Syringomyelia:

Pain, Sensory Abnormalities, and Neuroimaging

Tea Cohodarevic, Angela Mailis, and Walter Montanera

Abstract: The purpose of this study was to evaluate the characteristics of pain(s) and their
relationship to somatosensory abnormalities, and magnetic resonance imaging (MRI) spinal
cord findings, in 27 patients with long-standing painful syringomyelia. Detailed histories,
pain drawings, and physical examination data were collected. MRIs were reviewed by an
experienced neuroradiologist. Average symptom duration was 14 years. Pain was the sole
symptom at onset in 59% of the patients, cited as the primary cause of disability in 67% of
the subjects. It was reported as spontaneous in 93% of the cases, paroxysmal in 70%, and
touch/pressure-evoked in 22% and rated as moderate in 70% and severe in 11%. Pain and
somatosensory abnormalities primarily involved the upper/lower torso and back, but all
other body areas were affected. Primarily, hypoesthetic sensory abnormalities to touch,
pinprick, and cold were found, only partially matching the areas of pain in most patients.
MRIs revealed large cervicothoracic syrinx in most patients with cord atrophy and arach-
noiditis in several. Blinded infusions of sodium amytal (SA) dramatically modified hypoes-
thesia in 2 patients. The temporary modification of negative sensory abnormalities under
SA suggests that some sensory deficits are dynamically maintained as a result of central
nervous system (CNS) plasticity, superimposed on phenomena arising from structural
neural tissue damage.
Key words: Syringomyelia, pain, sodium amytal.

C
avitation of the of the spinal cord was first
described by Estienne in 1546,1 whereas its
association with Chiari malformation was
noted by Portal in 1804.2 Ollivier dAngers
described cavitation of the spinal cord in conti-
nuity with the fourth ventricle in 18243 and 3
years later he named it syringomyelia. Two major
types of syringomyelia have been described.4 The
communicating type is primarily associated with
Chiari malformations and other abnormalities in
the region of the foramen magnum. In this type,
there is a direct communication of the syrinx with
the ventricular system (at least partially responsi-
ble for maintenance of the syrinx). In the non-
communicating type (including posttraumatic,
idiopathic, and tumor- and arachnoiditis-associ-
ated syrinx), the posterior fossa and foramen
magnum region are normal, and a direct com-
munication of the syrinx with the ventricular sys-
From the Comprehensive Pain Program, Department of Medicine; the
Playfair Neuroscience Unit; and the Department of Radiology, Toronto
Western Hospital and the University of Toronto, Toronto, Ontario,
Canada.
Address reprint requests to Angela Mailis, MD, Msc, FRCPC(PhysMed),
Comprehensive Pain Program, Toronto Western Hospital, 4BFell-174,
399 Bathurst St, Toronto, Ontario M5T 2S8, Canada. E-mail:
angela.mailis@uhn.on.ca
2000 by the American Pain Society
1526-5900/00/0101-0008$5.00/0
tem is not evident. Syringomyelia is a relatively
infrequent disorder, constituting less than 1% of
admissions in neurological clinics.5
The neurological disturbances depend on the
extent and site of cavitation and consequent
gliosis. Signs and symptoms are summarized by
Schliep5 and include primarily sensory distur-
bances, motor disturbances, long tract function
impairment, trophic disorders of skin, neuro-
genic arthropathies, and pain and/or dysesthe-
siae. Pain and/or dysesthesiae are reported as a
very frequent complaint, ranging from 14.5% to
85%.6-12 Dissociated sensory loss (ie, loss of ther-
mal and pain sensation with preservation of
touch) is considered a classic sign. However, it has
been reported variably, ranging from 31% to
100% of the cases.4,5,7-9,11,12 Certain authors have
emphasized that sensory loss is not necessarily
dissociated in syringomyelia.13 Others5 have
reported dissociated sensory loss early in the dis-
ease that progresses later to touch and proprio-
ception impairments as well.
Pain may present as an early symptom of
syringomyelia14 or can be aggravated by sneez-
ing and coughing.15 The pain has been reported
to be most commonly confined to the upper
extremities or thorax, whereas few patients have
pain in the lower extremities.16 Burning, aching,

54 The Journal of Pain, Vol 1, No 1 (Spring), 2000: pp 54-66

ORIGINAL REPORT/Cohodarevic et al
and pressing are the most common descriptors
of the pain.16 More than 1 kind of pain with vari-
able descriptors and intensity may occur.
A survey of the Canadian Syringomyelia
Network (CSN) participants during their 1996
annual meeting17 discovered a 97.5% prevalence
of pain; 47% of the participants were completely
unable to work, with the overwhelming majority
attributing this to intractable pain. Of the partic-
ipants, 90% stated that there was a connection
between stress, activity, and pain. The most com-
mon pain complaints in this survey involved the
head/neck region (90%), shoulders/arms (75%),
and chest/abdomen (50%).
This survey triggered the present descriptive
study in patients with syringomyelia to elucidate:
(A) pain onset and evolution, temporal and spa-
tial characteristics, and severity/interference with
quality of life; (B) the relation of pain and
somatosensory abnormalities; and (C) MRI char-
acteristics of cavitary lesions.
Materials and Methods
Patients with and without pain were invited to
participate in the study through advertisement
by the CSN (a Canadian support group of
patients with syringomyelia). Furthermore,
patients were recruited through the regular
referral route to the Comprehensive Pain
Program of the Toronto Western Hospital from
Neurosurgery and Neurology. Both posttrau-
matic syringomyelia and nontraumatic/nonma-
lignant syringomyelia patients were included. All
patients recruited through the Syringomyelia
Network were offered pain treatment if they so
wished. All patients volunteering for the study
signed an informed consent form approved by
the institutional board, and all aspects of the
study conformed to the ethical guidelines of the
1975 Declaration of Helsinki.
Specific information was collected in all
patients by detailed history and thorough physi-
cal examination by experienced doctors in neu-
ropathic pain conditions (TC, AM), and docu-
mented as follows:
1. Patients were asked to draw their pain(s) on
body maps. The pain drawings done by each
patient were divided (for purposes of later
analysis) into 7 body areas: head and neck,
upper torso/back, lower torso/back, right and
left upper extremity, and right and left lower
extremity.
2. A detailed pain history was obtained, docu-
menting pain descriptors, the relationship of
onset of pain(s) to onset of sensory and/or
motor abnormalities; types of experienced
pain(s), as well as their course and evolution;
qualitative, temporal, and spacial characteris-
55
tics; aggravating and relieving factors;
response to surgery and medications; and
degree of pain interference with lifestyle.
3. Detailed body maps of cutaneous sensation, as
well as all other neurological findings obtained
upon examination, were recorded on standard-
ized forms currently in use by the Compre-
hensive Pain Program of the Toronto Western
Hospital. Gross sensory abnormalities to light
touch, pinprick, and cold were documented on
forms depicting body maps (anteriorly and pos-
teriorly) with the use of a soft paint brush, a
pinwheel (sterilized after each use), and a metal
roller, respectively. These body maps were iden-
tical to the ones used by the patients to draw
their subjective pain areas.
4. Sources of coexistent nociceptive pain (ie,
degenerative joint disease, myofascial pains,
etc.) were recognized and documented.
5. An experienced neuroradiologist (WM) read
the MRI films and documented in detail the
largest anteroposterior and transverse diame-
ter of the syrinx, the corresponding vertebral
levels, the presence of arachnoiditis, cord
atrophy or cord tethering, and the presence of
shunts or other devices. The neuroradiologist
was blinded to the patients symptoms, prior
surgical interventions, and MRI interpreta-
tions by other consultants.
6. Patients with significant pain were admitted
to the inpatient unit and subjected to a thor-
ough multidisciplinary evaluation that
included multiple placebo-controlled diagnos-
tic infusions with the patients signed consent,
a full psychiatric and psychological assess-
ment, and electrophysiological tests as
needed. All amytal infusions were videotaped
with the patients signed consent.
Results
Patient Demographics/Clinical Symptoms
and Signs
Twenty-seven patients with pain and
syringomyelia arising from a variety of causes
were involved in this study. Two patients with-
out pain filled out the questionnaires, but
declined to submit themselves to a physical
examination (because of distance). Therefore,
they were excluded, and the study includes only
subjects with pain. Of the subjects, 18 were
members of the CSN (but some of them had
been referred to us by their treating surgeons as
well). The demographic and other characteris-
tics of the patients are detailed in Table 1. In the
group of 7 with arachnoiditis, CNS neurosyphilis
in one patient and familial arachnoiditis in
another preceded the development of a syrinx,
56 Syringomyelia and Pain

Table 1. Patient Characteristics Table 2. Pain Characteristics

NO. OF PATIENTS % NO. OF PATIENTS %

Sex Onset
Male 10 Sudden 10 37
Female 17 Gradual 17 63
Age (yr) Mean = 40 (16-65) Types
Cause Spontaneous/ongoing 25 93
Chiari Type I 12 45 Spontaneous/paroxysmal 19 70
Arachnoiditis 7 26 Evoked (touch or pressure) 6 22
Idiopathic 6 22 Severity Mild Moderate Severe
Spinal cord injury 2 7 5 (19%) 19 (70%) 3 (11%)
Duration of symptoms (yr) Mean = 14 (1-32) Aggravators
Initial symptom Prolonged activity 13 48
Pain 16 59 Stress 8 30
Motor and/or sensory 8 30 Position/posturing 8 30
Pain and motor/sensory 3 11 Barometric changes 7 26
Current symptoms/signs Touch/pressure 6 22
Pain 27 100 Movement 6 22
Weakness (at least 1 limb) 22 82 Coughing/sneezing 4 15
Upper extremities 12 45 Relievers
Lower extremities 10 37 Physical modalities 11 41
Bilateral 17 63 Rest/relaxation 10 37
Paresthesiae 20 74 Distraction 6 22
Reflex abnormalities 21 78 Medications 6 22
Reduced/absent 8 30 Lying supine 4 15
Increased 6 22 Activity/exercises 4 15
Combinations 7 26 Pain areas
Babinski 2 7 Numer Mean = 4 range = 1-7
Bowel/bladder dysfunction 10 37 Upper torso/back 23 85
Balance/gait problems 5 19 Lower torso/back 17 63
Spacticity 5 19 Head/neck 16 59
Other* 6 22 Left arm/hand 15 56
Left leg/foot 14 52
*Charcot joints, kyphoscoliosis, muscular atrophy.
Right arm/hand 13 48
Right leg/foot 13 48

whereas the other 5 patients developed arach-

noiditis followed by syringomyelia after surgery
(with or without the use of pantopaque myelo-
gram dye).
The patients had symptoms or knew of the
diagnosis for an average of 14 years (range 1 to
32 years). Strikingly, the initial symptom (per the
patients recollection) was pain in 59% of the
patients, motor and/or sensory abnormalities in
30%, and a combination of pain and sensory/
motor symptoms in 11%.
Pain was present at variable intensity in all
patients. Weakness on examination (of variable
degree) in at least 1 limb was present in 82%; 2
patients were weak in both upper and lower
extremities. Other symptoms/signs detected
related to paresthesiae, reflex abnormalities,
bowel/bladder dysfunction, gait/balance distur-
bance, spasticity, and so on. These findings are
presented in detail in Table 1.
Characteristics of Pain
Particulars of pain are summarized in Table 2.
The onset of pain was abrupt in 37% of the
patients and gradual in 63%. Remarkably, one
third of the patients with acute pain onset experi-
enced the pain after spells of coughing or sneez-
ing, and another 2 patients after acute onset of
soft tissue injury (whiplash in a car accident and a
fall from a horse). In the overwhelming majority,
spontaneous ongoing, paroxysmal, and stimulus-
evoked pains were present alone or in variable
combinations. The most common descriptors for
spontaneous ongoing pain were burning, dull,
tight, throbbing, stinging, and cramping, and for
spontaneous paroxysmal pain sharp, shooting,
pinching, tingling, burning, and dull.
Although the majority of patients had very large
pain fluctuations throughout the day, they were
asked to indicate their average subjective pain rat-
ings, on a numerical scale from 0 to 10 (where 0 =
no pain and 10 = the worst pain). Pain rated as 1
to 4 was considered mild, pain rated 4 to 7 was
considered moderate, and pain rated as greater
than 7 was considered severe. Nineteen percent of
the patients were classified as having mild pain,
70% as having moderate pain, and 11% as having
ORIGINAL REPORT/Cohodarevic et al
Figure 1. Body maps depicting cumulative data expressed as percentage of patients who presented with pain complaints, and
abnormalities of cutaneous sensation. For purposes of classification, the body has been divided in 7 areas, as shown.
severe pain. Multiple factors (Table 2) were
reported to aggravate or ameliorate the pain.
Each patient had an average of 4 pain areas
(range 1 to 7), determined as described in
Methods. The most frequent area of pain was
the upper torso/back area, followed by the lower
torso/back and the head/neck area. At least 1
extremity was involved in 89% of the patients.
These findings are shown in Table 2 and Fig 1.
Cutaneous Sensory Abnormalities
Sensory examination (albeit not quantitative)
revealed the following gross areas of cutaneous
sensory abnormalities in rank order: upper
torso/back (85%), lower torso/back (67%),
head/neck area (56%), and variable involvement
of the extremities. At least 1 extremity had
abnormal cutaneous sensation in all patients
(100%). The average number of areas with
abnormal cutaneous sensation was 4.3 (range, 1
to 7) (ie, no patient had completely normal cuta-
neous sensation). Fig 1 shows cumulative data of
(1) body pain areas and (2) areas of abnormal
cutaneous sensation in the 27 patients.
The cutaneous sensory abnormalites were clas-
sified (1) based on the stimulus used
(touch/brush, pinprick, and cold) and (2) as posi-
tive (hyperesthesia) or negative (hypoesthesia)
(ie, the sensation was perceived as stronger or
weaker than that of a control [normal] area,
respectively). The overwhelming majority of
57
patients had pure negative sensory phenomena
(hypoesthesia) to 1 or more cutaneous testing
modalities in 1 or more body regions. Partic-
ularly, light touch was consistently impaired in
about 56% of the patients, pin prick in 67%, and
cold in 78%. Allodynia as the sole tactile abnor-
mality was found in only 2 patients. Some
patients presented with hypoesthesia and hyper-
esthesia to the same sensory modality, but in dif-
ferent parts of their bodies. The findings are
detailed in Fig 2. Overall, there was no patient
without sensory abnormality to 1, and most
often 2 or 3 cutaneous sensory modalities in (at
least) 1 body region.
The congruence, or match, of pain areas
(drawn by the subjects) and sensory abnormality
areas (documented by the physicians) in identical
body maps (as described previously in Materials
and Methods) were compared. Only 26% of the
patients had sensory abnormalities confined
exactly to the areas of pain. The majority (67%)
had partial match (ie, some sensory abnormali-
ties in nonpainful body regions and vice versa).
Two patients had a complete mismatch, as all
their sensory abnormalities were confined to
nonpainful body regions, whereas all the areas
of subjective pain had normal cutaneous sensa-
tions. Furthermore, in several subjects the area
of perceived pain was much smaller than the
area of sensory abnormalities. The findings are
detailed in Fig 3, and illustrative patient exam-
ples are given in Fig 4.
58
Figure 2. Cumulative data showing the percentage of
patients with gross cutaneous sensory abnormalities to touch,
pinprick, and cold. The majority of patients had hypoesthesia
to all sensory modalities. However, patients classified as
mixed had areas of hypoesthesia and hyperesthesia to the
same testing modalities in different parts of their bodies.
Treatment Responses
Overall, 21 patients (78%) had a total of 28 sur-
gical treatments as follows: decompression (n =
8), shunting (n = 14), shunt revisions (n = 3), and
syrinx drainage (n = 3). According to the patients
subjective reports and recollection at the time of
the interviews, pain improved in 33%, and (when
present) weakness improved in 25% and blad-
der/bowel problems in 80%. Six patients (22%)
had no surgical interventions. The reasons for
nonsurgical treatment were as follows: (1)
Extensive arachnoiditis and adhesions, (2) the
patient refused treatment, and (3) attempted
shunting but surgery failed (1 patient).
In regard to nonsurgical treatments, at the
time of referral, 20 patients (74%) were on com-
binations of different medications as follows:
ten patients (37%) on tricyclics, 7 (26%) on ben-
zodiazepines, 4 (15%) on antiepileptics, 2 (7%)
on baclofen, and 14 (52%) on combination anal-
gesics or narcotic analgesics. In regard to anal-
gesic use in particular, 11 patients were on
acetaminophen and/or aspirin combined with
codeine or oxycodone (up to 10 tablets per day),
and 3 patients were on short- or long-acting
morphine or equivalent opioids (up to 200 mg
of morphine daily). Seven patients (26%) were
on no medications except the odd over-the-
counter analgesic. In 11 patients (41%) we
Syringomyelia and Pain
Figure 3. Match between pain areas and areas of abnormal
gross cutaneous sensation.
either initiated or modified treatment, using a
combination of neuropathic medications (tri-
cyclics or antiepileptics), as well as narcotic anal-
gesics. Overall, the effect of pharmacological
manipulations (in patients with stable pharma-
cological regimes and those for whom we initi-
ated or modified treatment) was modest
(reporting 20% to 30% pain reduction com-
pared with pretreatment levels, without partic-
ular reference to the types of pain responsive to
different medications). Although the effect of
narcotics alone was rated as modest, all patients
on these drugs considered them to be a neces-
sary adjunct. The remaining patients (n = 16 or
59% of the study subjects), who were almost all
volunteers, refused modification or initiation of
pharmacological interventions. The reasons
cited for refusal were as follows: (1) pain was
tolerable (n = 7/16, 44%); (2) other means of
pain control were effectively used (marijuana in
1 patient, alcohol consumption in a second
patient, and naturopathic remedies in the third
patient); and (3) 6 patients (38%) did not live in
Ontario or preferred to be treated by their local
physicians.
Disability Levels
Disability (at work and during homemaking
and leisure activities) in general, was rated by
the study subjects based on a simple system. The
disability level was considered mild if the subject
was unable to perform a few insubstantial tasks;
moderate if he/she was unable to perform sev-
eral tasks; and severe when most tasks (com-
pared with the premorbid performance level)
ORIGINAL REPORT/Cohodarevic et al 59

Figure 4. Examples of paired pain and sensory abnormalities maps in 6 cases. Pain is shown on the left and sensory abnormalities
on the right. In the sensory diagrams, the dotted areas represent negative sensory abnormalities to all 3 test modalities, whereas
the slashed areas show mixed sensory abnormalities (hypoesthesia and hyperesthesia) to 1 or more testing modalities. Underlying
diagnosis is stated at the top of each case. C, Chiari malformation.

could not be performed. Whereas 96% of the Responses to Intravenous SA

patients were employed in different occupa-
tions at the onset of syringomyelia-related
symptoms, including 1 who was a full-time stu-
dent, at the time of this study 70% of the sub-
jects were on long-term disability. Similarly, 74%
of the subjects stated that they were moderately
or severely disabled during their homemaking
or leisure activities. These data are detailed in
Table 3. Pain was cited by 67% of the subjects as
the exclusive or most important reason for dis-
ability.
Four patients with moderate or severe pain
were investigated as inpatients with several infu-
sions and submitted to multidisciplinary team
assessment. They all failed to respond to placebo-
controlled IV lidocaine infusion (at 3 to 5 mg/kg
of body weight over 30 minutes), in relation to
subjective pain and sensory abnormalities. Two of
them also failed to respond to placebo-controlled
SA infusions at 4 to 7 mg/kg body weight (rate 50
mg/min). However, in 1 patient with a C2-T1
syrinx and previous surgical intervention, all (neg-
60 Syringomyelia and Pain

Table 3. Disability Status (n = 27) 1. In this group of patients, no comments were

NO. OF PATIENTS %
Work status at onset
Professional FT 5 19
White collar FT 14 52
White collar PT 2 7
Blue collar FT 3 11
Student 1 4
On disability pension 1 4
Work status at present
Same as at onset 6 22
Long-term disability 19 70
Old age pension 2 7
Disability related to homemaking and leisure
None 2 7
Mild 5 19
Moderate 15 56
Severe 5 19
Note. Professional denotes a university degree, white collar denotes
an office worker, and blue collar denotes skilled labor.
Abbreviations: FT, full time; PT, part time.
ative) cutaneous abnormalities normalized under
amytal but not under normal saline. The fourth
patient had profound sensory deficits to all cuta-
neous modalities from the neck and below, asso-
ciated with a C2-conus syrinx and severe cord
atrophy. This patient failed to respond to normal
saline, but under SA infusion she converted tem-
porarily the areas of hypoesthesia in both upper
and lower distal extremities and part of the
abdomen to areas of significant hypersensitivity
to touch, prick, and cold. This illustrative case is
presented later in this article.
MRI Findings
Information about the size/extent of syrinx was
extracted through (1) MRI films in 11 patients
and (2) review of available paper records in
another 5. The actual review of these 11 MRIs
revealed that 8 patients had extensive cervi-
cothoracic syrinx. In 1 patient only the shunt was
seen at the C4-6 level while the original syrinx
had collapsed (its original size unknown). The
remaining 2 patients had a small thoracic syrinx
(2 and 5 levels, respectively) associated with
severe arachnoiditis. Overall, in this group of 11
patients, arachnoiditis was present in 4, moder-
ate or severe cord atrophy was present in 9, and
cord tethering was present in 3 patients.
Detailed data of reviewed MRI films are listed in
Table 4. MRI paper records also indicated that
extensive syringomyelia was present in the other
5 patients. In this group, the smaller syrinx was
involving 8 thoracic levels with coexistent arach-
noiditis and the largest extended from the brain-
stem to the L1 level. Arachnoiditis was present in
made in the available paper records about
details such as cord tethering and atrophy. The
MRI abnormalities extracted from paper records
are listed in Table 5.
Illustrative Case Report
The patient is a 38-year-old woman who devel-
oped clumsiness of the left hand at age 16 and
gait difficulties at the age of 20. After investiga-
tions, the diagnosis of extensive idiopathic
syringomyelia was made, and in 1980 she was
shunted, which stopped the progression of
motor and bowel/bladder symptoms. She was
always aware of lack of sensation and, as an
infant, she was chewing constantly her finger-
nails and picking on her toenails, sustaining
painless bleeding. On one occasion, as an adult,
she sustained painless third-degree burns on her
shoulder while using a curling iron. In July 1984,
she started experiencing episodic left-sided
chest wall/back pain, occurring once or twice a
year and lasting 1 to 3 weeks. She was sedated
heavily through these episodes, so she could
sleep through her pain. In December 1994, she
was involved in a rear-end collision and sus-
tained a mild whiplash injury. Since then, she
has complained of almost daily headaches, back
pain, and very frequent episodes of incapacitat-
ing torso pain (at least once per month), that
could not be explained on the basis of ongoing
tissue injury as a result of her accident. During
the 4 days of admission, the patient was com-
plaining of back pain and headaches only; she
was free of episodic chest pain. On physical
examination, she proved to be an obese woman
with unstable gait and strongly positive
Rhomberg sign (she felt she was walking on
feathers). She had almost no nailbeds and all
her terminal finger phalanges were distorted
and deformed because of her long-standing
habit of finger-chewing. Profound loss of sen-
sation to all cutaneous modalities was shown
from the neck and below, associated with severe
impairment of position sense and vibration
sense. Spinal MRI revealed a C2-conus collapsed
syrinx (the size of the original one was
unknown) and severe extensive cord atrophy.
Videotaped single-blinded normal saline infu-
sion failed to alter back pain and headache rat-
ings or sensory abnormalities. Subsequently,
blinded infusion of 250 mg of SA produced, to
our surprise (as well as the patients), reversal of
the profound hypoalgesia of all distal extremi-
ties and a part of the abdomen, and replace-
ment by significant hyperalgesia to all sensory
testing modalities, lasting about 25 to 30 min-
utes. The hands of this patient are shown in
ORIGINAL REPORT/Cohodarevic et al 61

Table 4. Magnetic Resonance Imaging Finding (Most Recent Films Reviewed)

MAXIMAL CORD ARACHNOIDITIS/
PATIENT LEVEL DIAMETER ATROPHY TETHERING SURGERY ADHESIONS COMMENTS

1. C2-T11 AP = 5 mm Severe At T3 Yes Yes Cord displaced (T8-11)

T = 10 mm and clumping cauda
at T4 disc level equina
2. C1-T8/9 AP/T = 3 mm at Moderate No Yes No Congenital fusion at C5-6
C6-7 disc level
3. 1. C1 AP = 2 mm Moderate Yes (shunt) Yes Original syrinx C1 T11.
2. T8-11 T = 5 mm at Y7 to severe Now, syrinx collapsed
between C1 and T8
4. C2-T7 AP/T = 0.9 mm Moderate No No No Chiari Type I
at C2 to severe
5. C4-T6 AP = 0.7 mm Moderate No Yes No Chiari Type I
T = 0.9 mm to severe
at T2-3
6. C1-T6 AP/T = 6 mm Moderate No No No Chiari Type I
at C6-7
7. T3-8 AP = 1.1 mm Mild Yes Yes Yes Macrocystic cord
T = 1.3 mm degeneration
at T4
8. C1-T7/8 AP = 3 mm Severe No Yes No Collapsed syrinx,
T = 4 mm but metal artifact
at C7 obliterates lower end
9. T11-12 AP = 8 mm Moderate Yes Yes Yes Arachnoiditis, most
T = 9 mm pronounced thoracic
at T12 level
10. ? No No No No Only shunt seen at C4-6,
origin size/extent of
syrinx unknown
11. C2-conus AP/T = 1 mm Severe Yes, at site Yes No Cavity at T11-12
at T5 of shunt
T5 small residual cavity
Syrinx collapsed,
shunt at C6
Abbreviations: AP, anteroposterior diameter; T, transverse diameter.

Figure 5, and her self-recorded pain drawings as
well as the sensory abnormalities to pinprick
before and after SA infusion in Figure 6.
Discussion
This study provides significant and novel infor-
mation about pain and somatosensory deficits in
syringomyelia. The patients in this study repre-
sent a selected sample (because they all had
pain). Etiologically and pathophysiologically,
they constituted a mixed group (communicating
and noncommunicating syringomyelia).
Clinical Findings
Our study patients suffered from painful
syringomyelia of very long duration (average of
14 years) and mixed etiology. Remarkably, pain
was either the first symptom signaling the onset
of the disease (59%) or occurred early on, in
combination with sensory/motor deficits (30%).
It also constituted the most important cause of
long-term disability according to the patients
self-reports (67%), despite the fact that two
thirds of the patients rated the pain as moder-
ate. Both spontaneous ongoing, paroxysmal, and
stimulus-evoked pains were present. We assume
that particularly those pains characterized as
burning have been classified by other authors
as dysesthetic.10 Although the study included
no patients without pain (for reasons cited
above), the prevalence of pain in 97.5% of the
participants in the 1996 Syringomyelia Network
meeting17 (who may indeed constitute a repre-
sentative sample of the population with the dis-
order), suggests that pain is an extremely fre-
quent symptom with variable severity.
Surgery in the study group had long-term ame-
liorating effects on pain only in one third of the
patients, whereas it seemed to have a much bet-
ter effect on bowel and bladder problems. Our
data relating to surgical outcome may be of lim-
ited value, because they are self-reported and
62
Table 5. Magnetic Resonance Imaging Findings (Paper Records)
MAXIMAL CORD
PATIENT LEVEL DIAMETER ATROPHY
12. HH T3-11
13. GB C2-T1
14. SA C4-T10
15. TJ C2-T1
16. RK Brainstem-L1
Abbreviations: AP, anteroposterior diameter; T, transverse diameter.
based on recollection only and without objective
measures. However, they are in accordance with
the variable response to surgery recorded in the
literature. Surgical interventions (decompression
and shunting of different types) have been
reported to produce improvement of variable
degree. The improvement expressed as amelio-
ration of the neurological deficit in most stud-
ies relates to motor deficit alone, and ranges
from 0% to 94% as summarized by Levy et al9 in
Chiari malformation. The results are better in the
idiopathic group without tonsillar ectopia and
the posttraumatic group, as well as when the
preoperative symptoms are of shorter dura-
tion.11,12,18 Patients requiring multiple surgeries
do poorly with progression of the neurological
deficit.12 Milhorat et al10 reported particularly
poor response of dysesthetic pains to surgical
interventions, whereas other types of pain may
respond favorably to shunting with or without
decompression.15 In addition, the modest effect
of combination pharmacological manipulations
in our study group is in accordance with the
rather poor response to medical treatments of
persistent (particularly dysesthetic) pain.10 It is
worth pointing out that combination analgesics
or opioids were considered a necessary adjunct
for pain relief by our patients, despite the less
than optimal response of pain to these medica-
tions. Unfortunately, based on the current study,
we are not able to ascertain the response of dif-
ferent types of experienced pains to therapy.
The acute onset of pain in several study sub-
jects, particularly after abrupt increases of
intrathoracic and intra-abdominal pressure,
deserves separate mention. Possibly acute hydro-
dynamic abnormalities within an established
syrinx may be responsible for further acute and
rather irreversible cord damage.
In the study group, arachnoiditis by itself
seemed to be quite prevalent. However, it is not
possible to ascertain if arachnoiditis contributes
to pain, given the fact that no patients without
pain were included. In some patients, extensive
Syringomyelia and Pain
ARACHNOIDITIS/
TETHERING SURGERY ADHESIONS COMMENTS
Yes Yes Shunt attempted failed/
Extensive arachnoiditis
Yes
No
Yes
Yes Syringomyelia 10 y after
spinal cord injury/with
paraplegia
multilevel cord atrophy was present as well.
Given the extent and degree of MRI abnormali-
ties discovered in this group, one cannot stop
marveling about the resilience of the spinal cord
to injury, considering that only 3 patients were
wheelchair-bound.
The extent of mismatch or incongruence
between painful and sensory areas in terms of
region or size (best illustrated in Fig 4) is quite
striking. It should be noted, however, that
because the study was not quantitative, subtle
thermal or tactile deficits in our study could have
been undetected. Bouhassira et al19 reported
that intensity of spontaneous pain in syringo-
myelia was not correlated with the area of max-
imal thermal deficit in quantitative sensory test-
ing. Patients with painless syringomyelia showed
the same magnitude of mechanical and thermal
deficits. The authors concluded that spinothala-
mic impairment is a necessary, but not sufficient,
condition for the development of pain in
syringomyelia and that evoked pains are sus-
tained by specific pathophysiological mecha-
nisms less dependent on spinothalamic deficits.
It should be stressed that, because all patients
in this study had extensive syringomyelia, our
findings and conclusions may not be extrapo-
lated to patients with very small syrinx that occa-
sionally constitutes an incidental finding. If these
patients present with pain, we feel that thor-
ough evaluation is mandated before the pain is
automatically attributed to this very small syrinx.
Response to SA
One of the most striking observations in the
current study is the modification of sensory cuta-
neous abnormalities under SA infusion. This
medium-action barbiturate has been widely used
in our pain unit as a valid diagnostic tool for
many years in bolus infusions of 50 mg/min, 4 to
7 mg/kg of body weight.20-23 The effect of barbi-
turates, in general, has been extensively
reviewed elsewhere.20 In summary, barbiturates
ORIGINAL REPORT/Cohodarevic et al 63

Figure 5. Hands of the patient described in the case report

(C2-conus syrinx with severe diffuse cord atrophy). Notice the
dystrophic nailbeds and deformed terminal phalanges sec-
ondary to intense chronic painless finger-chewing despite
profound hypoesthesia to all cutaneous modalities below the
neck.

produce reversible depression of the CNS, rang-

ing from mild sedation and sleep to general anes-
thesia. Some possess anticonvulsant properties
and may exert euphoric effects. Nonanesthetic
doses preferentially suppress polysynaptic
responses (enhanced inhibition and/or dimin-
ished facilitation). Inhibition is both presynpatic
(spinal cord) and postsynaptic (cortical and sub-
cortical structures). In the peripheral nervous sys-
tem, barbiturates selectively depress autonomic
ganglia transmission and reduce choline esters
nicotinic excitation (partially accounting for the
drop in blood pressure). Inhibition is reported to
occur primarily at gamma-aminobutyric acid
(GABA) sites. In summary, barbiturates exert (1)
GABA-mimetic effects and (2) ionotropic AMPA,
kainate, and NMDA receptor noncompetitive
antagonistic effects.
In regard to patients with neuropathic pain
and hyperesthesia to different cutaneous modal-
ities, we have shown that SA exerts a dramatic
modulatory and selective influence on centrally
mediated allodynia, with much less effect on pin
prick and cold hyperalgesia and no effect on
deep pain as measured by algometry.20,21 In our
studies on neuropathic pain patients20,21 and sev-
eral other ongoing (unpublished) studies, we
observed also that structural (anatomic) sensory
hypoesthesias (after documented spinal cord
lesions, and severe nerve injury or neurectomy)
retain their borders under SA infusion. In con-
trast, we have reported in the past24 that wide-
spread nondermatomal sensory deficits to multi-
ple cutaneous testing modalities in patients with
little or no detectable peripheral or central
pathology tend to almost normalize or disappear
under SA infusion. Therefore, the temporary
modification of negative sensory abnormalities
during SA infusion in the 2 reported cases (with
Figure 6. (Top) Pain map as drawn by patient. (Middle)
Pinprick hypoalgesia (1 sensory abnormality was selected for
this figure to avoid confusion) illustrated by dotted areas.
Dense deficit is shown by more intense dotting. (Bottom)
Reversal of pinprick hypoalgesia (dotted areas) to hyperalge-
sia (slashed areas) in the abdomen and distal extremities dur-
ing infusion of sodium amytal.
extensive and unquestionable structural deficits)
was startling and unexpected.
The nature of nondermatomal negative sen-
sory deficits in humans is largely unknown.
Moriwaki et al25 reported, in patients with neu-
ropathic pain, progressive shrinking of rather
localized hypoesthesia surrounding an area of
allodynia, in parallel with obtained pain relief by
variable therapeutic interventions. The phenom-
ena were attributed to centrally mediated fac-
tors. We first reported the responses to normal
saline and SA infusions in 11 patients with wide-
spread (quadratomal or hemisensory) cutaneous
deficits unexplainable by detectable nociceptive
or neuropathic pathology and postulated a cen-
trally mediated psychobiological substrate.24
Subsequently, 2 other reports appeared in the lit-
erature relating to widespread deficits in
64
patients with complex regional pain syndromes,
also attributable to central factors.26,27
It is well established in experimental animals
that many spinal neurons can be tonically inhib-
ited by activity in descending pathways. This
descending inhibition is assumed to be part of an
intrinsic system able to suppress pain.28-37
Schaible et al38 studied modification of tonic
descending inhibition (TDI) in anesthetized cats
during acute inflammation of the knee joint
before and after reversible cold block to the
lower thoracic spine. TDI in the ipsilateral and
sometimes contralateral leg neurons was
observed, and progressively increased during the
development of inflammation. They postulated
that this increase in TDI could be due to several
mechanisms: (1) Ascending activity from the
inflamed joints could enhance the effectiveness
of the descending pathways by exciting
inhibitory networks in supraspinal regions rele-
vant to the generation of TDI (this hypothesis is
consistent with the proposal that the nervous
system develops central inhibition directed
towards its excitatory drives39-43). (2) Enhanced
excitability of spinal neurons during inflamma-
tion could also facilitate the synaptic effects of
inhibitory impulses, thus making these neurons
more susceptible to all their inputs, afferent as
well as descending. They concluded that the
increased effectiveness of TDI counteracts the
enhanced excitability of spinal neurons. Whereas
this study and others have shown alterations of
TDI during acute inflammation, additional mech-
anisms could be involved in chronic inflamma-
tory conditions (ie, marked heterotopic
inhibitory influences44 and local peptide changes
secondary to altered neuronal expression).
Therefore, local and other changes also could
modify segmental inhibitory mechanisms.
CNS-generated positive sensory phenomena
have been shown in animals, even beyond the
distribution of a peripheral nerve.45 In humans,
centrally mediated A low-thresholdmechano-
receptor allodynia spreading beyond the region
of injury or the territory of a nerve, is considered
the product of central sensitization.20,21,46 These
studies and others have shown that the CNS is
capable of generating positive sensory phenom-
ena. Therefore, it is only logical to assume that it
is equally capable of generating negative sensory
phenomena. Whereas inhibition has been shown
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