Syringomyelia Flashcards
What is syringomyelia?
abnormal fluid filled cavity (syrinx) within the central canal of the spinal cord
What causes development of a syrinx?
Disrupted CSF drainage from the central canal, commonly caused by a Chiari malformation or previous trauma to the cervical/ thoracic spine
.
What is Syringobulbia?
fluid-filled cavity within the medulla of the brainstem
List 4 causes of syringomyelia
Chiari malformation
Trauma
Tumours
Idiopathic
List 5 features of of syringomyelia
Cape-like loss of sensation to pain + temperature
Spastic weakness
Neuropathic pain
Upgoing plantars
Autonomic features
Describe the cape like distribution of sensory disturbance
Affects neck, shoulders + arms
What sensory modalities are preserved in syringomyelia?
Light touch, proprioception + vibration
What causes the cape-like loss of sensation to pain and temperature in syringomyelia?
Compression of the anterior white commissure + damage to crossing neural fibers of the lateral spinothalamic tract first → bilateral dissociated sensory loss
List 4 autonomic features of syringomyelia
Horner syndrome (compression of sympathetic chain, rare)
Bowel + bladder dysfunction
Anhidrosis
Erectile dysfunction
What investigations are used for syringomyelia?
Full spine MRI with contrast: to r/o a tumour or tethered cord
Brain MRI: to r/o a Chiari malformation
Describe management of syringomyelia
Conservative Mx usually sufficient
If progressive neurological Sx: surgery
What surgical management may be required for syringomyelia?
Removal of tumour if present
Decompression by drainage of CSF e.g. with cerebral shunt
What medications may be helpful in syringomyelia?
Analgesia: Amitriptyline
Baclofen for spasticity
How can expansion of the syrinx and damage to LMNs in the anterior horns at the segment level present?
Initially bilateral weakness
→ eventual bilateral flaccid paresis + muscle atrophy
Typically begins in hands
How can expansion of the syrinx and damage to UMNs in the medial part of the lateral corticospinal tract present?
uni-/bi-lateral spastic paresis BELOW the level of the syrinx
