Syringomyelia

Examination
Proceed as per normal for the upper limbs
Once dx is made, request
o Examine the neck
Scars of previous Sx
Scoliosis
o The cranial nerves
Horners syndrome
Ataxia and nystagmus
Bulbar palsy (syringobulbia)
Loss of temperature and pain sensation from the outer part of the face
progressing towards the center
o The lower limbs
Spastic paraparesis
Presentation
Sir, this patient has got syringomyelia as evidenced by
LMN pattern of weakness of both ULs
o Wasting and weakness of the small muscles of the hands and forearms
o Reduced tone and reflexes
There is dissociated sensory loss with
o Loss of sensation to pinprick in the ULs and upper chest
o With intact sensation to vibration and proprioception
I also noticed presence of
o Scars and old burn marks on his fingers
o But I did not detect any Charcots joints of the ULs
o La main succulente ugly, cold, puffy, cyanosed hands with stumpy fingers
and podgy soft palms
Examination of the face
o There was no evidence of bulbar palsy
Palatal movements were normal, and CN XI and XII were intact
o There was also no Horners syndrome
o No ataxia or nystagmus
o However there is loss of sensation to pinprick of the face in an onion skin
pattern
Examination of the neck
o No surgical scars noted
o No kyphoscoliosis
Examination of the lower limbs
o Spastic paraparesis

In summary, this patient has syringomyelia with presence of wasting of the upper
limbs, dissociated sensory loss and spastic paraparesis of the lower limbs. This has
resulted with complications of repeated trauma of his hands.

Questions
What is syringomyelia?
Cavity formation with presence of a large fluid filled cavity in the grey matter of the
cervical spinal cord which is in communication with the central canal and contains
CSF.
Triad of LMN weakness of the ULs, dissociated sensory loss in the ULs and UMN
weakness in the LLs
How do patients present?
Rare disorder, 4th to 5th decades, male=females
Painless trauma or burns in the upper limbs, poorly localised pain in the ULs
What is the pathophysiology?
At the level of the syrinx
o LMN anterior horn cell affected
o Dissociated sensory loss affects the decussating fibres of the spinothalamic
tract
Below the level of the syrinx
o Affecting the pyramidal corticospinal tract with spastic paraparesis of the LLs
and preservation of sphincters
Extension into the upper cervical cord and medulla
o Horners syndrome
o Bulbar palsy (CN IX-XII)
o Ataxia and nystagmus ( affects the medial longitudinal bundle if lesion from
C5 upwards)
o Onion skin pattern loss of pain in the face (spinal nucleus of V CN which
extends from the pons to the upper cervical cord)
What are the differential diagnoses for dissociated sensory loss?
Anterior spinal artery occlusion (affects the spinothalamic tract)
DM neuropathy, leprosy, hereditary amyloidotic polyneuropathy
What are your differential diagnoses for syringomyelia?
Craniovertebral anomalies
Spinal cord injuries
Intramedullary tumours of the spinal cord
Arachnoiditis around the foramen magnum obstructing CSF flow
Hematomyelia

What are the associated abnormalities?

Arnold-Chiari malformation
Bony defects around the foramen magnum
Hydrocephalus
Spina bifida
Spinal cord tumours
How would you Ix?
MRI scan of the spinal cord
How would you manage?
Drainage of the syrinx to the subarachnoid space
Syringoperitoneal drainage
In AC malformation, cervical laminectomy and removal of the lower central portion
of the occipital bone
Intramedullary tumour excision
What is syringobulbia?
Syrinx in the medulla of the brainstem
Usually extension of the syringomyelia but can be isolated
Results in
o Horners
o Ataxia and nystagmus
o Bulbar palsy
o CN V, VII, IX and X especially
o Onion skin pattern of loss of pain sensation of the face