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Syringomyelia as a cause of limb hypertrophy

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Jaishree Shantilal Mehta at Gujarat Vidyapith - Ahmedabad
Jaishree Shantilal Mehta
S Khanna
S Khanna
S Khanna
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A case of limb hypertrophy secondary to syringomyelia is presented.
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Syringomyelia as a Cause of Limb
Hypertrophy
J. Mehta, S. Khanna
Summary
A case of limb hypertrophy secondary to
syringomyelia is presented.
Key words: Syringomyelia, Limb hypertrophy.
Introduction
Syringomyelia is classically associated with
segmental weakness and wasting (generally involving
the hand and arm) with loss of tendon reflexes and
segmental dissociated anesthesia. We are presenting a
case of syringomyelia with limb hypertrophy, instead
of atrophy.
Case Report
A 54 year old, right handed male was seen in the
neurology department with the complaints of
decreased sensations on the left half of the face, arm
and chest for the last 36 years. He had gradually
progressive history of objects falling from left hand
without knowledge and proximal as well as distal
weakness of left upper limb. He had insidious onset
unsteadiness of gait of 7 years duration. There was no
history of cranial nerve involvement or sensory
symptoms in the lower limb. There was no history of
spinal injury or meningitis. General examination
revealed a short statured patient with thoracic
kyphoscoliosis and convexity to right. There was a
localized painless swelling in the region of left arm
suggestive of rupture of tendon of biceps brachii. The
left elbow showed a painless swelling with crepitus
and restriction of movements i.e. Charcot’s joints.
There were scars and burns on the fingers.
Neurological examination showed normal higher
mental functions, left Horner’s syndrome, decreased
sensation on left half of face, loss of taste sensation on
left half of tongue, left 9th and 10th cranial nerve
palsy. Left sternocleidomastoid and trapezius were
weak. The tongue showed wasting, fasciculations on
left half and deviation to left on protrusion. Motor
system examination revealed hypertrophy of left hand
and forearm (Fig. 1). There was difference of 3 cm in
the forearm girth. Fasciculatory tremors were seen on
the left hand fingers. There was hypotonia of left
upper limb and normal tone in other extremities.
Power was grade 3 to 4 in left upper limb, 4 to 4+ in
left lower limb, 4+ in right lower limb and 5 in right
93Neurology India, 50, March 2002
SHORT REPORTS
Fig. 1 :T2WI MRI (sagittal) showing anterior displacement of
the upper cervical spine with cord impingement.
Fig. 1 : Showing hypertrophy of left hand and forearm.
Fig. 2 : Showing syrinx extending from cervico-medullary
junction to T6 and ACM grade 1.
94 Neurology India, 50, March 2002
SHORT REPORTS
upper limb. Deep tendon reflexes revealed absent left
biceps, triceps and supinator reflexes. The plantars
were extensor bilaterally. Sensory examination
revealed loss of hot cold sensations and pain on left
arm and left half of chest. Position sense was lost in
left upper limb. MRI of the brain and spine (T1, T2,
Proton PD sequences) revealed grade 1 Arnold Chiari
malformation with long syrinx extending from
cervico-medullary junction to T6 (Fig. 2). X-ray of the
hand did not reveal any bony hypertrophy. A syringo-
subarachnoid shunt was done. There was no fresh
deterioration in neurological status, after the surgery.
Discussion
The association of hypertrophic changes in bones,
muscles, other tissues especially upper limb has been
recognized in syringomyelia.
1,2
Kazumasa et al
2
described limb hypertrophy in 11.5% of cases in their
series. In our experience of 25 cases, this is the first
case. Different mechanisms have been postulated for
neurogenic hypertrophy in syringomyelia. These
include stimulation of sympathetic nervous system
3
causing defective circulation and edema. Delaporte et
al
4
postulated that muscle hypertrophy occurring in
syringomyelia is secondary to neural abnormalities.
These result in release of molecules which accelerate
proliferation and protein synthesis rates in muscle
cells involved in the development of hypertrophy.
In this case, it seems that combination of factors i.e.
stimulation of pre-ganglionic sympathetic neurons in
the intermediolateral columns, as well as anterior horn
cells stimulation by the syrinx cavity, may have been
responsible.
References
1. Schliep G : Syringomyelia and syringobulbia. In : Handbook
of clinical neurology. Vinken PJ, Bruyn GW (eds.)
Amsterdam: North-Holland, 1978; 255-327.
2. Kazumasa S, Yoshiko O, Ichiro Y et al : Syringomyelia as a
cause of body hypertrophy.
The Lancet.
1996; 347 : 1593-
1595.
3. Osswald W, Guimaraes S : Adrenergic mechanisms in blood
vessels: morphological and pharmacological aspects.
Rev
Physiol Biochem Pharmacol
1983; 96 : 53-122.
4. Delaporte C, Defer G, Diaz C et al : Increased growth of
myoblasts from hypertrophic muscles in syringomyelia.
J Neurol Sci
1991; 105 : 183-91.
Correspondence to : Dr. J. Mehta, Department of Neurology,
Indira Gandhi Medical College, Shimla-1, H.P., India.
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