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Distal and Generalized Sensory Symptoms
Distal and Generalized Sensory Symptoms
Pain is the sensory symptom which most frequently brings a patient to neurologic
attention. The first step in evaluating pain is to determine its character. Neuropathic
pain has sharp, burning, or electrical qualities. Nociceptive pain, by comparison,
has dull and aching qualities. While cold or freezing sensations may be due to neu-
ropathic processes, these sensations are also often the result of vascular insuffi-
ciency. In general, pain may be divided into focal (Chap. 16) and generalized or
distal-predominant (this chapter) in distribution. Because patients may report only
the most prominent location of symptoms, you must inquire specifically about pain
involving the face, scalp, trunk, back, arms, hands, groin, legs, and feet.
Numbness is the second important symptom of sensory dysfunction. When
patients use the term numbness, they may mean one of several different sensations:
a lack of feeling or deadness, a sensation that the affected body part is covered by
something such as a glove, sock, or extra layer of skin, or a sensation that the body
part is asleep. Some patients will report that their socks are bunched up around their
feet, that they are walking on sponges, or that the hands or feet are buried in cement.
Be aware of the common, erroneous description of limb weakness as numbness.
Paresthesia is a sense of tingling, pins and needles, abnormal vibration, or a feel-
ing that a body part is asleep. Some patients with paresthesia may describe it ini-
tially as pain or numbness, and an accurate description of the problem may be
obtained only by specifically asking about a perception of pins-and-needles.
Paresthesia is more specific for neurologic dysfunction than any other sensory
symptom.
There are several abnormal sensations which are actually due to motor dysfunc-
tion. The most common of these are cramps, twitching (fasciculations), and
restlessness.
It is important to note that because sensory symptoms are entirely subjective,
they can be feigned or exaggerated quite easily. Important clues to factitious sen-
sory symptoms include a large number and variety of symptoms, requests for
Pain and temperature sensations from the limbs and trunk are mediated by small-
diameter nerve fibers. These fibers travel through the nerves and nerve roots, reach-
ing their cell bodies in the dorsal root ganglia. After entering the spinal cord, pain
and temperature fibers ascend one or two segments before decussating in the ventral
white commissure. They ascend through the spinal cord in the spinothalamic tract,
eventually reaching the ventroposterolateral nucleus of the thalamus. These fibers
from the thalamus project to the insula.
Vibratory and proprioceptive sensations are mediated by large-diameter nerve
fibers. These fibers also travel through the nerves and nerve roots to reach cell bod-
ies in the dorsal root ganglia and enter the spinal cord. Within the cord, fibers from
the lower extremity travel through the gracile fasciculus while those from the upper
extremity travel through the cuneate fasciculus. These fibers reach the gracile and
cuneate nuclei in the medulla and decussate in the medial lemniscus. Lemniscal
fibers synapse in the ventroposterolateral nucleus of the thalamus and project to the
primary sensory cortex of the postcentral gyrus.
Sensory Examination
Sensory examination should include tests of pinprick, vibration, position sense, and
cortical modalities.
Pinprick examination of every square inch of the skin is time consuming and leads
to a great deal of extraneous and usually misleading information. There are several
strategies which improve both the diagnostic yield and efficiency of this portion of
the sensory examination. First, take a thorough history in order to get a sense of the
likely distribution of sensory deficits. The examination of a patient with a probable
length-dependent polyneuropathy requires a different strategy than the examination
of a patient with a mononeuropathy or monoradiculopathy. It is often quite helpful
to ask the patient to trace the area of cutaneous sensory loss before initiating the
sensory examination. When testing pinprick, begin by examining a completely nor-
mal area to establish that the patient can perceive the sharp sensation of a pinprick
accurately. Next, test an area that is suspected to be abnormal based on the
Sensory Examination 231
a b
AXILLARY
C3
C4 RADIAL
C5 T2
MEDIAL
T3
BRACHIAL
T4
T1 CUTANEOUS
T5
T6 MEDIAL
T7 ANTEBRACHIAL
T8 CUTANEOUS
C8 T9
C6 T10 MUSCULOCUTANEOUS
T11
T12
L1
ULNAR
SACRAL PUDENDAL
C7 L2 MEDIAN
L3
LAT.FEMORAL
CUTANEOUS OBTURATOR
FEMORAL
L4
SUPERFICIAL
PERONEAL
SAPHENOUS
S1 L5
SURAL
DEEP
PERONEAL
Fig. 15.1 Cutaneous sensory distributions of the nerve roots and individual nerves on the ventral
surface of the body. Note in particular the T2 (axilla), T4 (areolae), and T10 (umbilicus) derma-
tomes. See Fig. 16.1 for additional details on the innervation of the hand
information gained from the history, and march the pin from the abnormal area back
into the normal area. For example, in a patient with a suspected length-dependent
polyneuropathy, begin distally at the toes to see whether the patient can detect the
pinprick, and then march the pin proximally, determining where the patient first
perceives the sensation properly. More subtle deficits require a different technique.
In a patient with mild sensory abnormalities, it is helpful to use one body part as a
control and compare it to the possibly abnormal part. For example, when testing a
patient with a possible right L5 radiculopathy, poke the dorsum of the left foot with
the pin and tell them that this sensation is 100 %. Next, poke the dorsum of the right
foot and ask the patient to assign a percentage to the strength of this sensation.
When examining pinprick for the purposes of a screening examination, establish
that sensation is preserved by testing two or three pinpricks in each dermatome and
nerve distribution (Fig. 15.1).
Because pinprick and temperature perception are both mediated by small-
diameter nerve fibers, examining temperature usually does not add much new infor-
mation to the neurologic examination. False positive abnormalities in cold sensation
testing are frequent in patients with cold extremities. In addition, it may be difficult
to find standardized cold and warm stimuli. Despite these limitations, if pinprick
sensation is entirely preserved and there is a high index of suspicion for small-diam-
232 15 Distal and Generalized Sensory Symptoms
eter nerve fiber dysfunction, testing perception of warmth and coldness may help to
disclose an important deficit. Temperature testing is also useful for patients who do
not tolerate or cannot accurately report the results of the pinprick examination.
Examine vibratory sensation with a 128-Hz tuning fork. Absence of vibratory per-
ception at the great toe is clearly abnormal. If the patient cannot appreciate vibration
at the great toe, move the tuning fork to the medial or lateral malleolus, to the knee,
and then to the sternum until they feel the vibration. The normal duration of tuning
fork perception is not clearly defined. Some use the very restrictive criterion that
both patient and doctor should feel the vibratory perception for the same length of
time. Others use cutoffs such as 10 or 12 s of vibratory perception at the great toe.
Because none of the techniques for vibratory perception have been standardized,
experimentation is necessary to establish a personal standard of normal and abnor-
mal. My technique involves striking the tuning fork vigorously so that its heads hit
each other, then placing the base of the tuning fork on the interphalangeal joint of
the great toe. Using this technique, the approximate number of seconds of vibratory
perception at the great toe may be computed by subtracting the first digit of the
patient’s age from 16. For example, a 49-year-old patient should have 12 s of vibra-
tory perception at the great toe.
Joint position examination generally offers little more information beyond
what is obtained by testing vibratory perception. To test joint position, instruct the
patient to close their eyes and tell them that you will move their great toe either
upward or downward. Grasp the toe by the sides (not by the top and bottom) and
move it by no more than 5 mm. Normal subjects should be able to detect this
movement reliably.
The most commonly tested cortical sensory modalities are graphesthesia, stereog-
nosis, and two-point discrimination. These higher-order sensory functions are con-
sidered functions of the contralateral parietal lobe. Accurate testing of these
modalities may occur only when the patient has otherwise preserved gross touch
and pinprick sensation. Test graphesthesia by instructing the patient to close their
eyes and then asking them to interpret a number that you trace on their palm. Trace
at least five different numbers (2, 3, 6, 7, and 8 are the most straightforward ones to
interpret) on one palm, and compare the results to the other palm. Test stereognosis
by asking the patient to distinguish between a quarter, dime, and nickel placed in
each hand. Most patients can do this easily. Finally, test two-point discrimination by
Polyneuropathy: Length-Dependent 233
placing the tips of a caliper against the patient’s skin. Determine whether they can
discriminate between one point and two points separated by 5–10 mm. Abnormal
graphesthesia, stereognosis, and two-point discrimination point to dysfunction in
the contralateral parietal lobe, but should not be judged too strictly, as false-positive
test results are common.
Polyneuropathy: Length-Dependent
Polyneuropathy: Non-length-dependent
Evaluation of Polyneuropathy
Laboratory Screening
The purposes of EMG and NCS are to confirm the presence of a polyneuropathy, to
determine its severity, and to distinguish between axonal and demyelinating forms.
Although localization of sensory complaints to the peripheral nerves is often quite
straightforward, diagnostic mimics including disorders of the central nervous sys-
tem, polyradiculopathy, and psychogenic disorders make EMG and NCS helpful in
confusing cases. While neuropathy severity is often obvious from clinical history
and examination, electrodiagnostic studies may offer insight into disease severity
which becomes important when determining appropriate treatment. Distinguishing
between axonal and demyelinating pathologies is perhaps the most important role
of EMG and NCS. Axonal polyneuropathies are characterized by decreased
response amplitudes with normal or mildly reduced conduction velocities, whereas
demyelinating polyneuropathies are characterized by relatively preserved response
amplitudes and severe slowing of conduction velocities. The distinction between
these two sites of nerve pathology is important, as demyelinating polyneuropathies
often respond to immunomodulatory therapy while axonal polyneuropathies do not.
EMG and NCS should be employed judiciously, as their sensitivities and specifici-
ties are both limited. Because NCS assesses only large-diameter nerve fibers, it is
frequently normal when pain (mediated by small-diameter nerve fibers) is the only
symptom. Although many EMG studies offer little in the way of additional diagnos-
tic information for polyneuropathy, the following factors always prompt me to
order EMG:
Nerve Biopsy
Sural nerve biopsy may offer additional diagnostic information for several different
and uncommon etiologies of polyneuropathy including vasculitis, amyloidosis,
tuberculosis, and sarcoidosis. In most cases, nerve biopsy shows nonspecific axon
loss which offers little diagnostic information beyond what is provided by the clini-
cal examination and electrodiagnostic studies. Skin-nerve biopsy may help to con-
firm the diagnosis of a small-fiber neuropathy, but does not offer specific information
that is helpful in patient management.
236 15 Distal and Generalized Sensory Symptoms
Etiologies of Polyneuropathy
A detailed discussion of the vast number of causes of polyneuropathy can and does
fill a multivolume textbook (Dyck et al. 1993). The following is a summary of some
of the most common and important causes of polyneuropathy:
Diabetes Mellitus
Diabetes mellitus is the most common cause of polyneuropathy in the USA. It may
take many forms, including a length-dependent sensorimotor polyneuropathy, a
painful small-fiber polyneuropathy, diabetic thoracic radiculopathy (Chap. 17), dia-
betic amyotrophy (Chap. 16), mononeuropathy multiplex (Chap. 11), or a syndrome
which resembles chronic inflammatory demyelinating polyneuropathy (Chap. 10).
Although elevated blood hemoglobin A1c percentages are often used to confirm the
presence of diabetes in patients with suspected diabetic neuropathy, oral glucose-
tolerance testing is more sensitive (Sumner et al. 2003). Because diabetes is so com-
mon, it is important to not wear blinders by assuming that diabetes is the sole
explanation for neuropathy in a diabetic patient: always conduct a thorough evalua-
tion to exclude other reversible causes. Tight glycemic control may arrest progres-
sion of neuropathy symptoms, but reverses symptoms only rarely. Strict and rapidly
achieved euglycemia may lead to “insulin neuritis” or treatment-related diabetic
neuropathy in which neuropathy symptoms, particularly severe pain, worsen within
2 months of establishing better blood sugar control (Gibbons and Freeman 2010). In
most cases, agents for neuropathic pain are the mainstay of therapy. Treatment with
α-lipoic acid (600 mg qd-tid) may help in some cases (Ziegler et al. 2006). One
additional critical aspect of diabetic neuropathy care is prevention of foot ulcers
with properly fitting shoes, careful daily foot examinations, and periodic podiatric
assessments.
B12 deficiency may affect the brain, optic nerves, spinal cord, and peripheral
nerves. In a small percentage of patients, the peripheral nerves are affected in
isolation. Because the spinal cord is involved in up to 90 % of cases with
neurologic involvement, B12 deficiency is discussed further in Chap. 17 (Puri
et al. 2005).
Etiologies of Polyneuropathy 237
Monoclonal Gammopathy
Vasculitis
Ethanol
Charcot–Marie–Tooth Disease
HIV
Thyroid Dysfunction
Medication-Induced Neuropathies
Idiopathic
After extensive screening, many patients with polyneuropathy still lack an identifi-
able cause. Patients with idiopathic polyneuropathy tend to have mild disease which
progresses slowly over years. While there is no specific treatment to reverse idio-
pathic polyneuropathy, patients do get symptomatic relief from agents for neuro-
pathic pain.
Sensory symptoms fall into two classes: positive symptoms in which there is an
emergence of an abnormal sensation such as pain or paresthesias, and negative
symptoms in which sensorimotor function is attenuated or lost such as numbness
or weakness. Broadly speaking, medications are available to mask positive
240 15 Distal and Generalized Sensory Symptoms
symptoms, but unless a reversible cause is identified, there are few treatments for
negative neuropathic symptoms. A wide variety of agents is available to treat
neuropathic pain and paresthesias (Table 15.3). Note that because all of the oral
medications work by reducing the firing of abnormal nerve impulses, the firing
of normal ones may also be suppressed, leading to sedation and other cognitive
side effects.
Other Causes of Distal Sensory Symptoms 241
Spine Disease
Plantar Fasciitis
Inflammation of the plantar fascia is a source of foot pain that may mimic polyneu-
ropathy when it is bilateral. Wear and tear damage to the plantar fascia is particu-
larly common in obese people and in runners, and leads to pain which is most severe
with the first steps in the morning. Pain is most often centered at the medial calca-
neus, but may involve the entirety of the sole. On physical examination, the pain of
plantar fasciitis may be reproduced by dorsiflexing the toes and palpating the plan-
tar fascia. Treat plantar fasciitis with nonsteroidal anti-inflammatory medications,
gentle physical therapy, and in refractory cases, local steroid injections. Although
patients should avoid activities that precipitate pain, it is usually impractical for
patients to comply with instructions to stay off of their feet.
The tarsal tunnel is formed by connective tissue posterior to the medial malleolus.
The tibial nerve passes through the tarsal tunnel in order to reach and innervate the
sole. Compression of the tibial nerve within the tarsal tunnel, therefore, may lead to
numbness, pain, and paresthesias in the bottom of the foot. Tarsal tunnel syndrome
should not be considered the lower extremity analog of carpal tunnel syndrome
(Chap. 16), though, because it is quite uncommon and occurs almost always in the
setting of serious ankle trauma rather than as a gradual-onset process secondary to
overuse. Polyneuropathy and plantar fasciitis are more likely explanations for
symptoms in patients referred for tarsal tunnel syndrome. Signs on examination
242 15 Distal and Generalized Sensory Symptoms
include numbness in the sole of the foot and paresthesias reproduced by percussing
the tibial nerve just posterior to the medial malleolus. Be aware that sensation over
the sole is frequently decreased due simply to the thickness of the overlying skin
rather than to any specific neuropathology, so pinprick examination abnormalities
must be interpreted cautiously. Electrodiagnostic studies show slowing of tibial
nerve conduction studies across the ankle, but are frequently challenging and unin-
formative in patients with tarsal tunnel syndrome, particularly in patients with
underlying polyneuropathy. Surgical release of the tibial nerve at the tarsal tunnel is
curative, though seldom performed due to the rarity of the condition.
Morton Neuroma
Cramps
Cramps are characterized by a squeezing pain which most commonly occur in the
calves and feet, and have a tendency to be worse at night. They are discussed in
greater detail in Chap. 14.
Raynaud Phenomenon
Raynaud phenomenon is a very common condition caused by low blood flow to the
tips of the fingers and toes. It is diagnosed when the skin of the tips of the digits
undergoes a biphasic color change (at least two of pallor, erythema, and cyanosis)
(Brennan et al. 1993). Although these color changes are the defining features of
Raynaud phenomenon, patients may be referred for evaluation of neuropathy when
the most prominent complaints are tingling, burning pain, or numbness. The first
step in treating Raynaud phenomenon is to counsel the patient to avoid the cold or
to wear gloves or socks when exposed to cold. Obviously, most patients have tried
these techniques before consulting with a neurologist. The calcium channel block-
ers (e.g., nifedipine 30–180 mg qd) and topical nitroglycerin theoretically produce
vasodilation but unfortunately, these agents provide only marginal symptom relief
and potential side effects likely outweigh the benefits.
Patients with generalized, whole body pain are often referred for evaluation of pos-
sible neuropathy, but rarely have a neurologic disorder. The conditions which result
in generalized body pain are usually psychiatric or rheumatologic in origin.
Psychiatric conditions which may produce whole body pain include depression,
anxiety, malingering, adjustment disorders, malingering, and conversion disorders.
The difficult task in treating these patients is to help them recognize the psychiatric
component of their illness, not feel insulted by the diagnosis, and facilitate contact
with a psychiatrist who will be better-suited to treat their problems.
244 15 Distal and Generalized Sensory Symptoms
Fibromyalgia
Although not a neurologic disease, many patients with fibromyalgia are evaluated
by neurologists. This disease of uncertain etiology affects mostly younger and
middle-aged women and is characterized by aching pain involving the neck, mid-
back, lower back, arms, legs, and chest wall. Patients with fibromyalgia may also
describe burning, tingling, and lancinating pains. Weakness is a subjective com-
plaint, although none is demonstrable on examination. Neurocognitive symptoms
include forgetfulness, impaired concentration, and fatigue. Research diagnostic cri-
teria include the presence of chronic widespread pain and tenderness to palpation of
11 of 18 specific points (Wolfe et al. 1990). By the time a patient with fibromyalgia
is referred to a neurologist, an extensive battery of tests to exclude other diagnoses
is usually available for interpretation. False positive results are common due to the
sheer number of studies ordered. Treatment of fibromyalgia is frequently challeng-
ing. A straightforward discussion of the diagnosis reduces accumulation of disabil-
ity whereas evasiveness and unnecessary, protracted evaluations do more harm than
good (White et al. 2002). Patients with fibromyalgia may show a modest response
to antidepressants. Nonsteroidal anti-inflammatory drugs are usually not effective
and narcotics should be avoided. Encourage patients to pursue alternative therapies,
as conventional medical treatment is frequently disappointing. In some cases, refer-
ral to a rheumatologist (preferably one with an interest in the condition who will not
dismiss the patient) may be helpful.
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