuclide bone scanning and plain radiography are indi-
women, a characteristic it shares with migraine. Like migraine cated if fracture or bony abnormality such as metastatic
headache, hemicrania continua is associated with nausea and disease is considered in the differential diagnosis. Positron
vomiting, as well as sonophobia and phonophobia. Unlike the emission tomography may help further delineate and char-
pain of cluster and migraine headache, the pain of hemicrania acterize tumors responsible for the patient’s pain and neuro-
continua is continuous with intermittent severe exacerbations logical symptoms.
of pain. This pain is unilateral and is side locked (i.e., it does Screening laboratory tests consisting of complete blood cell
not change sides like migraine headache occasionally does). count, erythrocyte sedimentation rate, and automated blood
Hemicrania continua is an indomethacin responsive headache,
with complete resolution of headache and associated symptoms
with therapeutic doses of indomethacin. The cause of hemicra- TABLE 4.1 Comparison of Cluster Headache
nia continua is unknown, but like other trigeminal autonomic and Hemicrania Continua
cephalgias, functional magnetic resonance scanning and pos-
itron emission tomography reveal activation in the posterior Comparison Cluster Hemicrania Migraine
Factors Headache Continua Headache
hypothalamus during exacerbation of headaches.
Gender predominance Male Female Female
Absolute response to Negative Positive Negative
SIGNS AND SYMPTOMS indomethacin
Chronobiological Positive Negative Negative
During attacks of hemicrania continua, patients exhibit the fol-
pattern
lowing physical findings suggestive of Horner syndrome on the Alcohol trigger Positive Negative Sometimes
ipsilateral side of the pain: Length of attacks Longer Continuous, with Longer than cluster
• Conjunctival and scleral injection exacerbations headache
• Lacrimation Autonomic symptoms, Present Present Negative
• Nasal congestion lacrimation, scleral
• Rhinorrhea injection, eyelid pto-
• Ptosis of the eyelid sis, nasal stuffiness
As in cluster headache, the patient may become agitated syndrome
during attacks, rather than seeking dark and quiet as does the Photophobia Negative Positive Positive
Sonophobia Negative Positive Positive
patient with migraine. In contrast to cluster headache, alcohol
Nausea and vomiting Negative Positive Positive
consumption does not seem to trigger attacks of hemicrania
Continuous pain with Negative Positive Negative
continua. Between attacks, the neurological examination of a exacerbations
patient with hemicrania continua should be normal.
11
12 SECTION 1 Headache and Facial Pain Syndromes
Fig. 4.1 Hemicrania continua is a unilateral side-locked headache with associated signs of autonomic dys-
function. In contrast to cluster headache, which occurs primarily in men, hemicrania continua occurs primarily
in women.
chemistry testing should be performed if the diagnosis of hemi-
crania continua is in question. Intraocular pressure should be
measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Hemicrania continua is a clinical diagnosis supported by a
combination of clinical history, abnormal physical examina-
tion during exacerbation of baseline headache, radiography,
and MRI. Pain syndromes that may be confused for hemicra-
nia continua include cluster headache, chronic paroxysmal
hemicrania, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, ice pick headache,
and other indomethacin responsive headaches. Trigeminal
neuralgia involving the first division of the trigeminal nerve
is uncommon and is characterized by trigger areas and tic-
like movements. Demyelinating disease is generally associated
with other neurological findings, including optic neuritis and
other motor and sensory abnormalities. The pain of cluster
headache is episodic, whereas the pain of hemicrania conti-
nua is continuous with acute severe exacerbations (Fig. 4.3).
Cluster headache also has a male predominance, a chronobio-
logical pattern of attacks, and a lack of response to treatment
with indomethacin.
TREATMENT
Hemicrania continua uniformly responds to treatment with
indomethacin. Failure to respond to indomethacin puts the
diagnosis of hemicrania continua in question. A starting dose of
25 mg daily for 2 days and titrating to 25 mg three times per day
is a reasonable treatment approach. This dose may be carefully
increased up to 150 mg per day. Indomethacin must be used
carefully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to cyclo-
oxygenase-2 (COX-2) inhibitors in the treatment of hemicrania
continua have been noted in the headache literature. Underlying
sleep disturbance and depression are best treated with a tricyclic
antidepressant compound, such as nortriptyline, which can be
started at a single bedtime dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose hemicrania continua correctly may put the
patient at risk if intracranial pathological conditions or demy-
elinating disease, which may mimic the clinical presentation
of hemicrania continua, is overlooked. MRI is indicated in all
patients thought to have hemicrania continua. Failure to diag-
nose glaucoma, which may cause intermittent ocular pain, may
result in permanent loss of sight.
CLINICAL PEARLS Hemicrania continua is classified as a trigeminal
autonomic cephalgia. The trigeminal autonomic cephalgias are a group of
distinct headache syndromes that share a number of common and often over-
lapping clinical characteristics (Table 4.2). The diagnosis of hemicrania con-
tinua is made by obtaining a thorough, targeted headache history. Between
attacks, patients with hemicrania continua should have a normal neurological
examination. If the neurological examination is abnormal between attacks,
the diagnosis of hemicrania continua should be discarded and a careful search
for the cause of the patient’s neurological findings should be undertaken.
 CHAPTER 4 Hemicrania Continua 13

A B

C D
Fig. 4.2 A 57-Year-Old Woman With Recurrent GBM Postcontrast T1-weighted image (A) and rCBV map
(B) demonstrate two adjacent necrotic, peripherally enhancing lesions with elevated rCBV (white arrow) con-
sistent with recurrent GBM. After subtotal reresection of the peripheral lesion and 2 weeks of treatment
with TMZ and bevacizumab, postcontrast T1-weighted image (C) and rCBV map (D) show reduced size and
decreased intensity of enhancement of the residual medial necrotic rim-enhancing lesion with no significant
rCBV elevation (white arrow). Findings are consistent with response to treatment. The patient had a rela-
tively long OS (>17 months) after bevacizumab initiation. (From Boxerman JL, Shiroishi MS, Ellingson BM,
et al. Dynamic susceptibility contrast MR imaging in glioma: review of current clinical practice. Magn Reson
Imaging Clin N Am. 2016;24(4):649–670.) rCBV, Cerebral blood volume; GBM, glioblastoma multiforme; OS,
overall survival rate; TMZ, temozolomide.

SUNCT Paroxysmal Hemicrania Daily persistent

hemicrania continua headache

5 s–4 min 2–30 min 15–180 min Time

Continuous baseline headache Continuous

with acute exacerbations
Fig. 4.3 The Temporal Relationships of Trigeminal Autonomic Cephalalgias. (Modified from Silberstein
SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am.
2013;97(2):321–328.)
14 SECTION 1 Headache and Facial Pain Syndromes

TABLE 4.2 Characteristics of the Trigeminal SUGGESTED READINGS

Autonomic Cephalgias Benitez-Rosario MA, McDarby G, Doyle R, Fabby C. Chronic clus-
• Unilateral ter-like headache secondary to prolactinoma: uncommon cephal­
• Short duration algia in association with brain tumors. J Pain Symptom Manage.
• High frequency 2009;47:271–276.
• Orbital, periorbital, or temporal Evans RW. Case studies of uncommon and rare headache disorders.
• Associated autonomic symptoms Neurol Clin. 2016;34(3):631–650.
• Lacrimation Jay GW, Barkin RL. Primary headache disorders, Part I: migraine and
• Conjunctival injection the trigeminal autonomic cephalalgias. Dis Mon. 2017;63(11):308–
• Nasal congestion 338.
• Ptosis Schytz HW, Hargreaves R, Ashina M. Challenges in developing drugs
• Eyelid edema for primary headaches. Prog Neurobiol. 2017;152:70–88.
Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias oth-
er than cluster headache. Med Clin North Am. 2014;97(2):421–428.

ICD-10 CODE G50.0
CLINICAL SYNDROME
Charlin syndrome, also known as nasociliary neuralgia and
Charlin-Sluder cephalgia, is an uncommon cause of head and
face pain. As with most headache syndromes, the exact cause
of the pain of Charlin syndrome is unknown. However, the
pathogenesis of this uncommon cause of head and face pain is
thought to be dysfunction of the nasociliary ganglion in a man-
ner analogous to the dysfunction of the sphenopalatine gan-
glion thought to be the source of cluster headache. The pain of
Charlin syndrome has a rapid onset to peak, with attacks lasting
45 to 60 minutes. In some patients, these attacks can be trig-
gered by sensory stimulation of the affected areas. Although in
many ways similar to cluster headache (e.g., retroorbital loca-
tion of pain, profuse unilateral rhinorrhea, rapid onset to peak,
and short duration of attacks), many dissimilarities also exist.
In contrast to cluster headache, alcohol consumption does not
appear to trigger attacks of Charlin syndrome and the seasonal
and chronobiological patterns so characteristic of cluster head-
ache do not seem to be a factor (Table 5.1). Blockade of the
sphenopalatine ganglion, which is so effective in the treatment
of cluster headache, is of little value in the treatment of Charlin
syndrome. Patients suffering from Charlin syndrome uniformly
respond to daily nasociliary nerve blocks with local anesthetic,
as described subsequently.
SIGNS AND SYMPTOMS
Patients suffering from Charlin syndrome present with the
complaint of severe paroxysms of ocular or retroorbital pain
that radiates into the ipsilateral forehead, nose, and maxillary
region. This pain is associated with voluminous ipsilateral rhi-
norrhea and congestion of the nasal mucosa and significant
inflammation of the affected eye (Fig. 5.1).
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters secondary
5
Charlin Syndrome
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 5.2). Magnetic
resonance angiography (MRA) also may be useful in helping
identify aneurysms, which may be responsible for the patient’s
neurological findings. In patients who cannot undergo MRI,
such as a patient with a pacemaker, computed tomography (CT)
is a reasonable second choice. Radionuclide bone scanning and
plain radiography are indicated if fracture or bony abnormal-
ity such as metastatic disease is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
Charlin syndrome is in question. Intraocular pressure should
be measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Charlin syndrome is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic Charlin
syndrome include cluster headache, temporal arteritis, trigemi-
nal neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania
(see Table 5.1). Trigeminal neuralgia involving the first division
of the trigeminal nerve is uncommon and is characterized by
trigger areas and tic-like movements. Demyelinating disease is
generally associated with other neurological findings, including
optic neuritis and other motor and sensory abnormalities. The
pain of chronic paroxysmal hemicrania lasts much longer than
the pain of Charlin syndrome.
TREATMENT
The treatment of Charlin syndrome is analogous to the treat-
ment of trigeminal neuralgia. The use of anticonvulsants such as
carbamazepine and gabapentin represents a reasonable starting
point. High-dose steroids tapered over 10 days also have been
anecdotally reported to provide relief. For patients who do not
respond to the previously mentioned treatments, daily nasocil-
iary ganglion block with local anesthetic and steroid is a rea-
sonable next step. Underlying sleep disturbance and depression
associated with the pain of supraorbital neuralgia are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
15
16 SECTION 1 Headache and Facial Pain Syndromes

TABLE 5.1 Comparison of Cluster Headache

and Charlin Syndrome
Cluster Charlin
Comparison Factors Headache Syndrome
Ocular and retroorbital location Yes Yes
Unilateral Yes Yes
Rapid onset to peak Yes Yes
Severe intensity Yes Yes
Attacks occur in paroxysms Yes Yes
Duration of attacks short Yes Yes
Pain free between attacks Yes Yes
Significant rhinorrhea during attacks Yes Yes
Alcohol triggers attacks Yes No
Tactile trigger areas No Yes
Seasonal pattern of attacks Yes No
Chronobiological pattern of attacks Yes No
Significant eye inflammation No Yes
Responds to sphenopalatine ganglion Yes No Fig. 5.2 Multiple Sclerosis Fluid-attenuated inversion recovery (FLAIR)
block parasagittal MR image depicts the extensive demyelinated plaques of
Responds to nasociliary block No Yes progressive multiple sclerosis. (From Haaga JR, Lanzieri CF, Gilkeson
RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia:
Mosby; 2003:466.)

COMPLICATIONS AND PITFALLS

Failure to diagnose Charlin syndrome correctly may put the
patient at risk if an intracranial pathological condition or demy-
elinating disease, which may mimic the clinical presentation
of Charlin syndrome, is overlooked. MRI is indicated in all
patients thought to have Charlin syndrome. Failure to diagnose
glaucoma or temporal arteritis, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.

CLINICAL PEARLS Nasociliary nerve block via the medial orbital

approach is especially useful in the diagnosis and palliation of pain secondary
to Charlin syndrome. Given the uncommon nature of this headache syndrome
and its overlap with the symptoms of cluster headache and other neurological
problems, including cavernous sinus thrombosis and intracranial and retro-
orbital tumors, Charlin syndrome must remain a diagnosis of exclusion. All
patients suspected to have Charlin syndrome require MRI of the brain with and
without gadolinium contrast material and thorough ophthalmological and neu-
rological evaluation. Nasociliary nerve block via the medial orbital approach
should be performed only by clinicians familiar with the regional anatomy.

Fig. 5.1 Patients suffering from Charlin syndrome present with the
complaint of severe paroxysms of ocular or retroorbital pain that radi-
ates into the ipsilateral forehead, nose, and maxillary region. The pain is
associated with voluminous ipsilateral rhinorrhea and congestion of the
nasal mucosa and significant inflammation of the affected eye.
SUGGESTED READINGS
Becker M, Kohler R, Vargas MI, Viallon M, Delavelle J. Pathology of
the trigeminal nerve. Neuroimaging Clin N Am. 2008;18:283–307.
Craven J. Anatomy of the cranial nerves. Anaesth Intensive Care Med.
2010;11:529–534.
Lewis DW, Gozzo YF, Avner MT. The “other” primary headaches in
children and adolescents [review]. Pediatr Neurol. 2005;33:303–313.
Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
view. Philadelphia: Saunders; 2009:15–17.
Waldman SD. Charlin’s syndrome. In: Waldman SD, ed. Atlas of
Pain Management Injection Techniques. Philadelphia: Saunders;
2007:20–24.
 6
Sexual Headache

ICD-10 CODE R51
THE CLINICAL SYNDROME
Sexual headache, which is also known as primary headache
associated with sexual activity, is a term used to describe a
group of headaches associated with sexual activity. Clinicians
have identified the following three general types of headache
associated with sexual activity:
• Explosive type
• Dull type
• Postural type
Each of these sexual headache types was previously called
benign coital headache, but this term has been replaced by sex-
ual headache because each may occur with sexual activity other
than coitus (Fig. 6.1). In general, sexual headache includes a
benign group of disorders, but a rare patient may have acute
subarachnoid hemorrhage during sexual activity, which may be
erroneously diagnosed as the benign explosive type of sexual
headache. There is no gender predilection for sexual headache,
and the occurrence of all types of sexual headache may be epi-
sodic rather than chronic. Rarely, more than one type of sexual
headache occurs in the same patient. Sexual headaches have
been associated with the use of cannabis, pseudoephedrine, oral
contraceptives, and amiodarone.
occipital, but some patients volunteer that the pain felt “like the
top of my head was going to blow off.” The pain is usually bilat-
eral, but isolated cases of unilateral explosive sexual headache
have been reported. The pain usually remains intense for 10 to
15 minutes and then gradually abates. Some patients note some
residual headache pain for 2 days.
Dull Type of Sexual Headache
The dull type of sexual headache begins during the early por-
tion of sexual activity. This headache type has an aching char-
acter and begins in the occipital region. The headache becomes
holocranial as sexual activity progresses toward orgasm. It may
peak at orgasm, but in contrast to the explosive type of sexual
headache, the dull type disappears rapidly after orgasm. Ceasing
sexual activity usually aborts the dull type of sexual headache.
Some headache specialists think the dull type of sexual head-
ache is simply a milder version of the explosive type of sexual
headache.
Postural Type of Sexual Headache
The postural type of sexual headache is similar to the explosive
type of sexual headache in that it occurs just before or during
orgasm. Its rapid onset to peak and severe intensity also are sim-
ilar to that of the explosive type. It differs from the explosive
type of headache in that the headache symptoms recur when the

SIGNS AND SYMPTOMS

Patients with sexual headache present differently depending on
the type of sexual headache experienced. Each clinical presen-
tation is discussed subsequently.

Explosive Type of Sexual Headache

The explosive type of sexual headache is the most common type
of sexual headache encountered in clinical practice. The patient
usually fears he or she has had a stroke. The patient may be less
forthcoming about the circumstances surrounding the onset of
headache, and tactful questioning may be required to ascertain
the actual clinical history. The explosive type of sexual headache
occurs suddenly, with an almost instantaneous onset to peak
just before or during orgasm. The intensity of the explosive type
of sexual headache is severe and has been likened to the pain of Fig. 6.1 Sexual headaches show no gender predilection and are gen-
acute subarachnoid hemorrhage. The location of pain is usually erally benign.

17
18 SECTION 1 Headache and Facial Pain Syndromes

Fig. 6.2 Brain magnetic resonance angiography performed 1 month

after sexual headache onset showing multifocal vasoconstriction in
the left posterior cerebral artery (arrows). (From Hu CM, Lin YJ, Fan
YK, et al. Isolated thunderclap headache during sex: orgasmic head-
ache or reversible cerebral vasoconstriction syndrome? J Clin Neu-
rosci. 2010;17[10]:1349–1351, fig. 1, ISSN 0967-5868, https://doi.
org/10.1016/j.jocn.2010.01.052. http://www.sciencedirect.com/science/ A
article/pii/S0967586810002419.)

patient stands up, in a manner analogous to postdural puncture

headache. The postural component of this type of sexual head-
ache is thought to be due to minute tears in the dura that may
occur during intense sexual activity.

TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters secondary
to intracranial and brainstem pathological conditions, including
tumors, demyelinating disease, and hemorrhage. More impor-
tantly, MRI helps identify bleeding associated with leaking intra-
cranial aneurysms. Magnetic resonance angiography (MRA)
and cerebral arteriography may be useful in helping identify
aneurysms or other arterial abnormalities responsible for the
patient’s neurological symptoms (Figs 6.2 and 6.3). In patients B
who cannot undergo MRI, such as patients with pacemakers,
Fig. 6.3 Cerebral angiogram revealed segmental (boundaries denoted
computed tomography (CT) is a reasonable second choice. Even
by large arrows) irregularities (small arrows) of the basilar artery in
if blood is not present on MRI or CT, if intracranial hemorrhage both anterior-posterior (A) and lateral (B) views. (From Delasobera BE,
is suspected, lumbar puncture should be performed. Osborn SR, Davis JE. Thunderclap headache with orgasm: a case of
Screening laboratory tests consisting of complete blood cell basilar artery dissection associated with sexual intercourse. J Emerg
count, erythrocyte sedimentation rate, and automated blood Med. 2012;43[1]:e43–e47, fig. 1.)
chemistry testing should be performed if the diagnosis of sexual
headache is in question. Intraocular pressure should be mea- cluster headache, migraine, and chronic paroxysmal hemi-
sured if glaucoma is suspected. crania. Trigeminal neuralgia involving the first division of the
trigeminal nerve is uncommon and is characterized by trigger
areas and tic-like movements. Demyelinating disease is gener-
DIFFERENTIAL DIAGNOSIS ally associated with other neurological findings, including optic
Sexual headache is a clinical diagnosis supported by a combi- neuritis and other motor and sensory abnormalities. The pain of
nation of clinical history, normal physical examination, radi- chronic paroxysmal hemicrania and cluster headache is associ-
ography, MRI, and MRA. Pain syndromes that may mimic ated with redness and watering of the ipsilateral eye, nasal con-
sexual headache include trigeminal neuralgia involving the gestion, and rhinorrhea during the headache. These findings are
first division of the trigeminal nerve, demyelinating disease, absent in all types of sexual headache. Migraine headache may

or may not be associated with nonpainful neurological findings
known as aura, but the patient almost always reports some sys-
temic symptoms, such as nausea or photophobia, not typically
associated with sexual headache.
TREATMENT
It is generally thought that avoiding the inciting activity for a
few weeks decreases the propensity to trigger sexual headaches.
If this avoidance technique fails or is impractical because of
patient preference, a trial of propranolol is a reasonable next
step. A low dose of 20 to 40 mg as a daily dose and titrating
in 20-mg increments to 200 mg as a divided daily dose until
prophylaxis occurs treats most patients suffering from sexual
headache. Propranolol should be used with caution in patients
with asthma or cardiac failure and patients with brittle diabetes.
If propranolol is ineffective, indomethacin may be tried. A
starting dose of 25 mg daily for 2 days and titrating to 25 mg
three times per day is a reasonable treatment approach. This dose
may be carefully increased to 150 mg per day. Indomethacin
must be used carefully, if at all, in patients with peptic ulcer dis-
ease or impaired renal function. Anecdotal reports of a positive
response to cyclooxygenase-2 (COX-2) inhibitors and magne-
sium in the treatment of sexual headache have been noted in the
headache literature. Underlying sleep disturbance and depres-
sion are best treated with a tricyclic antidepressant compound,
such as nortriptyline, which can be started at a single bedtime
dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose sexual headache correctly may put the
patient at risk if intracranial pathology or demyelinating
disease, which may mimic the clinical presentation of sexual
CHAPTER 6 Sexual Headache 19
headache, is overlooked. MRI, MRA, and occasionally cerebral
angiography are indicated in all patients thought to have sex-
ual headache. Failure to diagnose glaucoma, which also may
cause intermittent ocular pain, may result in permanent loss
of sight.
CLINICAL PEARLS The diagnosis of sexual headache is made by obtain-
ing a thorough, targeted headache history. As mentioned earlier, patients may
not be forthcoming about the events surrounding the onset of their headache,
and the clinician should be sensitive to this fact. Patients suffering from sexual
headache should have a normal neurological examination. If the neurological
examination is abnormal, the diagnosis of sexual headache should be dis-
carded and a careful search for the cause of the patient’s neurological findings
should be undertaken.
SUGGESTED READINGS
Delasobera BE, Osborn SR, Davis JE. Thunderclap headache with
orgasm: a case of basilar artery dissection associated with sexual
intercourse. J Emerg Med. 2012;43(1):e43–e47.
Evans RW. Diagnostic testing for migraine and other primary head-
aches. Neurol Clin. 2009;27:393–415.
Hu CM, Lin YJ, Fan YK, et al. Isolated thunderclap headache during
sex: orgasmic headache or reversible cerebral vasoconstriction
syndrome? J Clin Neurosci. 2010;17:1349–1351.
Jolobe OMP. The differential diagnosis includes reversible cerebral
vasoconstrictor syndrome. Am J Emerg Med. 2010;28:637.
Kim HJ, Seo SY. Recurrent emotion-triggered headache following
primary headache associated with sexual activity. J Neurol Sci.
2008;273:142–143.
Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exer-
tional and sexual headaches in a Turkish population of headache
patients. J Clin Neurosci. 2008;15:774–777.
 7
Cough Headache
ICD-10 CODE R51
THE CLINICAL SYNDROME
Cough headache is a term used to describe headaches triggered
by coughing and other activities associated with a Valsalva
maneuver, such as laughing, straining at stool, lifting, and bend-
ing the head toward the ground (Fig. 7.1). Clinicians have iden-
tified the following two types of cough headache:
• Benign primary cough headache
• Symptomatic cough headache
Initially, both types of cough headache were thought to be
related to sexual and exertional headaches, but they are now
considered distinct clinical entities. A strong male predilection
is seen for benign cough headache and no gender predilection
for symptomatic cough headache.
SIGNS AND SYMPTOMS
Patients suffering from cough headache present differently
depending on the type of cough headache experienced. Each
clinical presentation is discussed.
Benign Cough Headache
Benign cough headache is not associated with obvious neuro-
logical or musculoskeletal disease. More than 80% of patients
with benign cough headache are males, in contradistinction to
symptomatic cough headache, in which no gender predilection
is seen. The onset of benign cough headache is abrupt, occur-
ring immediately after coughing or other activities that cause
a Valsalva maneuver. Although the intensity of pain is severe
and peaks rapidly, it lasts only seconds to minutes. The charac-
ter of the pain associated with benign cough headache is split-
ting or sharp, and the pain is in the occipital region bilaterally
and occasionally the vertex of the skull. No accompanying neu-
rological or systemic symptoms are seen, as with cluster and
migraine headaches. The age of onset of benign cough head-
ache is generally in the late fifth or sixth decade of life. If such
headaches occur before age 50, there should be strong clinical
suspicion that the patient either has symptomatic cough head-
ache or a pathological condition in the posterior fossa, such as
Arnold-Chiari malformation or tumor. Tumors of the foramen
20
magnum also may mimic the presentation of benign cough
headache even if no neurological symptoms are present.
Symptomatic Cough Headache
Symptomatic cough headache is almost always associated with
structural abnormalities of the cranium, such as Arnold-Chiari
malformation I and II or intracranial tumors (Fig. 7.2). The
symptoms associated with symptomatic cough headache are
thought to be due to herniation of the cerebellar tonsil through
the foramen magnum into the space normally occupied by the
upper portion of the cervical spinal cord. Similar to benign
cough headache, the onset of pain associated with symptomatic
cough headache is abrupt, occurring immediately after cough-
ing or other activities that cause a Valsalva maneuver. Although
the intensity of pain is severe and peaks rapidly, it lasts only
seconds to minutes. In contrast to benign cough headache,
associated neurological symptoms may be present, including
difficulty swallowing, faintness, and numbness in the face and
upper extremities. These associated symptoms should be taken
very seriously because they are indicative of increased intracra-
nial pressure and herniation of the intracranial contents.
The character of the pain associated with symptomatic cough
headache is splitting or sharp, and pain is in the occipital region
bilaterally and occasionally the vertex of the skull. The age of
onset of symptomatic cough headache is generally in the third
decade of life, although, depending on the amount of neurolog-
ical compromise, it may occur at any age. In contrast to benign
cough headache, which occurs predominantly in men, symp-
tomatic cough headache occurs with equal prevalence in both
genders.
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters second-
ary to intracranial and brainstem pathological conditions,
including tumors and demyelinating disease. Special attention
to the foramen magnum may help identify more subtle abnor-
malities responsible for posterior fossa neurological signs and
symptoms. MRI helps identify bleeding associated with leaking
intracranial aneurysms, which may mimic the symptoms of
both types of cough headache. Magnetic resonance angiography

Herniation of
cerebellar tonsil
Spinal cord
Fig. 7.1 Symptomatic cough headache is often associated with structural abnormalities, such as Arnold-
Chiari malformation, and usually occurs in the third decade of life.
Fig. 7.2 Low-lying cerebellar tonsils (straight arrows) of a Chiari mal-
formation are shown deforming the medulla (curved arrow) in a sagit-
tal T1-weighted spin echo image. 4, Fourth ventricle. (From Stark DD,
Bradley WG Jr, eds. Magnetic Resonance Imaging. 3rd ed. St Louis:
Mosby; 1999:1841.)
CHAPTER 7 Cough Headache 21
(MRA) may be useful in helping identify aneurysms responsi-
ble for the patient’s neurological symptoms. In patients who
cannot undergo MRI, such as patients with pacemakers, com-
puted tomography (CT) is a reasonable second choice. Lumbar
puncture should be performed if intracranial hemorrhage is
suspected, even if blood is not present on MRI or CT. Plain
radiographs of the cervical spine also may be useful in the eval-
uation of Arnold-Chiari malformations and should be included
in the evaluation of all patients with cough headache.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of cough
headache is in question. Intraocular pressure should be mea-
sured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Cough headache is a clinical diagnosis supported by a combina-
tion of clinical history, physical examination, radiography, MRI,
and MRA. Pain syndromes that may mimic cough headache
include benign exertional headache, ice pick headache, sexual
headache, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, cluster headache,
and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
ments. Demyelinating disease is generally associated with other
neurological findings, including optic neuritis and other motor
and sensory abnormalities. The pain of chronic paroxysmal
hemicrania and cluster headache is associated with redness and
watering of the ipsilateral eye, nasal congestion, and rhinorrhea
during the headache. These findings are absent in all types of
22 SECTION 1 Headache and Facial Pain Syndromes

cough headache. Migraine headache may or may not be associ-
ated with painless neurological findings known as aura, but the
patient almost always reports some systemic symptoms, such
as nausea or photophobia, not typically associated with cough
headache.
TREATMENT
Indomethacin is the treatment of choice for benign cough head-
ache. A starting dose of 25 mg daily for 2 days and titrating to
25 mg three times per day is a reasonable treatment approach.
This dose may be carefully increased up to 150 mg per day.
Indomethacin must be used carefully, if at all, in patients with
peptic ulcer disease or impaired renal function. Headache spe-
cialists have noted anecdotal reports of a positive response to
cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign
cough headache. Underlying sleep disturbance and depression
are best treated with a tricyclic antidepressant compound, such
as nortriptyline, which can be started at a single bedtime dose
of 25 mg.
The only uniformly effective treatment for symptomatic
cough headache is surgical decompression of the foramen mag-
num. This surgery is usually done via suboccipital craniectomy.
Surgical decompression prevents the low-lying cerebellar ton-
sils from obstructing the flow of spinal fluid from the cranium
to the spinal subarachnoid space during a Valsalva maneuver.
COMPLICATIONS AND PITFALLS
Failure to diagnose cough headache correctly may put the
patient at risk if intracranial pathology or demyelinating dis-
ease, which may mimic the clinical presentation of cough head-
ache, is overlooked. MRI and MRA are indicated in all patients
thought to have cough headache. Failure to diagnose glaucoma,
which also may cause intermittent ocular pain, may result in
permanent loss of sight.
SUGGESTED READINGS
Berciano J, Poca M-A, García A, Sahuquillo J. Paroxysmal cervi-
cobrachial cough-induced pain in a patient with syringomy-
elia extending into spinal cord posterior gray horns. J Neurol.
2007;54:678–681.
Chen YY, Lirng JF, Fuh JL, et al. Primary cough headache is associat-
ed with posterior fossa crowdedness: a morphometric MRI study.
Cephalalgia. 2004;24:694–699.
Rothrock JF. Headaches caused by vascular disorders. Neurol Clin.
2014;32(2):305–319.
Cutrer M, DeLange FJ. Cough, exercise, and sex headaches. Neurol
Clin. 2014;32(2):433–450.
Langridge B, Phillips E, Choi D. Chiari malformation type 1: a sys-
tematic review of natural history and conservative management.
World Neurosurg. 104:213–219
Pascual J, Rubén Martín A, Oterino A. Headaches precipitated by
cough, prolonged exercise or sexual activity: a prospective etiolog-
ical and clinical study. J Headache Pain. 2008;9:259–266.
Pascual J. Primary cough headache. Curr Pain Headache Rep.
2005;9:272–276.
Pascual J. Cough and exertional headache, primary. In: Encyclopedia
of the Neurological Sciences. 2nd ed. Oxford: Academic Press;
2014:881–884.
Waldman SD. Arnold Chiari malformation type I. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:27–28.
Waldman SD. Arnold Chiari malformation type II. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:29–30.

CLINICAL PEARLS Any patient presenting with headaches associated

with exertion or Valsalva maneuver should be taken very seriously. Although
statistically most of these headaches ultimately are proved to be of benign
cause, a few patients have potentially life-threatening disease. The diagno-
sis of cough headache is made by obtaining a thorough, targeted headache
history and performing a careful physical examination. The clinician must sep-
arate patients suffering from benign cough headache from patients suffering
from symptomatic cough headache. Patients with benign cough headache
should have a normal neurological examination. If the neurological examina-
tion is abnormal, the diagnosis of benign cough headache should be discarded
and a careful search for the cause of the patient’s neurological findings should
be undertaken.

Sudden Unilateral Neuralgiform
Conjunctival Injection
ICD-10 CODE G50.0
THE CLINICAL SYNDROME
Sudden unilateral neuralgiform conjunctival injection tearing
(SUNCT) headache is an uncommon primary headache disor-
der that is one of a group of three headache syndromes known
as the trigeminal autonomic cephalgias (Table 8.1). Whether
SUNCT headache is in fact a distinct headache entity or simply
a constellation of symptoms that occurs on a continuum along
with the other trigeminal autonomic cephalgias is a point of
ongoing debate among headache and pain management spe-
cialists (Fig. 8.1). As with most headache syndromes, the exact
cause of the pain of SUNCT headache is unknown; however, the
pathogenesis of this uncommon cause of head and face pain is
thought to be dysfunction of the trigeminal autonomic reflex.
The pain of SUNCT headache has a rapid onset to peak,
with attacks lasting 5 seconds to 4 minutes and the frequency
of attacks ranging from 20 to 200 attacks per day. In some
patients, these attacks can be triggered by sensory stimulation
of the affected areas, such as when washing the face, brushing
the teeth, and so forth. Although in many ways similar to clus-
ter headache (e.g., unilateral, periorbital, and frontal location
of pain, sclera injection, rapid onset to peak, short duration of
attacks, and pain-free periods between attacks), SUNCT exhib-
its many dissimilarities as well. In contrast to cluster head-
ache, alcohol consumption does not seem to trigger attacks of
SUNCT headache, and there do not seem to be the seasonal
and chronobiological patterns so characteristic of cluster head-
ache, although SUNCT headache occurs most frequently in the
morning and afternoon (Table 8.2).
Blockade of the sphenopalatine ganglion, which is so effec-
tive in the treatment of cluster headache, is of little value in the
treatment of SUNCT headache. Patients suffering from SUNCT
headache may respond to daily trigeminal nerve blocks with
local anesthetic, as described subsequently.
SIGNS AND SYMPTOMS
Patients with SUNCT headache present with the complaint
of severe paroxysms of ocular or periorbital pain that radi-
ate into the ipsilateral temple, forehead, nose, cheek, throat,
8
Tearing Headache
and maxillary region. This pain is associated with significant
inflammation of the affected eye and associated autonomic
signs and symptoms (Figs 8.2 and 8.3). The pain is neural-
giform and severe to excruciating in intensity (Table 8.3).
SUNCT occurs on the right side 70% of the time in a manner
analogous to trigeminal neuralgia. Like trigeminal neuralgia,
rare cases of bilateral SUNCT headache have been reported.
Also, like trigeminal neuralgia, the pain of SUNCT headache
rarely switches sides. SUNCT headache occurs slightly more
frequently in males. It can occur at any age, with a peak inci-
dence in the fifth decade.
TESTING
Magnetic resonance imaging (MRI) of the brain provides
the clinician with the best information regarding the cra-
nial vault, vasculature, and its contents (Fig. 8.4). MRI is
highly accurate and helps identify abnormalities that may
put the patient at risk for neurological disasters secondary to
intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease. Magnetic resonance
angiography (MRA) also may be useful in helping iden-
tify aneurysms, which may be responsible for the patient’s
TABLE 8.1 Trigeminal Autonomic
Cephalgias
Cluster headache
Chronic paroxysm hemicranias
Sudden unilateral neuralgiform conjunctival injection tearing headache
Paroxysmal Cluster
SUNCT hemicrania headache
5 s–4 min 2–30 min 15–180 min Time
Overlap Overlap
between duration between duration
Fig. 8.1 Overlap Between Attack Duration in Trigeminal Autonomic
Cephalalgias SUNCT, Sudden unilateral neuralgiform conjunctival injec-
tion tearing. (From Leone M, Bussone G. Pathophysiology of trigeminal
autonomic cephalalgias. Lancet Neurol. 2009;8:755–774.)
23
24 SECTION 1 Headache and Facial Pain Syndromes

TABLE 8.2 Comparison of Cluster Headache

and Sudden Unilateral Neuralgiform FF
Conjunctival Injection Tearing Headache
Cluster SUNCT
Comparison Factors Headache Headache
Ocular and retroorbital location Yes Yes CI, Mi
Unilateral Yes Yes
Rapid onset to peak Yes Yes
Severe intensity Yes Yes
Attacks occur in paroxysms Yes Yes
Duration of attacks short Yes Yes EO, Pt
Pain free between attacks Yes Yes
Significant rhinorrhea during attacks Yes No
Alcohol triggers attacks Yes No
Tactile trigger areas No Yes
Seasonal pattern of attacks Yes No
NC, Rh
Chronobiological pattern of attacks Yes No La
Significant eye inflammation No Yes
Responds to sphenopalatine ganglion block Yes No
Responds to trigeminal nerve block No Yes

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.
Fig. 8.3 Autonomic signs and symptoms, ipsilateral to the pain,
observed in a patient with sudden unilateral neuralgiform conjunctival
injection tearing. CI, Conjunctival injection; EO, eyelid edema; FF, fore-
head and facial flushing; La, lacrimation; Mi, miosis; NC, nasal conges-
tion; Pt, ptosis; Rh, rhinorrhea. (From Kitahara I, Fukuda A, Imamura Y,
et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome.
World Neurosurg. 2015;84[4]:1080–1083, fig. 1.)

TABLE 8.3 Descriptors of Pain Associated

With Sudden Unilateral Neuralgiform
Conjunctival Injection Tearing Headache
Stabbing
Shooting
Lancinating
Shock-like
Sharp
Piercing
Pricking
Staccato-like

Fig. 8.2 Patients with sudden unilateral neuralgiform conjunctival

injection tearing headache present with severe paroxysms of ocular or
periorbital pain that radiates into the ipsilateral temple, forehead, nose,
cheek, throat, and maxillary region that is associated with significant
inflammation of the affected eye.
neurological findings. In patients who cannot undergo MRI,
such as patients with pacemakers, computed tomography
(CT) is a reasonable second choice. Radionuclide bone scan-
ning and plain radiography are indicated if fracture or bony
abnormality such as metastatic disease is considered in the
differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
SUNCT headache is in question. Intraocular pressure should be
measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
SUNCT headache is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic SUNCT
headache include cluster headache, temporal arteritis, trigem-
inal neuralgia involving the first division of the trigeminal
nerve, demyelinating disease, primary stabbing headache, hyp-
nic headache, and chronic paroxysmal hemicrania, although
because of the overlapping features of all headache and facial
pain syndromes, SUNCT headache can be easily mistaken for
another type of headache or facial pain (Fig. 8.5; Table 8.4).
Trigeminal neuralgia involving the first division of the trigem-
inal nerve is uncommon and is characterized by trigger areas
and tic-like movements. Demyelinating disease is generally
associated with other neurological findings, including optic
CHAPTER 8 Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache 25

D
Fig. 8.4 Magnetic resonance imaging (MRI) with right-sided short-lasting unilateral neuralgiform headache
attacks with conjunctival injection and tearing syndromes, demonstrating an aberrant loop of the causative
vessels abutting the right trigeminal nerve. Right (R) and left (L) sides of the patient are indicated on the MRI
images. MRI (A) and operative (B) images for case 1. Axial (A, upper panel) and sagittal (lower panel) images
are shown. The side of the first branch of the right trigeminal nerve (RTN) was compressed by the vertebral
artery (VA). MRI (C) and operative (D) images for case 2. Axial (C, top panel) and 2 sagittal images are shown.
Compression of the side of the first branch of the right trigeminal nerve by the superior cerebellar artery (SCA)
was suspected (C, middle panel). The side of the third branch of the right trigeminal nerve was compressed
by the anterior inferior cerebellar artery (AICA) (C, bottom panel). FTN, The first branch of the trigeminal nerve;
LTN, left trigeminal nerve; PV, petrosal vein; RC, right cerebellum; TTN, the third branch of the trigeminal
nerve; VII–VIII N, VII and VIII nerves. (From Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical
treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84[4]:1080–1083, fig. 2.)
26 SECTION 1 Headache and Facial Pain Syndromes

Cluster headache TABLE 8.4 Differential Diagnosis: Sudden

Unilateral Neuralgiform Conjunctival Injection
Tearing Headache
Cluster headache
Temporal arteritis
Trigeminal neuralgia
Demyelinating disease
Neck Primary stabbing headache
Paroxysmal hemicrania Hypnic headache
Chronic paroxysmal hemicrania

neuritis and other motor and sensory abnormalities. The pain

of chronic paroxysmal hemicrania lasts much longer than the
pain of SUNCT headache.
Shoulder,
neck, arm
SUNCT TREATMENT
The treatment of SUNCT headache is analogous to the treatment
of trigeminal neuralgia, although the pharmacological manage-
ment of this uncommon headache disorder is disappointing.
The use of anticonvulsants such as lamotrigine and gabapen-
tin represents a reasonable starting point. High-dose steroids
tapered over 10 days also have been anecdotally reported to pro-
vide relief. For patients who do not respond to the previously
Hemicrania continua mentioned treatments, daily trigeminal nerve block with a local
anesthetic and steroid is a reasonable next step.
Occasionally, retrogasserian injection of glycerol, balloon
compression of the gasserian ganglion, and microvascular
decompression of the trigeminal nerve root are required to
provide palliation of pain. Underlying sleep disturbance and
depression associated with the pain of SUNCT headache are
best treated with a tricyclic antidepressant compound, such as
nortriptyline, which can be started at a single bedtime dose of
Migraine 25 mg.

COMPLICATIONS AND PITFALLS

Failure to diagnose SUNCT headache correctly may put the
patient at risk if an intracranial pathological condition or demy-
elinating disease, which may mimic the clinical presentation
of SUNCT headache, is overlooked. MRI is indicated in all
patients thought to have SUNCT headache. Failure to diagnose
NVOP glaucoma or temporal arteritis, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.

but may be bilateral in up to 30% of cases (this has been marked by a

Fig. 8.5 Pain Location in the Trigeminal Autonomic Cephalgias and
Neurovascular Orofacial Pain (NVOP) The TACs are characterized by
orbital and periorbital pain. In paroxysmal hemicrania and hemicrania
continua, large adjacent areas are affected. Migraine is largely unilateral
lighter shaded area contralaterally). Neurovascular orofacial pain is char-
acterized by its location in the lower two-thirds of the face with intra-
oral and perioral areas frequently involved as primary sites. Two-headed
arrow above diagram indicates the side shift that occurs in specific
headache. SUNCT, Sudden unilateral neuralgiform conjunctival injec-
tion tearing. (Modified from Benoliel R, Sharav Y. The trigeminal auto-
nomic cephalgias (TACs). In: Sharav Y, ed. Orofacial Pain and Headache.
Edinburgh: Elsevier; 2008:225–254.)
 CHAPTER 8 Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache
CLINICAL PEARLS Trigeminal nerve block with local anesthetic is
especially useful in the diagnosis and palliation of pain secondary to SUNCT
headache. Given the uncommon nature of this headache syndrome and its
overlap with the symptoms of cluster headache and other neurological prob-
lems, including cavernous sinus thrombosis and intracranial and retroorbital
tumors, SUNCT headache must remain a diagnosis of exclusion. All patients
suspected to have SUNCT headache require MRI of the brain with and without
gadolinium contrast material and thorough ophthalmological and neurologi-
cal evaluation. Trigeminal nerve block should be performed only by clinicians
familiar with the regional anatomy.
SUGGESTED READINGS
Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical
treatment, and cure for SUNCT syndrome. World Neurosurg.
2015;84(4):1080–1083.
Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalalgias.
III. Short-lasting unilateral neuralgiform headache attacks with
conjunctival injection and tearing. Oral Surg Oral Med Oral Pathol
Oral Radiol Endodontol. 2007;104:773–771.
Leone M, Bussone G. Pathophysiology of trigeminal autonomic ceph-
alalgias. Lancet Neurol. 2009;8:755–774.
Levin M. Nerve blocks and nerve stimulation in headache disorders.
Tech Reg Anesth Pain Manage. 2009;13:42–49.
27
Levin M. Nerve blocks in the treatment of headache. Neurotherapeu-
tics. 2010;7:197–203.
Nagel MA, Burns TM, Gilden D. SUNCT headaches after ipsilateral
ophthalmic-distribution zoster. J Neurol Sci. 2016;366:207–208.
Nobuhiro, Yoshiharu T, Takamasa N, et al. Four patients with
short-lasting unilateral neuralgiform headache with conjunctival
injection and tearing (SUNCT) successfully treated with lamo-
trigine. J Neurol Sci. 2017;381(suppl):941.
Pulido Fontes L, Mayor Gómez S. SUNCT (Short-lasting unilateral
neuralgiform headache attack with conjunctival injection and tear-
ing) associated with pituitary lesion. Neurología (English Edition).
2015;30(7):458–459.
Rozen TD. Trigeminal autonomic cephalalgias. Neurol Clin.
2009;27:537–557.
Waldman SD. Gasserian ganglion block. In: Waldman SD, ed. Atlas of
Interventional Pain Management. 3rd ed. Philadelphia: Saunders;
2009:32–38.
Waldman SD. Gasserian ganglion block: balloon compression tech-
nique. In: Waldman SD, ed. Atlas of Interventional Pain Manage-
ment. 3rd ed. Philadelphia: Saunders; 2009:43–47.
Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
view. Philadelphia: Saunders; 2009:15–17.
Waldman SD. Trigeminal nerve block: coronoid approach. In: Wald-
man SD, ed. Atlas of Interventional Pain Management. 3rd ed.
Philadelphia: Saunders; 2009:47–50.
Williams MH, Broadley SA. SUNCT and SUNA: clinical features and
medical treatment. J Clin Neurosci. 2008;15:527–534.
 9
Primary Thunderclap Headache

thunderclap headache can be located anywhere in the head or

ICD-10 CODE G44.53
neck. Nausea and vomiting are present approximately 75% of the
time. However, the nuchal rigidity and other focal neurological
signs often associated with acute subarachnoid hemorrhage and
other neurologically devastating syndromes in which severe head-
THE CLINICAL SYNDROME ache of acute onset are a prominent feature are uniformly absent.
Thunderclap headache, which is also known as lone acute severe
headache, is an uncommon type of headache that may be the
result of an underlying vascular or nonvascular intracranial
TESTING
abnormality or may represent a primary headache syndrome of Testing in patients suspected of having primary thunder-
unknown cause. Common and uncommon causes of thunder- clap headache has two immediate goals: (1) to identify
clap headache are listed in Table 9.1. The more benign, though occult intracranial pathological conditions or other dis-
no less painful, primary thunderclap headache occurs over three eases that may mimic primary thunderclap headache and
times more frequently than the serious secondary thunderclap may require specific urgent treatment and (2) to identify
headache. Because of the often-threatening causes of the less the presence of subarachnoid hemorrhage. All patients with
common secondary thunderclap headache (e.g., subarachnoid a recent onset of severe headache thought to be secondary
hemorrhage, cerebral venous thrombosis), urgent evaluation
including computed tomography (CT) and/or magnetic reso-
nance imaging (MRI) of the brain and cerebrospinal fluid anal- TABLE 9.1 Main and Rare Causes of
ysis are indicated in all patients suspected of having thunderclap Thunderclap Headache
headache. Main Causes Rare Causes
One of the most severe headaches encountered in clinical Vascular Disorders
practice, thunderclap headache is characterized by a very rapid
Subarachnoid hemorrhage Pituitary apoplexy, arteritis, angiitis
onset to peak of less than 1 minute. The headache may last from Intracerebral hemorrhage Unruptured vascular malformation, aneurysm
1 to 10 days and, because of its intensity, almost always provokes Cerebral venous thrombosis Arterial hypertension
an urgent trip to the emergency department, where the head- Spontaneous intracranial Cerebral segmental vasoconstriction
ache is invariably initially misdiagnosed as the sentinel head- hypotension
ache of acute subarachnoid hemorrhage or other potentially Cervical artery dissection
catastrophic headache syndromes (Tables 9.2 and 9.3). This is
Nonvascular Disorders
not surprising in that primary thunderclap headache is virtually
indistinguishable clinically from subarachnoid hemorrhage, Greater occipital neuralgia
Intermittent hydrocephalus by colloid cyst
one of the most neurologically devastating forms of cerebro-
Infections
vascular accident. Thus, because of the serious consequences of
misdiagnosis, by necessity primary thunderclap headache is a Meningitis, encephalitis Erve virus
diagnosis of exclusion. Sinusitis

Primary Headache Disorders

SIGNS AND SYMPTOMS Migraine Cluster headache
Primary thunderclap headache Tension headache, new daily persistent
As mentioned earlier, primary thunderclap headache is character- headache
ized by a very rapid onset to peak of less than 1 minute without Primary exertional headache
obvious inciting factors (e.g., sexual activity, coughing, strain- Primary cough headache
ing at stool). The patient with primary thunderclap headache is Modified from Linn FHH. Primary thunderclap headache. In: Aminoff
almost always convinced that he or she is having a stroke and MJ, ed. Handbook of Clinical Neurology. Vol. 97. New York: Elsevier;
often appears frightened and anxious. The headache of primary 2010:473–481.

28
 CHAPTER 9 Primary Thunderclap Headache 29

TABLE 9.2 Comparison of Primary

Thunderclap Headache and Subarachnoid
Hemorrhage
Primary Thunderclap Subarachnoid
Comparison Factors Headache Hemorrhage
Severe headache Yes Yes
Nausea and vomiting Yes Yes
Focal neurological signs No Yes
Nuchal rigidity No Yes
Photophobia No Yes
Vertigo No Yes A
Neck and back pain No Yes

TABLE 9.3 Diseases That May Mimic

Primary Thunderclap Headache
Hemorrhagic
Ischemic
Neoplasm
Infection
Meningitis
Encephalitis B
Abscess
Parasitic
Hypertensive crisis
Loss of spinal fluid
Postdural puncture headache
Spontaneous spinal fluid leak
Collagen-vascular disease
Lupus cerebritis
Vasculitis
Polymyositis
Headache
Cluster headache C
Primary exertional headache
Primary cough headache Fig. 9.1 Computed Tomography Scan Showing Subarachnoid
Migraine Hemorrhage (SAH) Right middle cerebral artery aneurysm in a 58-year-
Ice pick headache old man with SAH and intracranial hematoma (IH). (A) Volume rendering
Primary sexual headache image from computed tomography angiography (CTA) clearly displays
the relationship of the aneurysm to bone structures, adjacent branch
vessels, and aneurysmal neck (arrow). (B) Maximum intensity projec-
to primary thunderclap headache should undergo emergent tion (MIP) image from CTA clearly demonstrates the relationship of the
aneurysm (arrow). (C) Thin-MIP image from CTA shows the relationship
CT of the brain to rule out any pathological condition that of the aneurysm to IH (arrowhead), and the ruptured aneurysm has a
could be responsible for the patient’s symptoms (Fig. 9.1). small nipple (arrow). (From Chen W, Yang Y, Xing W, et al. Applications
Modern multidetector CT scanners have a diagnostic accu- of multislice CT angiography in the surgical clipping and endovascular
racy approaching 100% for subarachnoid hemorrhage if CT coiling of intracranial aneurysms. J Biomed Res. 2010;24:467–473.)
angiography of the cerebral vessels is part of the scanning
protocol. Cerebral angiography may also be required if sur- hemorrhage. Blood typing and crossmatching should be con-
gical intervention is being considered and the site of bleeding sidered in any patient in whom surgery is being contemplated
cannot be accurately identified. or who has preexisting anemia. Careful serial ophthalmologi-
MRI of the brain and magnetic resonance angiography cal examination should be performed on all patients with sub-
(MRA) may be useful if an aneurysm is not identified on CT arachnoid hemorrhage to chart the course of papilledema.
studies and may be more accurate in the diagnosis of arterio- Lumbar puncture is useful in revealing the presence or
venous malformations (AVMs) (Fig. 9.2). Screening laboratory absence of blood in the spinal fluid, but its utility may be limited
tests, including an erythrocyte sedimentation rate, complete by the presence of increased intracranial pressure, making lum-
blood count, coagulation studies, and automated blood chem- bar puncture too dangerous. Electrocardiographic abnormal-
istry, should be performed in patients with subarachnoid ities are common in patients with subarachnoid hemorrhage
30 SECTION 1 Headache and Facial Pain Syndromes

A B C

D E F
Fig. 9.2 Magnetic Resonance Imaging Showing Arteriovenous Malformation Patient with aneurysm-re-
lated false aneurysm (FA) in right parietal region. Preangiographic T1-weighted magnetic resonance axial
image (A) and T2-weighted magnetic resonance coronal image (B) show round lesion (arrow) with flow void
and mixed signal in the center and mixed signal on the periphery. Fluid-attenuated inversion recovery image
(C) reveals small area of surrounding edema (arrow). (D) Flow in the center of FA (arrow) on two-dimensional
time-of-flight magnetic resonance angiography. (E) Preembolization digital subtraction angiography image. (F)
Residual inflow to FA (arrow) on postembolization digital subtraction angiography (DSA) image (arrow indicates
arteriovenous malformation). (From Brzozowski K, Frankowska E, Piasecki P, et al. The use of routine imaging
data in diagnosis of cerebral pseudoaneurysm prior to angiography. Eur J Radiol. 2011;80:e401–e409.)

and are thought to be due to abnormally high levels of circu-

lating catecholamines and hypothalamic dysfunction; however,
they are rarely present in patients with primary thunderclap
headache.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of primary thunderclap headache
generally can be thought of as the diagnosis of the lesser of
two evils because most of the diseases that mimic primary
thunderclap headache are also associated with significant
mortality and morbidity. Table 9.3 lists diseases that may
be mistaken for primary thunderclap headache. Prominent
among them is subarachnoid hemorrhage, stroke, colla-
gen-vascular disease, infection, neoplasm, hypertensive cri-
sis, spinal fluid leaks, and a variety of more benign causes of
headache.
TREATMENT
Although no generally accepted treatment for primary thunder-
clap headache has been defined, the following guidelines may
be useful for the clinician when faced with a patient thought to
have this uncommon headache syndrome. First and foremost,
if test results reveal no evidence of intracranial pathology or
other serious, life-threatening diseases, constant reassurance
that the patient does not have a stroke or brain tumor is indi-
cated. In general, drugs used to treat headaches whose primary
mechanism of action is vasoconstriction (e.g., ergots, triptans)
should be avoided. Anecdotal reports indicate that intravenous
nimodipine may help abort acute attacks and prevent recurrent
headache episodes. Gabapentin also has been advocated as a
reasonable treatment for primary thunderclap headache and,
given its favorable risk-to-benefit ratio, may be a reasonable
therapeutic option.
 CHAPTER 9
COMPLICATIONS AND PITFALLS
Complications and pitfalls in the diagnosis and treatment of
primary thunderclap headache generally fall into three catego-
ries. The first category involves the failure to recognize a sentinel
bleed associated with subarachnoid hemorrhage and evaluate
and treat the patient before significant morbidity or mortality
occurs. The second category involves misdiagnosis that results
in unnecessary testing, specifically, cerebral angiography, which
itself is associated with significant morbidity and rarely death.
The third category involves iatrogenic morbidity and rarely mor-
tality from the use of medications to treat primary thunderclap
headache (e.g., triptans, ergots) that not only do not treat this pri-
mary headache syndrome but also have significant side effects.
CLINICAL PEARLS Primary thunderclap headache is a diagnosis of exclu-
sion. It is frequently misdiagnosed as the sentinel headache of subarachnoid
hemorrhage, causing the treating physician to order urgent diagnostic testing,
which is associated with its own significant mortality and morbidity. The lack
of focal neurological findings in a patient with acute headache should point
the clinician toward the diagnosis of benign primary headaches including pri-
mary thunderclap headache, cough headache, exertional headache, atypical
migraine, and headache associated with sexual activity. This does not mean
that urgent computerized scanning of the brain and analysis of the patient’s
cerebrospinal fluid are not indicated.
SUGGESTED READINGS
Arenaza-Basterrechea N, Iglesias Díez. F, López Sarnago P. Thunder-
clap headache secondary to pneumocephalus. Neurología (English
Ed). 2017;32(2):132–133.
Chih-Ming H, Ya-Ju L, Yang-Kai F, Shih-Pin C, Tzu-Hsien L. Iso-
lated thunderclap headache during sex: orgasmic headache or
reversible cerebral vasoconstriction syndrome? J Clin Neurosci.
2010;17:1349–1351.
Primary Thunderclap Headache 31
de Bruijn SFTM, Stam J, Kappelle LJ. CVST Study Group: thunderclap
headache as first symptom of cerebral venous sinus thrombosis.
Lancet. 1996;348:1623–1625.
Esmanhotto BB, Piovesan EJ, Moreschi FA, et al. Thunderclap head-
ache prevalence and modification of preexisting headache patterns
after endovascular embolization in patients with ruptured intra-
cranial aneurysm. J Neurol Sci. 2015;357(suppl 1):e166–e167.
Janardhan V, Biondi A, Riina HA, et al. Vasospasm in aneurysmal
subarachnoid hemorrhage: diagnosis, prevention, and manage-
ment. Neuroimaging Clin N Am. 2006;6:483–496.
Linn FHH. Primary thunderclap headache. In: Aminoff MJ, ed.
Handbook of Clinical Neurology. Vol. 97. New York: Elsevier;
2010:473–481.
Manno EM. Subarachnoid hemorrhage. Neurol Clin. 2004;22:347–
366.
Hennessy M. All thunderclap headaches are equal but some are more
equal than others. Clin Radiol. 2015;70(suppl 1):S6–S7.
Newfield P. Intracranial aneurysms: vasospasm and other issues. In:
Atlee JL, ed. Complications in Anesthesia. 2nd ed. Philadelphia:
Saunders; 2007:719–723.
Palestrant D, Connolly ES. Subarachnoid hemorrhage. Neurobiol Dis.
2007:265–270.
Pouration N, Dumont AS, Kassell NF. Subarachnoid hemorrhage. In:
Alves W, Skolnick B, eds. Handbook of Neuroemergency Clinical
Trials. New York: Elsevier; 2006:17–44.
Yamakawa M, Hashimoto Y, Sakamoto T, et al. Clinical features of
patients presenting reversible cerebral vasoconstriction without
thunderclap headache. J Neurol Sci. 2017;381(suppl):1124.
 10
Hypnic Headache

ICD-10 CODE G44.81 TESTING

Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
THE CLINICAL SYNDROME MRI is highly accurate and helps identify abnormalities that
Also known as alarm clock headache, hypnic headache is a term may put the patient at risk for neurological disasters second-
used to describe an uncommon headache syndrome charac- ary to intracranial and brainstem pathological conditions,
terized by its propensity to wake the person at the same time including tumors and demyelinating disease. MRI helps iden-
each night. Always occurring during sleep, hypnic headache is tify bleeding associated with leaking intracranial aneurysms,
of short duration and rarely lasts more than 15 minutes after which may mimic the symptoms of both types of hypnic head-
the patient is awakened by the pain (Fig. 10.1). Research sug- ache. Magnetic resonance angiography (MRA) may be useful in
gests that hypnic headache occurs most commonly during identifying aneurysms responsible for the patient’s neurologi-
rapid eye movement (REM) sleep and it has been suggested that cal symptoms. In patients who cannot undergo MRI, such as
hypothalamic dysregulation may be the pathologic mechanism patients with pacemakers, computed tomography (CT) is a rea-
that triggers this rare headache syndrome (Fig. 10.2). Hypnic sonable second choice. Lumbar puncture should be performed
headache occurs frequently, with a mean incidence of at least
15 attacks per month. The location of the headache pain varies,
and the intensity of pain described as moderate with an ach-
ing character. Unlike cluster headache, which also occurs after
sleep, patients with hypnic headache exhibit no autonomic signs
or symptoms. Hypnic headache is a disease of the late fifth and
sixth decades, with a mean age of onset of 63 years, although
cases in the pediatric and adolescent population have been
reported. It occurs more commonly in females.

First night: 2:34 AM

SIGNS AND SYMPTOMS
Hypnic headache is associated with no obvious neurological or
musculoskeletal disease. Specifically, there are no autonomic signs
or symptoms as are often seen with cluster headache. Furthermore,
no accompanying focal neurological signs or systemic symptoms
occur as with cluster headache and migraine headache, although
rarely nausea can occur. The age of onset of benign hypnic head-
ache is generally in the late fifth or sixth decade of life. Although
no specific location is seen in hypnic headache, they are bilateral Second night: 2:34 AM
in 66% of patients. When the headaches are unilateral, they tend to
occur on the same side night after night. What is fascinating to the
treating physician and frustrating to the patient is the fact that hyp-
nic headache wakes the patient from a sound sleep at almost the
same time each night. Because the onset of hypnic headache occurs
later in life, it must be considered a diagnosis of exclusion as with
the other uncommon primary headache syndromes, for example,
cough headache and thunderclap headache. The clinician must
assiduously search for other explanations for the patient’s headache Third night: 2:34 AM
symptomatology, including intracranial pathological conditions Fig. 10.1 Hypnic headache is also known as alarm clock headache due
and systemic disease. to its propensity to wake the person up at the same time each night.

32

Fig. 10.2 Gray Matter Decrease in the Hypothalamus in Hypnic
Headache Voxel-based morphometry shows decreased gray matter
in the hypothalamus. These structural data suggest an involvement of
the hypothalamus in the pathophysiology of hypnic headache. (From
Obermann M, Holle D. Headache, hypnic. In: Aminoff MJ, Daroff RB,
eds. Encyclopedia of the Neurological Sciences. 2nd ed. Oxford: Aca-
demic Press; 2014:516–519, fig. 1.)
TABLE 10.1 Nocturnal Headaches That May
Be Confused With Hypnic Headache
Cluster headache
Headache associated with sleep apnea
Headache associated with nocturnal arterial hypertension
Headache associated with increased intracranial pressure
Analgesic rebound headache
if intracranial hemorrhage is suspected even if blood is not
present on MRI or CT. Plain radiographs of the cervical spine
also may be useful in the evaluation of Arnold-Chiari malfor-
mations and should be included in the evaluation of all patients
with hypnic headache.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of hyp-
nic headache is in question. Intraocular pressure should be
measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Hypnic headache is a clinical diagnosis supported by a com-
bination of clinical history, physical examination, radiogra-
phy, MRI, and MRA. Pain syndromes that may mimic hypnic
headache include the uncommon primary headaches benign
exertional headache, ice pick headache, and sexual headache,
although the unique same-time nocturnal occurrence should
help the clinician easily identify the patient’s symptoms as
hypnic headache. The clinician must consider other types of
headache that occur more frequently at night, including clus-
ter headache and headaches associated with sleep apnea, noc-
turnal arterial hypertension, analgesic rebound, and increased
intracranial pressure (Table 10.1). Given that hypnic headache
CHAPTER 10 Hypnic Headache 33
occurs in an older population, temporal arteritism must also
be considered.
Less commonly, hypnic headache may be confused with tri-
geminal neuralgia (involving the first division of the trigem-
inal nerve) or demyelinating disease. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
ments. Demyelinating disease is generally associated with
other neurological findings, including optic neuritis and other
motor and sensory abnormalities. The pain of chronic parox-
ysmal hemicrania and cluster headache is associated with red-
ness and watering of the ipsilateral eye, nasal congestion, and
rhinorrhea during the headache. These findings are absent in
hypnic headache. Migraine headache may or may not be asso-
ciated with painless neurological findings known as aura, but
patients almost always report some systemic symptoms, such
as nausea or photophobia, not typically associated with hypnic
headache.
TREATMENT
Indomethacin and lithium carbonate are the treatments of
choice for hypnic headache, with indomethacin being slightly
more effective for the unilateral form of the syndrome.
Indomethacin at a starting dose of 25 mg daily for 2 days and
titrating to 25 mg three times per day is a reasonable treatment
approach. This dose may be carefully increased up to 150 mg per
day. Indomethacin must be used carefully, if at all, in patients
with peptic ulcer disease or impaired renal function. Headache
specialists have noted anecdotal reports of a positive response
to cyclooxygenase-2 (COX-2) inhibitors in the treatment of
benign hypnic headache. Lithium carbonate is used in the same
manner as in the treatment of cluster headache and has its basis
in use in its proven efficacy in the treatment of other diseases
thought to have a chronobiological basis, such as cluster head-
ache and bipolar disorders. However, the therapeutic window
of lithium carbonate is small, and this drug should be used with
caution. A starting dose of 300 mg at bedtime may be increased
after 48 hours to 300 mg twice per day. If no side effects are
noted after 48 hours, the dose may be increased again to 300
mg three times per day. Anecdotal reports exist that gabapentin
and pregabalin also may be useful in decreasing the frequency
and intensity of attacks of hypnic headache. Unlike with cluster
headache, oxygen inhalation has been ineffective in aborting
attacks of hypnic headache once the patient has been awakened
by the pain.
COMPLICATIONS AND PITFALLS
Failure to diagnose hypnic headache correctly may put the
patient at risk if an intracranial pathological condition or demy-
elinating disease, which may rarely mimic the clinical pre-
sentation of hypnic headache, is overlooked. MRI and MRA
are indicated in all patients thought to have hypnic headache.
Failure to diagnose glaucoma, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.
34 SECTION 1 Headache and Facial Pain Syndromes
CLINICAL PEARLS Any patient presenting with nocturnal headaches
should be taken very seriously. Although statistically most of these headaches
ultimately are proved to be of benign cause, a few patients have potentially
life-threatening disease. The diagnosis of hypnic headache is made by obtain-
ing a thorough, targeted headache history and performing a careful physical
examination. The clinician must separate patients with hypnic headache from
patients with headaches caused by an intracranial pathological condition
such as tumors or systemic disease such as nocturnal arterial hypertension.
Patients with hypnic headache should have a normal neurological examina-
tion. If the neurological examination is abnormal, the diagnosis of benign hyp-
nic headache should be discarded and a careful search for the cause of the
patient’s neurological findings should be undertaken.
SUGGESTED READINGS
Alberti A. Headache and sleep. Sleep Med Rev. 2006;10:431–437.
Berciano J, Poca M-A, García A, Sahuquillo J. Paroxysmal cervi-
cobrachial hypnic-induced pain in a patient with syringomy-
elia extending into spinal cord posterior gray horns. J Neurol.
2007;254:678–681.
Chen YY, Lirng JF, Fuh JL, et al. Primary hypnic headache is associat-
ed with posterior fossa crowdedness: a morphometric MRI study.
Cephalalgia. 2004;24:694–699.
Fowler MV, Capobianco DJ, Dodick DW. Headache in the elderly.
Semin Pain Med. 2004;2:123–128.
Manni R, Ghiotto N. In: Aminoff M, ed. Handbook of Clinical Neurol-
ogy. New York: Elsevier; 2010:469–472.
Obermann M. Holle D. Headache, hypnic. In: Encyclopedia of the
Neurological Sciences. 6th ed. Oxford: Academic Press; 2014:516–
519.
Pascual J, González-Mandly A, Martín R, Oterino A. Headaches
precipitated by cough, prolonged exercise or sexual activity:
a prospective etiological and clinical study. J Headache Pain.
2008;9:259–266.
Pascual J. Primary hypnic headache. Curr Pain Headache Rep.
2005;9:272–276.
Silva-Néto RP, Almeida KA. Hypnic headache: a descriptive study of
25 new cases in Brazil. J Neurol Sci. 2014;338(1–2):166–168.
Silva-Néto RP, Almeida KA. Hypnic headache in childhood: a litera-
ture review. J Neurol Sci. 2015;356(1–2):45–48.
Waldman SD. Arnold Chiari malformation type I. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:27–28.
Waldman SD. Arnold Chiari malformation type II. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:29–30.
 11
Nummular Headache

to intracranial and brainstem pathological conditions, includ-

ICD-10 CODE R51
THE CLINICAL SYNDROME
Nummular headache is an uncommon chronic headache syn-
drome characterized by constant localized pain with superim-
posed paroxysms of stabbing jabs and jolts of mild to moderate
intensity that occur in a coin-shaped localized area of the scalp.
Most commonly located in the parietal region, the pain of num-
mular headache is unilateral and localized to a single area. It
rarely if ever switches sides. The scalp overlying the area may
be tender to touch and stimulation of the area; for example, the
brushing of hair may exacerbate the pain. Nummular headache
occurs slightly more commonly in women and is generally not
seen before the fourth decade of life, but rare reports of chil-
dren suffering from nummular headache sporadically appear
in the literature. Nummular headache has been associated with
coexistent migraine headache and occipital neuralgia. A high
prevalence of autoimmune indices and disorders have also
been identified in patients suffering from nummular headache.
Nummular headache is also known as coin-shaped headache.
ing tumors and calvarial lesions (Fig. 11.2). Magnetic resonance
angiography (MRA) also may be useful in helping identify
aneurysms, which may be responsible for the patient’s pain. In
patients who cannot undergo MRI, such as patients with pace-
makers, computed tomography (CT) is a reasonable second
choice. Radionuclide bone scanning and plain radiography are
indicated if fracture or bony abnormality, such as metastatic
disease, is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry should be performed if the diagnosis of nummular
headache is in question.
DIFFERENTIAL DIAGNOSIS
Nummular headache is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic nummular
headache include chronic paroxysmal hemicrania and jolts and
jabs headache. Trigeminal neuralgia involving the first division
of the trigeminal nerve is uncommon and is characterized by

SIGNS AND SYMPTOMS

A patient with nummular headache complains of a unifocal
region of pain and sensitivity most commonly occurring in
the vertex of the parietal region (Fig. 11.1). The pain is almost
always unilateral and does not switch sides, although rare
reports exist of bilateral nummular headache. Some patients
describe the pain of nummular headache as a constant dull ache
or sensitivity in the affected area with superimposed paroxysms
of lancinating pain. The pain is chronic, although spontaneous
remissions have been rarely reported. Some patients with num-
mular headache exhibit anxiety and depression because the
intensity of the associated pain leads many patients to believe
they have a brain tumor.

TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that Fig. 11.1 Patients Suffering From Nummular Headache Complain
may put the patient at risk for neurological disasters secondary of Unifocal Area of Pain and Scalp Sensitivity.

35
36 SECTION 1 Headache and Facial Pain Syndromes

A B
Fig. 11.2 Calvarial Metastases (A) Abnormal enhancement (arrows) is present within the diploë on this
gadolinium-enhanced T1-weighted image. Expansion of the left parietal bone occurs, affecting the inner table
more than the outer table. (B) Heterogeneous hyperintensity (arrows) persists within the calvaria on this
T2-weighted image. The right parietal lesion is no longer imaged on this more superior section. (From Edel-
man RR, Hesselink JR, Zlatkin MB, Crues JV III, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadel-
phia: Saunders; 2005.)

trigger areas and tic-like movements. Demyelinating disease is

generally associated with other neurological findings, including
optic neuritis and other motor and sensory abnormalities. The
pain of chronic paroxysmal hemicrania lasts much longer than
the pain of nummular headache and is associated with redness
and watering of the ipsilateral eye.
CLINICAL PEARLS The diagnosis of nummular headache is made by
taking a careful, targeted headache history. Patients with nummular headache
should have a normal neurological examination. If the results of the neurolog-
ical examination are abnormal, the diagnosis of nummular headache should
be discarded and a careful search for the cause of the patient’s neurological
findings should be undertaken.

TREATMENT
Nummular headache uniformly responds to treatment with
indomethacin. Failure to respond to indomethacin puts the
diagnosis of nummular headache in question. A starting dosage
of 25 mg daily for 2 days and titrating to 25 mg three times a day
is a reasonable treatment approach. This dose may be carefully
increased to 150 mg/day. Indomethacin must be used carefully,
if at all, in patients with peptic ulcer disease or impaired renal
function. Anecdotal reports of a positive response to cycloox-
ygenase-2 (COX-2) inhibitors in the treatment of nummular
headache have been noted in the headache literature, as well as
a successful treatment with gabapentin. Underlying sleep dis-
turbance and depression are best treated with a tricyclic antide-
pressant compound, such as nortriptyline, which can be started
at a single bedtime dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose nummular headache correctly may put
the patient at risk if an intracranial pathological condition or
calvarial disease, which may mimic the clinical presentation
of nummular headache, is overlooked. MRI is indicated in all
patients thought to have nummular headache.
SUGGESTED READINGS
Cohen GL. Nummular headache: what denomination? Headache.
2005;10:1417–1418.
Evans RW, Pareja JA. Nummular headache. Headache. 2005;45:164–
165.
Mathew NT. Indomethacin responsive headache syndromes. Head-
ache. 1981;21:147–150.
Pareja JA, Caminero AB, Serra J, et al. Nummular headache: a coin-
shaped cephalgia. Neurology. 2002;58:1678–1679.
Pareja JA, Pareja J, Barriga FJ, et al. Nummular headache: a prospec-
tive series of 14 new cases. Headache. 2004;44:611–614.
Pareja JA, Pareja J, Yangüela J. Nummular headache, trochleitis, su-
praorbital neuralgia, and other epicranial headaches and neural-
gias: the epicranias. J Headache Pain. 2003;4:125–131.
Iwanowski P, Kozubski W, Losy J. Nummular headache in a patient
with ipsilateral occipital neuralgia—a case report. Neurol Neuro-
chir Pol. 2014;48(2):141–143.
Chen WH, Chen YT, Lin CS, Li TH, Lee LH, Chen CJ. A high preva-
lence of autoimmune indices and disorders in primary nummular
headache. J Neurol Sci. 2012;320(1–2):127–130.

ICD-10 CODE M31.6
THE CLINICAL SYNDROME
As the name suggests, headache associated with temporal arte-
ritis is located primarily in the temples, with secondary pain
often located in the frontal and occipital regions. A disease of
the sixth decade and beyond, temporal arteritis affects whites
almost exclusively, and there is a 3:1 female gender predom-
inance. Temporal arteritis is also known as giant cell arteritis
because of the finding of giant multinucleated cells that infiltrate
arteries containing elastin, including the temporal, ophthal-
mic, and external carotid arteries (Fig. 12.1A). Approximately
half of patients with temporal arteritis also have polymyalgia
rheumatica.
SIGNS AND SYMPTOMS
Headache is seen in most patients with temporal arteritis. The
headache is in the temples and is usually continuous. The char-
acter of the headache pain associated with temporal arteritis is
aching and has a mild to moderate level of intensity. A patient
with temporal arteritis also may complain of soreness of the
scalp, making the combing of hair or resting the head on a firm
pillow extremely uncomfortable.
Although temporal headache is present in almost all patients
with temporal arteritis, the finding of intermittent jaw claudi-
cation is pathognomonic for the disease (see Fig. 12.1B). In an
elderly patient, jaw pain while chewing should be considered sec-
ondary to temporal arteritis until proved otherwise. In the pres-
ence of strong clinical suspicion that the patient has temporal
arteritis, immediate treatment with corticosteroids is indicated
(see discussion of treatment). The reason immediate treatment
is needed is the potential for sudden painless deterioration of
vision in one eye secondary to ischemia of the optic nerve.
In addition to the signs and symptoms mentioned previously,
patients with temporal arteritis experience myalgia and morn-
ing stiffness. Muscle weakness associated with inflammatory
muscle disease and many other collagen-vascular diseases is
absent in temporal arteritis, unless the patient has been treated
with prolonged doses of corticosteroids for other systemic dis-
ease, such as polymyalgia rheumatica. The patient also may
experience nonspecific systemic symptoms, including malaise,
weight loss, night sweats, and depression.
12
Headache Associated With
Temporal Arteritis
On physical examination, a swollen, indurated, nodular tem-
poral artery is present. Diminished pulses are often noted, as is
tenderness to palpation. Scalp tenderness to palpation is often
seen. Funduscopic examination may reveal a pale, edematous
optic disc. The patient with temporal arteritis often appears
chronically ill, depressed, or both.
TESTING
Erythrocyte sedimentation rate should be obtained in all
patients suspected to have temporal arteritis. In temporal arteri-
tis, the erythrocyte sedimentation rate is greater than 50 mm/h
in more than 90% of patients. Less than 2% of patients with biop-
sy-proved temporal arteritis have normal erythrocyte sedimen-
tation rates. Ideally, the blood for the erythrocyte sedimentation
rate should be obtained before beginning corticosteroid therapy
because the initial level of elevation of this test is useful not only
to help diagnose the disease but also as a mechanism to establish
the efficacy of therapy. The erythrocyte sedimentation rate is a
nonspecific test, and other diseases that may manifest clinically
in a manner similar to temporal arteritis, such as malignancy or
infection, also may markedly elevate the erythrocyte sedimen-
tation rate. Confirmation of the clinical diagnosis of temporal
arteritis requires ultrasound imaging and/or a temporal artery
biopsy. Ultrasound imaging can provide confirmation by identi-
fication of a halo sign surrounding the affected temporal artery.
The finding of a positive halo sign strongly supports the diagno-
sis of temporal arteritis (Fig. 12.2).
Given the simplicity and safety of temporal artery biopsy, it
probably should be performed on all patients suspected of hav-
ing temporal arteritis. The presence of an inflammatory infil-
trate with giant cells in the biopsied artery is characteristic of
the disease. Edema of the intima and disruption of the internal
elastic lamina strengthen the diagnosis. A small percentage of
patients with clinical signs and symptoms strongly suggestive
of temporal arteritis who also exhibit a significantly elevated
erythrocyte sedimentation rate have a negative temporal artery
biopsy result. As mentioned, in the presence of a strong clini-
cal impression that the patient has temporal arteritis, an imme-
diate blood sample for erythrocyte sedimentation rate testing
should be obtained and the patient started on corticosteroids.
Complete blood cell count and automated chemistries, includ-
ing thyroid testing, are indicated in all patients with suspected
temporal arteritis to help rule out other systemic disease that
may mimic the clinical presentation of temporal arteritis.
37
38 SECTION 1 Headache and Facial Pain Syndromes
Ophthalmic
artery
Fig. 12.1 (A) Temporal arteritis is a disease of the sixth decade that occurs almost exclusively in whites, with
a predilection of 3:1 for women. (B) The sine qua non of temporal arteritis is jaw claudication.
Halo sign
Cephalad to the orbit
Superficial temporal artery transverse view
Fig. 12.2 Positive Halo Sign on Transverse Ultrasound Image in a
Patient With Temporal Arteritis The patient’s sedimentation rate was
98.
If the diagnosis of temporal arteritis is in doubt, magnetic res-
onance imaging (MRI) of the brain provides the best information
regarding the cranial vault and its contents. MRI is highly accu-
rate and helps identify abnormalities that may put the patient
at risk for neurological disasters secondary to intracranial and
brainstem pathological conditions, including tumors and demy-
elinating disease. More importantly, MRI helps identify bleed-
ing associated with leaking intracranial aneurysms. Magnetic
resonance angiography (MRA) may be useful to help identify
aneurysms responsible for neurological symptoms. In patients
who cannot undergo MRI, such as patients with pacemakers,
computed tomography (CT) is a reasonable second choice. If
intracranial hemorrhage is suspected, lumbar puncture should
be performed, even if blood is not present on MRI or CT. Case
reports of the utility of positron emission tomography (PET)/
computerized tomography scans in the diagnosis of giant cell
arteritis of the temporal, occipital, and vertebral arteries may
offer additional diagnostic options (Fig. 12.3). Intraocular pres-
sure should be measured if glaucoma is suspected.
Temporal artery
External
carotid artery
DIFFERENTIAL DIAGNOSIS
Headache associated with temporal arteritis is a clinical diag-
nosis supported by a combination of clinical history, abnormal
findings on physical examination of the temporal artery, normal
radiography, MRI findings, an elevated erythrocyte sedimen-
tation rate, and a positive temporal artery biopsy result. Pain
syndromes that may mimic temporal arteritis include tension
type headache, brain tumor, other forms of arteritis, trigeminal
neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, migraine headache, cluster headache,
and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
ments. Demyelinating disease is generally associated with other
neurological findings, including optic neuritis and other motor
and sensory abnormalities. The pain of chronic paroxysmal
hemicrania and cluster headache is associated with redness and
watering of the ipsilateral eye, nasal congestion, and rhinorrhea
during the headache. These findings are absent in all types of
sexual headache. Migraine headache may or may not be associ-
ated with painless neurological findings known as aura, but the
patient almost always reports some systemic symptoms, such as
nausea or photophobia, not typically associated with the head-
ache of temporal arteritis.
TREATMENT
The mainstay of treatment for temporal arteritis and its associ-
ated headaches and other systemic symptoms is the immediate
use of corticosteroids. If visual symptoms are present, an ini-
tial dose of 80 mg of prednisone is indicated. This dose should
be continued until the symptoms of temporal arteritis have
 CHAPTER 12 Headache Associated With Temporal Arteritis 39

Fig. 12.3 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/

CT) scan (axial slices), PET brain protocol—spectrum color scale, CT (same slice) and fusion PET/CT. High
18F-FDG uptake is evident in temporal arteries (red arrows), in their branches (yellow arrows), occipital
arteries (green arrows), and vertebral arteries (white arrows). (From Rehak Z, Vasina J, Ptacek J, et al. PET/
CT in giant cell arteritis: high 18F-FDG uptake in the temporal, occipital and vertebral arteries. Rev Esp Med
Nucl Imagen Mol. 2016;35[6]:398–401, fig. 2. ISSN 2253-654X, https://doi.org/10.1016/j.remn.2016.03.007.
http://www.sciencedirect.com/science/article/pii/S2253654X16300051.)

completely abated. At this point, the dose may be decreased by
5 mg/week if the symptoms remain quiescent and the eryth-
rocyte sedimentation rate does not increase. Cytoprotection of
the stomach mucosa should be considered because ulceration
and gastrointestinal bleeding are possible. If the patient cannot
tolerate corticosteroids, or the maintenance dose of steroids
remains so high as to produce adverse effects, azathioprine is a
reasonable next choice.
COMPLICATIONS AND PITFALLS
Failure to recognize, diagnose, and treat temporal arteritis
promptly may result in the permanent loss of vision. Failure to
diagnose the headache associated with temporal arteritis cor-
rectly may put the patient at risk if an intracranial pathological
condition or demyelinating disease, which may mimic the clin-
ical presentation of temporal arteritis, is overlooked. MRI of the
brain is indicated in all patients thought to have headaches asso-
ciated with temporal arteritis. Failure to diagnose glaucoma,
which also may cause intermittent ocular pain, may result in
permanent loss of sight.
CLINICAL PEARLS The diagnosis of headache associated with tempo-
ral arteritis is made by obtaining a thorough, targeted headache history. As
mentioned, jaw claudication is pathognomonic for temporal arteritis, and its
presence should be sought in all elderly patients presenting with headache.
Failure to recognize, diagnose, and treat temporal arteritis promptly may result
in the permanent loss of vision.
SUGGESTED READINGS
Bajkó Z, Bălaşa R, Szatmári S, et al. The role of ultrasound in the
diagnosis of temporal arteritis. Neurol Neurochir Pol. 2015;49(2):
139–143.
Dickason A, McArdle P. Jaw claudication in the presentation of
temporal arteritis: a review of the epidemiology and presenting
symptoms of 207 patients at Derriford Hospital, Plymouth. Br J
Oral Maxillofac Surg. 2015;53(10):e87.
Hazelman BL. Polymyalgia rheumatica. In: Waldman SD, ed. Pain
Management. Philadelphia: Saunders; 2009:449–454.
Paget SA, Spiera RF. Polymyalgia rheumatica and temporal arteritis.
In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadel-
phia: Saunders; 2007:1123–1127.
Rehak Z, Vasina J, Ptacek J, et al. PET/CT in giant cell arteritis: high
18F-FDG uptake in the temporal, occipital and vertebral arteries.
Rev Esp Med Nucl Imagen Mol. 2016;35(6):398–401.
Sloane J, Rice N, Kergozou E, et al. Temporal artery biopsy for giant
cell arteritis: an audit of 471 cases – what have we learnt? Br J Oral
Maxillofac Surg. 2015;53(10):e44.
Waldman SD. Connective tissue diseases. In: Waldman SD, ed. Pain
Review. 2nd ed. Philadelphia: Saunders Elsevier; 200916:43167–
44468.
Waldman SD. Temporal arteritis. In: Waldman SD, ed. Pain Review.
2nd ed. Philadelphia: Saunders Elsevier; 200916:22236–22328.
 13
Post–Dural Puncture Headache
ICD-10 CODE G97.1
THE CLINICAL SYNDROME
When the dura is intentionally or accidentally punctured, the
potential for headache exists. The clinical presentation of post–
dural puncture headache is classic and makes the diagnosis
straightforward if considering this diagnostic category of head-
ache. The diagnosis may be obscured if the clinician is unaware
that dural puncture may have occurred or in the rare instance
when this type of headache occurs spontaneously after a bout of
sneezing or coughing. The symptoms and rare physical findings
associated with post–dural puncture headache are due to low
cerebrospinal fluid pressure resulting from continued leakage of
spinal fluid out of the subarachnoid space.
The symptoms of post–dural puncture headache begin
almost immediately after the patient moves from a horizontal to
an upright position. The intensity peaks within 1 or 2 minutes
and abates within several minutes of the patient again assum-
ing the horizontal position. The headache is pounding in char-
acter, and its intensity is severe, with the intensity increasing
the longer the patient remains upright. The headache is almost
always bilateral and located in the frontal, temporal, and occip-
ital regions. Nausea and vomiting and dizziness frequently
accompany the headache pain, especially if the patient remains
upright for long periods. If cranial nerve palsy occurs, visual
disturbance may occur. Post–dural puncture headache is also
known as spinal headache.
SIGNS AND SYMPTOMS
The diagnosis of post–dural puncture headache is most often
made on the basis of clinical history rather than physical find-
ings on examination. The neurological examination in most
patients suffering from post–dural puncture headache is nor-
mal. If the spinal fluid leak is allowed to persist, or if the patient
remains in the upright position for long periods despite the
headache, cranial nerve palsies may occur, with the sixth cranial
nerve affected most commonly. This complication may be tran-
sient, but may become permanent, especially in patients with
vulnerable nerves, such as those with diabetes. If the neurolog-
ical examination is abnormal, other causes of headache should
be considered, including subarachnoid hemorrhage.
40
The onset of headache pain and other associated symptoms
such as nausea and vomiting that occurs when the patient
moves from the horizontal to the upright position and then
abates when the patient resumes a horizontal position is the
sine qua non of post–dural puncture headache (Fig. 13.1). A
history of intentional dural puncture, such as lumbar puncture,
spinal anesthesia, or myelography, or accidental dural puncture,
such as failed epidural block or dural injury during spinal sur-
gery, strongly points to the diagnosis of post–dural puncture
headache. As mentioned, a spontaneous postural headache that
manifests identically to headache after dural puncture can occur
after bouts of heavy sneezing or coughing and is thought to be
due to traumatic rents in the dura. In this setting, a diagnosis of
post–dural puncture headache is one of exclusion.
TESTING
Magnetic resonance imaging (MRI) with and without gado-
linium is highly accurate in helping confirm the diagnosis of
post–dural puncture headache. Enhancement of the dura with
low-lying cerebellar tonsils invariably is present. Poor visualiza-
tion of the cisterns and subdural and epidural fluid collections
also may be identified (Fig. 13.2).
No additional testing is indicated for a patient who has under-
gone dural puncture and then develops a classic postural headache,
unless infection or subarachnoid hemorrhage is suspected. In this
setting, lumbar puncture, complete blood cell count, and erythro-
cyte sedimentation rate are indicated on an emergent basis.
DIFFERENTIAL DIAGNOSIS
If the clinician is aware that the patient has undergone dural
puncture, the diagnosis of post–dural puncture headache is
usually made. Delayed diagnosis most often occurs in settings
in which dural puncture is not suspected. Occasionally, post–
dural puncture headache is misdiagnosed as migraine headache
because of the associated nausea and vomiting coupled with
visual disturbance. In any patient with dural puncture, infection
remains an ever-present possibility. If fever is present, immedi-
ate lumbar puncture and blood cultures should be obtained and
the patient started on antibiotics that cover resistant strains of
Staphylococcus. MRI to rule out epidural abscess also should be
considered if fever is present. Subarachnoid hemorrhage may
mimic post–dural puncture headache but should be identified
on MRI of the brain.
Another random document with
no related content on Scribd:
 “You are feeling so tired? It is the weather does it. Spring makes
you feel lazy. Have a smoke. You must feel lonesome, Danna,
stopping alone at Shiota? You must drop in to see me. The Tokyo-
born likes the Tokyo-born. Your talk won’t fall in with others. Does the
O-Jo-san come out to say nice things to you? The trouble with her is
she is all mixed up about right and wrong.”
“You were going to say something about O-Jo-san, and then you
went about scrubbing my head and I felt as it was coming off.”
“That was so. My head is so empty and I skip about so. I was
going to say that the priest fell head over ears in love with her.”
“The priest? What priest?”
“The priestling of Kaikanji, of course.”
“You haven’t said a word about a priest, full-fledged or half
fledged.”
“Haven’t I? I am so hasty, Danna. The priest, that priestling, was
good in looks, of a cast that girls like. This bozu(25) became, I tell
you, smitten by her of Shiota and at last wrote her a love letter....
Wait a bit, did he go at it himself? No, it was, he wrote.... Let me
see.... I am getting mixed up.... No, I am all right, I’ve got it ... so was
in fright and consternation.”
“Who was in fright and consternation?”
“The woman, of course.”
“By receiving the letter?”
“That would be a saving grace if she were. But she is not of the
kind to get scared.”
“Who was it really, then, that was in fright and consternation?”
“Why, he that spoke to her of his love.”
“I thought he did not go at it personally.”
“Oh, chuck it. It is all wrong. By receiving the letter, can’t you
see?”
 “Why then it must be the woman, who was in fright and
consternation.”
“No, the man.”
“If man, then it must be the priest?”
“Yes, the priest, of course.”
“But what scared him so?”
“What scared him? Why, he, the priest, was in the temple
assisting the abbot in the afternoon service. Then all of a sudden,
the woman rushed into the temple.... Lord, she must be off a great
deal.”
“Did she do anything?”
“‘If I am so dear to you, come let us make love before our all
mighty Buddha,’ she said and hugged him by the neck!”
“Ho?”
“Consternation was no word, for poor Taian, the priest. He got all
the shame he wanted by writing to a lunatic, and, disappearing that
night, he died.”
“Died?”
“At least I think he must have killed himself. He could not have
outlived the shame.”
“I don’t know about that.”
“Maybe he is still living somewhere. Death would not have come
out so well, the other party being a mad woman.”
“Very interesting, indeed.”
“Interesting is no word for it. Why the affair set the whole village a-
roaring with laughter. But the woman, being off her mind, was all
indifference as she still is. All will be well for a man so sober like you,
Danna; but the party being what she is, you don’t know what mess
you will get into, if you try to flirt with her.”
“I have got to be careful, eh? Ha, ha, ha!”
 The Spring breeze came lazily wafting from the genially warm
beach, and set the entrance curtain of the barber’s shop flapping
sleepily, and a swallow cast its flitting shadow in the mirror before
me, as it dived under the curtain with its body half turned. Under the
eve of a house on the other side of the road, a sexagenarian sat
squatting on a slightly raised seat, and was busy shelling bivalves in
silence. Every time a small knife went in between shells, a small
flabby lump fell into a basket, and the empty shells were thrown
away, two feet across the gossamer, there adding to the height of a
sparkling heap. Now and again the heap collapsed, sending the
oyster, clam, and other shells down into a small brook, to be buried
forever in its sandy bed. In no time the heap grew again under a
willow tree; but the old man was too busy to think of a life beyond
molluscs; he only went on throwing meatless shells upon gossamer.
His basket seemed to be bottomless and his Spring day endless.
The sandy stream ran under a twelve yard bridge, carrying the
warm water of Spring toward the sea shore. Down where Spring’s
water joined the tide of the sea, numberless fishing nets were drying
in the sun, hanging from erect poles of longer or shorter lengths and
were giving, one might suspect, a fish-smelling warmth to the soft
breeze wafting towards the village through their meshes. And one
saw between them the placid face of the sea, undulating slowly like
molten lead.
No harmony was possible between this scenery and my barber. If
he were a man of strong personality, strong enough to impress me
as powerfully as the scenery around, I should have been struck with
a sense of great incongruity. Fortunately, however, my man was not
so striking a character. However Tokyo-born, however high-spiritedly
he might talk, he was no match for the all genial and all embracing
influence of nature. My barber has been essaying to break up this all
subjugating power of nature with all his caustic effervescences; but
instead he has been swallowed up and was floating in the light wave
of Spring, not leaving a trace of the loud-talking Tokyo-born barber.
Inconsistency is a phenomenon to be found between persons or
things of equal standing, but possessed of hopeless incompatibility
in strength, spirit or physique. When distance is very great between
the two, inconsistency wears out, and will, instead, assume activity
only as part of a superior power. Thus it happens that cleverness
becomes a willing servant of greatness; the unintelligent of the
clever; and horses and cattle of the unintelligent. My barber is
making a comic exhibition of himself, with the beautiful scenery of
Spring for his background. He who tries to spoil the calm Springy
feeling is only adding to the profundity of that feeling. This man of
very cheap vaporing cannot but prove after all a colour in full
harmony with the Spring afternoon, which is symbolic of
gloriousness.
My man would make rather a good picture, and poetry, too, when
studied in this light, and I stayed talking with him long after my shave
was over, when a small head of a young priest put in an appearance,
slipping in by the entrance curtain and said:
“A shave please.”(26)
The newcomer was a jolly-looking little priest in an old-fashioned
grey cotton clothes, held together by a coil of light but cotton-wadded
belt, under a mosquito-net-like cloak.
“You got a scolding, didn’t you, the other day, for loafing, Ryonen-
san?”
“No, I was complimented.”
“Complimented for catching minnows on your way to an errand,
were you?”
“The Osho-san praised me, saying: ‘You did well Ryonen, to take
your time in play, young though you are.’”
“That accounts, eh? You have got many swellings in your head.
Too much trouble to shave a bumpy head like this; but I let you off
this time. Don’t come again with a freak of a head like this.”
“Thanks, I shall go to a better barber when my head is in good
shape.”
“Ha, ha, ha, this zig-zag thing has got a tongue to talk with to be
sure.”
 “Poor in work, but quite up in boozing, that is what you are, arn’t
you?”
“Poor in work? Say it again....”
“Not I, but it is the Osho-san who says it. Don’t get so mad, if you
know how old you are.”
“Humph, the idea! Isn’t that so Danna?”
“Ya—eh?”
“Priests, they live high above the stone steps, and have nothing
much to look after. That must be what makes them so free in tongue.
Even this little fellow can talk so. There lay down your head—lay
down, I say, do you hear? I will cut you if you don’t do as I tell you.
You understand? The red thing will run.”
“It hurts! Don’t be so rough.”
“If you can’t stand this sort of thing, how can you expect to be a
priest?”
“I am one already.”
“But not full feathered yet. Oh, say, by the way, how did Taian-san
die?”
“Taian-san is not dead.”
“Not dead? He must be dead.”
“Taian-san has got a new spirit and is now hard at his study at
Taibaiji temple in Rikuzen. Everybody expects he will make a great
priest by and by. A very good thing, indeed.”
“What is good? Priests may have their way; but it cannot be good
even for them to decamp at night? You ought to be careful, you
young one, it is woman who brings you trouble. Speaking of a
woman, does that crazy thing still come to the Osho-san?”
“I have never heard of a woman named ‘Crazy Thing’.”
“You blockhead, tell me, does she come or does she not.”
“No crazy woman comes; but Mr. Shiota’s daughter comes.”
 “The Osho-san may be great; but he won’t be able to make
anything of the poor girl. She is possessed by her former husband.”
“That lady is a very worthy woman. The Osho-san speaks highly
of her.”
“That beats all. Everything is topsy-turvy up there, above the
stone steps. Whatever the Osho-san may say, the mad must be mad
—Here now, all shaved. Hurry home and get another scolding.”
“No, not yet. I shall take little more time to get a good opinion of
the Osho-san.”
“Do as you please, you long-tongued brat.”
“Go on, you dry rot.”
“What!”
But the clean shaved head dived under and was on the other side
of the curtain, the Spring breeze softly fanning it.
 CHAPTER VI.
I sat at my desk, as the sun was going down. I had opened wide
all the paper screens and doors of my room. The people of the hotel
are not many, but its building is extensive. My room is far in the
interior, with many turns of passage, separating it from the quarters
inhabited by the not many people of the establishment, and no
sound comes to disturb my thinking. It has been especially quiet
today. I even fancied that the proprietor, his daughter, the young
maid and the man servant had all gone away unknown to me. Had
they done so, they could not, I thought, have gone to an ordinary
place; they must have flown to a land of hazes, or of cloud—so far,
far away that it may be reached only after floating lazily on the sea,
carelessly and too lazy to steer, until drifted to where the white sail
became indistinguishable from cloud or water, and indeed the sail
itself could not tell whether it was the cloud or water. Otherwise they
must have vanished, swallowed up in the spirit of Spring, the
elements of which they are composed returning to an invisible ether,
untraceable in the great expanse of space even with the help of a
microscope. They might have become the lark and flown to where
the evening dusk was deepening into purple, after they have sung
out the golden yellow of the rape flowers. Or else they might be
sweetly sleeping out the world a captive under a fallen camellia
flower, failing to steal its nectar, after having served to draw out
lengthily the long Spring day, by turning into a gad-fly. So quiet was
the day.
The Spring breeze passed freely through the empty house not
necessarily as a duty to those who welcomed it, nor yet out of spite
to those who resented it. It came naturally and went naturally, a
reflection of the impartial universe. With my chin resting in the palms
of my hands, my mind was as free and open as my room, and the
breeze would, uninvited, pass in and out with perfect freedom.
 You think of treading on, and you fear the earth might crack open
under you. You know the sky is hanging over you, and you dread
lightning might flash out and smite you. You are urged that you fail
your manhood unless you assert your antagonism and the world
becomes a place of endless trouble. To him that lives under the
firmament that has its East and West and has to walk on the rope of
interests, true love is one’s enemy and visible wealth dirt. A name
made and honour won may be likened unto honey which wise bees
leave behind by forgoing their sting, after making it appear that they
were manufacturing and sweetening it. The so-called pleasures all
come from love for things and contain in them all kinds of pain.
However, the world happens to have its poet and artist, who feed on
the essence of this world of relativity, and knows the absolute
principle of purity. They dine on heaven’s haze and quench their
thirst with dew. They talk of purple and discuss crimson, and no
regret detains them when death comes for them. Their pleasures are
not to become attached to things, but to become themselves part of
things. When they have become things themselves, they find no
room for their ego though they may explore the remotest confines of
the earth. They rise above worldly dirt and drink full of the boundless
air of purity. These things are said not merely to scare those
saturated with the odor of the lucre of the city, and alone to pose
loftily, but to convey the gospel contained in them and to invite their
brother beings to share in its blessings. To tell the truth, art and
poetry are principles all men are born with. Most people, who, having
counted their summers, are now living their grey winter, will be able
to reawaken their past with its joys of seeing light shining in them. He
who is unable to recall such a memory is one who has lived a life not
worth living.
I do not say that the poet’s joys consist in giving oneself
exclusively over to one thing, or surrendering ourselves solely to
another. He may, at one time enter and become a solitary flower, or
turn into a butterfly at another. Or like Wordsworth become a field of
daffodils, with his heart thrown into confusion by the wind.
Sometimes again he becomes lost in a scene and is unable clearly
to tell what is it that has captured his heart. Some people may call
this being possessed by nature. Others may describe it as the heart
listening to a music of an unstringed harp. Still others may see in it a
lingering in boundless regions or wandering in a limitless expanse,
being unable to know or to understand. Say what they like, they are
perfectly free to do so. I was precisely in this state of mind as I sat
resting half of my weight on my bent elbow that rested on my desk,
with my head perfectly vacant otherwise.
It was unmistakably clear that I was thinking nothing, looking at
nothing. I could not be said to have become or turned into anything,
as there was nothing of striking colours moving within my world of
consciousness. Nevertheless I was in motion. I might not be moving
in the world; but I was anyhow in motion. Not moved by a flower, not
moved by birds, not moved against humanity, still moving as in a
spell.
If I must explain this state of being somehow less oracularly, I
should say that my soul was moving with the Spring. I should say
that an etherial essence, obtained by compounding all the colours,
forces, substances and sounds of Spring into an esoteric electuary,
then by melting it in dews gathered in the land of immortals, and by
finally evaporating it in the sunshine of fairyland, found its way into
my pores before I became aware, and put me into my present state.
Ordinarily an absorption is accompanied by a stimulus, which will
make the process pleasant. In my case, how I came to it was quite
hazy no stimulation accompanying it. Because of this absence of
stimulation, there was something indescribably profound in my joy,
which was quite different from those that are transparent and noisy,
and occasion superficial excitement. Mine might be likened unto a
great expanse of ocean, moving from its unseen fathomless depth
from continent to continent, except that there was not quite as much
active vitality. But I was the more fortunate because of this
deficiency, as the manifestation of great vitality must needs
anticipate its exhaustion some day. There is no such worry in the
normality of things. My mind was presently in a state more airy than
normal and I was not only free from all anxiety that strong activity
might wear out, but I was above the common level of indifferent
normality. By airiness I mean simply elusiveness, and do not imply
any idea of over-weakness. Poets speak of melting airiness or
downy lightness, and the phrase exactly fits the condition I am
describing.
I wondered next, how it would work to make a picture of my fancy.
I doubted not for a moment that it would not make an ordinary
picture. An every-day painting is a mere reproduction, on a piece of
silk or canvas of what one sees around, as it is, or else after filtering
it through an æsthetic eye. A picture has done its part, when a flower
looks the flower it is, the water as it reflects in the eye, and human
characters animate as in life. If one is to rise above this common
level, one must let live on the canvas one’s theme, with touches that
utter sentiments exactly as one feels about it. Since the artists of this
order aim at working the special impressions they have received into
the phenomena they have caught, they may not be said to have
produced a picture, unless their brush speaks, at its every sweep, of
those impressions. Their work must bear out their claim that their
manner of perceiving this way and feeling that way has in no way
been influenced by or borrowed from old traditions or those going
before them, but that nevertheless theirs is the most correct and
beautiful. Otherwise they are not entitled to call the work their own.
Workers of the two classes are one in waiting for definite outside
impulses before they take up their brush, whatever differences there
may be in their depth and in the manner they treat their subjects. But
in my case, the subject I wished to treat was not so clearly defined. I
roused my senses to the highest pitch of wakefulness; but I looked in
vain for a shape, colour, shade, and lines thick and thin, in the
objects without me, to suit my fancy. My feelings had not come from
without; but even if they had, I could not raise my finger and point at
their cause as such distinctly, as they formed no definite object of
perception. All that there were, were only feelings, and the question
was how those feelings might be depicted to make a picture, nay
how I might give them expression so that others might, by looking at
my production, feel as I was feeling as nearly as possible.
An ordinary picture requires no feeling, but only the object to
reproduce. A picture of a higher order necessitates there existing the
object and feeling; one of the class still higher has nothing for its life
but feelings, and an object that will fit in with such feelings must be
caught to make a picture. But such an object is not easily
forthcoming, and even if it came, it would be no easy work to arrange
it appropriately. Even if arranged successfully, its presentation would
take such a form as would sometimes make it appear totally different
from anything in nature, so much so that it would make no picture at
all for ordinary people. The artist himself would not recognise that his
production represented anything in existence, but that his was only
an attempt to convey, however fractionally, his feelings at the very
moment when his fancy was aroused. He would consider it a most
creditable achievement if he, after scouring the length and breadth of
the country, with not a moment of forgetfulness, comes suddenly, at
the cross roads upon his lost child and folds it in his arms, not giving
time even for lightning to flash, saying, “Why you were here, my
child.” But that is where the rub comes in. If I can only work out this
tone, I shall not care what others may say of my picture. I shall, with
the least concern, let them say it is not a picture at all. If my
combination of colours, gave expression to my feelings even in part;
if the straight and curve of the lines spoke for a fraction of this spirit;
if the general disposition of the picture conveyed any of the
superprosaic thoughts, I shall not mind if the thing to assume a
shape in the picture should happen to be a horse, or a cow or
something neither a horse nor a cow. No, I shall not mind; but the
trouble was nothing would come forth to fit my fancy. I laid my sketch
book open on my desk and looked down upon it until my eyes almost
fell through it. It was useless.
I laid my pencil aside and thought; thought it was a mistake, to
begin with, that I should have tried to make a picture of abstract
feelings. Men are not so different from one another and there must
have been some who have had the same touch of thought as I have
and must have tried to perpetuate such feelings by some means or
other. By what means I wondered.
Music! The word flashed across my mind. Yes, music must be the
voice of nature, born under such necessity, under such
circumstances. It occurred, for the first time, to me, then, that music
is something that must be listened to and that must be learned.
Unfortunately, I am a perfect stranger to music.
 I wondered next if my fancy would not make poetry, and ventured
to step into the third dominion. In my memory, it was an individual
named Lessing, who arguing that the province of poetry are events
that occur conditioned on the passing of time, established the
fundamental principle that poetry and painting are not one but two
different arts. Seen in this light, poetry seems to give little promise of
making anything out of the situation of things, to which I have been
struggling to give expression. The physical condition of my feeling of
joy may have in it the element of time, but does not consist of an
event that progressively developed in the flow of time. My joy is
joyous not because No. 1 goes away and No. 2 comes in its place,
and not because No. 3 is born as No. 2 vanishes. I am joyful
because my joy is felt deeply and retained from the beginning. Say
this in an every-day language, and there will be no need of making a
factor of time. Poetry, like painting, will come of things arranged
separately. Only what scene and sentiment to bring into the poetry,
to portray this expansive and abandoned condition is the question.
Poetry should be forthcoming, in spite of Lessing, so soon as these
factors are caught. Homer and Virgil may be let alone. If poetry be fit
to give voice to a mood, that mood may be painted in words without
being under time restrictions and unaided by an event that
progresses in an orderly manner, as long as the simple spatial
requirements of painting are fulfilled.
The point of my pencil began to move slowly, very slowly at first,
then with more speed on my sketch book and in half an hour I got
these lines:

“Spring two or three months old,

Sadness is long as sweet young plants.
Flowers fall on the empty garden,
In the soulless hall lies a plain harp.
Immobile the spider in its maze hangs
Winding travels blue smoke up the bamboo beams.”

Reading the six lines over, I thought each of them might make a
picture and wondered why I had not set about drawing from the first.
I discussed with myself why it is easier to poetise than to paint.
Having come so far, I felt the rest ought not to be so very difficult to
follow, though a desire seized me that I should now sing a sentiment
that defied colour and brush. The squeezing my head this way and
that way yielded more lines:

“Not a word uttered sitting alone,

But a small light I see in the heart.
Unwontedly troublesome are human affairs.
Who shall forget this state?
Enjoying one day’s quiet
I know now how I passed hundreds of busy years.
My yearning, where shall I communicate?
Far, far away, in the land of white clouds.”

I read the whole piece over again. It was not so very poor; but as
a depiction of ethereal conditions I had just experienced, I felt
something still wanting. I might try to compose one more piece, and
with the pencil still between my fingers, I happened to look out of the
opened door way of my room to see at beautiful vision flitting across
the three feet space. What could it have been?
I now turned my eyes fully toward the doorway and the vision had
half disappeared behind the screen that stood pushed to one side. It
had apparently been moving before it caught my eyes, and had now
gone out of sight altogether as I stared in amazement toward it. I
stopped composing poetry, and instead I now kept my eyes fixed on
the open space in the doorway.
The clock had not ticked a full second when the vision returned
from the opposite direction to that which it had disappeared. It was
that of a slim woman in a wedding gown with long sleeves, walking
gracefully along the upstairs verandah of the wing of the hotel,
flanking my room. I did not know why, but the pencil fell from my
fingers, and the breath I was inhaling through my nose stopped of its
own accord. The sky was darkening, as if forewarning one of the
cherry season showers, to hasten the evening dusk; but the gowned
figure kept on appearing and disappearing in the heavily-charged
atmosphere, walking with benign gentleness along the verandah,
twelve yards away from me, overlooking an inner court.
The woman said nothing, nor looked either way. She was walking
so softly that the rustling of her silk gown seemed scarcely to catch
her ears. Some figures—I could not tell what from the distance—
adorned the skirt of her dress, and the figured and unfigured parts
shaded into each other like day into night. And the woman was
indeed, walking in the borderland of night and day.
What mystified me was what made her go so persistently to and
from along the verandah, dressed in her long-sleeved gown. Nor had
I any idea of how long she had been at this strange exercise in her
strange attire. There was, of course, no telling of her purpose. This
figure of a woman, appearing and disappearing across the open
doorway, repeating the incomprehensible movements, could not help
arousing a singular feeling in me. Could it be that she was moved by
her regret for the departing Spring, or how could she be so
absorbed? If so absorbed, why should she be dressed in such
finery?
That resplendent obi, that stood out so strikingly in the hue of
departing Spring, lingering at the threshold of gathering dusk, could it
be gold brocade? I fancied the bright ornament, moving backward
and forward, enshrouded in the gray of approaching night, was like
glittering stars in the early dawn of a Spring day; that every second
went out one by one, in this distant depth and then in that of the vast
vault of heavens, vanishing gradually into the deepening purple.
Another fancy struck me as the door of night was gradually
opening to swallow into its darkness this flowery vision. Super-
nature! this sight of fading away from the world of colours, with not a
sign of regret, nor of struggle, instead of shining an object of
admiration in the midst of golden screens and silver lights. But there
she was with the shadow of darkness closing in on her, pacing up
and down rhythmically, the very picture of composure, and betraying
no disposition to hurry or dismay, but calmly going over the same
ground again and again. If it be that she knew not the blackness
falling upon her, she must be a creature of extreme innocence. If she
knew but did not mind it as blackness, then, there must be
something uncanny about her. Black must be her native home, and
thus may she be resignedly surrendering her visionary existence to
return to her realm of darkness, walking so leisurely between the
seen and unseen worlds. The inevitable blackness into which the
figures adorning her long-sleeves shaded seemed to hint where she
had come from.
My imagination took another turn, bringing before me a vision of a
beautiful person, beautifully sleeping. Sleeping, alive she breathes
herself away into death, without ever awakening. This must break
the heart of those watching anxiously around the bed. If struggling in
pain and agony, the dear ones attending might think it merciful that
death came at once, to say nothing of the wish of the patient to
whom life had become not worth living. But what fault could the
innocent child have been guilty of that she should be snatched away
in a peaceful sleep? To be carried away to Hades while in sleep is
like being betrayed into a surprise and having life taken before the
mind is made up. If death it must be, the dying should be made to
resign to Fate, and one should like to say a prayer or two, yielding to
the inevitable. But if the fact of death alone was made clear, before
its conditions had been fulfilled, and if one had a voice to say a
prayer, one would use that same voice in hallooing, to call, even
forcibly back, the soul that has put one step in the other world. To
one passing away in sleep, it may be hard to have the soul called
back, pulled back, as it were, by the bond of worries of life that would
otherwise break, and that one may feel like saying: “Don’t call me
back; let me sleep.” Nevertheless those around would wish to call
aloud. I thought I might call that woman in the verandah the next
time she came into view, to wake her up from her waking sleep. But
my tongue lost its power of speech no sooner had she passed the
opening like a dream. Without fail, the next time, I thought. But again
she passed and disappeared before I could utter a word. I was
asking myself how this could be, when again she passed, and
appeared not to care a rap that she was being watched by one who
was in a frenzied state of mind about her. She passed and repassed
in a manner that told that one like me had never at all entered her
mind. As I was repeating my “next time” in my mind, the dark cloud
above let down, as if no longer able to hold back, a screen of fine,
soft rain, dismally shutting out the shadow of the woman.
 CHAPTER VII.
Chilly! With a towel in hand I went down stairs for a warm dip.
Leaving my clothes in a small chamber, four more steps downward
brought me into the bath room which was about eight mats in size.
Stones appeared plentiful, in these parts, the floor of the room being
paved with fine granite, as was also the tank and its walls. The
reservoir which the tank really was, was a hollow in the centre of the
floor, about four feet deep and about as many feet square. This was
a hot spring which contained, no doubt, various mineral ingredients;
but the water in the basin was perfectly clear and transparent, and
tasteless and without odour as well, as some finding its way into the
mouth testified. The spring is said to possess medical virtues, but I
did not know for what kind of ailments, as I have not taken the
trouble to find out. Nor was I subject to any chronic disease, and this
phase of the matter had never occurred to me. Only a line of poetry
that comes to me, every time I take a dip is that of the Chinese poet
Pai Le-tien:

“Soft and warm the water of the spring,

All impurities are cleansed away.”

A mention of a spring awakens in me the pleasant feeling which

this couplet expresses, and I hold that no hot spring deserves to be
called by that name unless it makes one feel that way. This is an
ideal, apart from which I have no demand to make of any hot spring.
Clear up to a little under my chin the pleasant warm water in the
tank reached and was indeed overflowing beautifully on all sides,
without making it known where it was welling up from.
Resting my head, with face upward, on the back of my hands
which held on to the slightly raised side of the tank, I let my body rise
up to the point of least resistance and I felt my soul float buoyantly
like the jelly-fish. Life is easy in this state of existence. You unlock
the door of prudence and cast all desires to the Four Winds, and
become part of the hot water, leaving it completely to the hot water to
make what it likes of you. The more floating, the less is the pain to
live for that which floats. There will be more blessings than to have
become a disciple of Jesus Christ, for one who lets even one’s soul
float. At this rate, even drowning is not without its picturesqueness. I
have forgotten what piece, but I think I remember reading
Swinburne, where the poet depicts a woman rejoicing in her eternal
peaceful rest. Millais’ “Ophelia,” which has ever been a source of
sentimental uneasiness to me, offers also something æsthetic, when
viewed in this light. Why he should have chosen so unpleasant a
scene has always been a puzzle to me; but now I saw that it made
an artistic production. A form, a figure, a look, floating in sweet
painlessness, as it were, whether on the surface, or under water or
floating and sinking, is indisputably æsthetic. With wild flowers
judiciously sprinkled on the banks and the water, the floating one,
and the floating one’s dress, making a harmonious and well
arranged ensemble of colours, it will without fail make a picture. But
if the floating one’s expression were nothing but peace itself, the
picture would almost make only a mythology or an allegory, while
convulsive pain will, on the other hand, destroy the whole effect. The
expression of a naive and care-unknown face will not bring out
human sentiments. What kind of a face should it be to be a success?
Millais’ “Ophelia” may be a success; but I doubt that he is one with
me in spirit. However, Millais is Millais and I am I; and I feel like
painting a person drowned. But I fancied that the face that I wanted
would not easily come to me.
Buoying myself in the bath, I next tried to make poetry of the
appreciation of the drowned:

“Will get wet in rain,

Will be cold in frost,
Will be dark underground,
On the wave when floating,
Under the wave when sunk,
Will be painless in warm Spring water.”
 It was raining outside, the soft, quiet, warm rain of Spring. The
plaintive “twang,” “twang” of a samisen heard at a distance on a
night like this is a peculiarly appealing sound, and it was catching my
ear, as I was humming my extempore song of the drowned. Not that
I pretend to know anything about this particular instrument of string
music; in fact I am rather dubious about my ear being able to tell any
difference of a higher or lower pitch of the second or third string.
Nevertheless, with a gentle mercy-like rain putting me in this fame of
mind, and even my soul lazily sporting in a delightfully pleasant
warm bath, it gladdened my heart to hear floating music, with not a
shadow of care within or without me.
The samisen awoke in me the long-forgotten memories of my
boyhood days, when I used to go out into my father’s garden and sit
under three pine trees, to listen to O-Kura-san, the fair daughter of a
saké shop on the other side of the street, sing and play a samisen on
calm Spring afternoons.
I was lost in living over again the long past, when the door of the
bath room opened. I thought somebody was coming in. Leaving my
body buoyant, I turned my eyes only toward the entrance. I had my
head resting on that part of the tank which was farthest from the
entrance door, so that my eyes covered obliquely the steps leading
downward, about seven yards away from me. Nothing as yet
appeared before my uplifted eyes: my ears caught only the sound of
the rain dropping from the eaves. The plaintive samisen had stopped
I did not know when. Presently something appeared at the head of
the steps. There was in the room a solitary small hanging oil lamp,
which, even at its best, shed but scanty light, to make things clear in
their colour; but what, with the rain outside shutting in the vapours,
and the whole place filled with a cloud of mist, there could be no
telling who it was coming down.
The dim figure carried its foot a step down; but one might have
fancied that the step stone was velvety smooth and its stepping so
noiseless that it could not have moved at all. The figure became
clearer in outline, and an artist as I am, my perception of the build of
a body is more accurate than you might have thought, so that, the
moment it came a step down, I knew that I was alone with a woman
in the bath room. The woman came fully in view before me as I was
debating with myself whether I should or should not take any notice
of her. The next moment I was lost to all but a beautiful vision. The
figure gracefully straightened itself to its full height, with the soft light
of the lamp playing about the warm light pink of the upper regions,
over which hung a cloud of dark hair. The sight swept away from me
all thoughts of formality, decorum and propriety, my only
consciousness then being that I had before me a superbly beautiful
theme.
Be the ancient Greek sculpture what it is, every time I see a nude
picture, which modern French painters make their life of, I miss
something in its unuttered power of impression, because of the
voluptuous extremes to which effort is made in order to bring out the
beauty of the flesh. This feeling has always been a source of mental
uneasiness to me, as I could not answer myself exactly why, pictures
of this class looked low in taste, as I think they do.
Cover the flesh, its beauty disappears; but uncovering makes it
base. The modern art of painting the nude does not stop at the
baseness of uncovering; but not content with merely reproducing the
figure stripped of its clothing, would make the nude shoulder its way
into the world of decorum and ceremony. Forgetting that being
wrapped in clothes is the normal state of human life, they are trying
to give the nude all the rights. They are striving to bring out strongly
the fact of being stark-naked, emphasizing the point excessively,
indeed, over-excessively beyond fullness. Art carried to this extreme
debases itself, in proportion as it coerces one who looks at it. The
beautiful begins, as a rule, to look the less beautiful, the more
beautiful it is struggled to make it appear. This is precisely what, in
human affairs, gives life to the proverb, “fullness is the beginning of
waning.”
Care-freeness and innocence generally present something
comfortably in reserve, which latter is an indispensable condition in
paintings as in literature. The great failing of modern art is its
labouring in the mud, which the so-called tide of civilization is
depositing everywhere. The painting of the nude is a good example
of it. In Japanese cities are what are called the geisha, who traffic in