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Syringomyelia
From WikEM
Contents
Background
- Fluid filled cyst (syrinx) within the spinal cord which can expand or elongate over time, destroying surrounding spinal cord tissue.
- Can be due to dilation of the neural tissue surrounding the central can itself (which can lead to obstructive hydrocephalus), or it can arise from within the parenchyma of the spinal cord.
- Typically occurs in the C2 and T9 distribution.
- Most commonly occurs in patients with a pre-existing Chiari malformation.
- Usually seen in young adults
- Most commonly insidious in onset but the effects of the cyst may manifest suddenly after coughing or sneezing
- Two forms
- Congenital- secondary to arnold chiari malformation and associated with hydrocephalus
- Acquired- secondary to an insult to the brain or spinal cord such as stroke, trauma, meningitis, hemorrhage, or tumor
Clinical Features
- "Cape-like" distribution of:
- Paralysis
- Paresthesias
- Pain
- stiffness of the back, shoulders, and extremities
- loss of ability to sense extreme hot or cold in the hands
- insidious onset
Differential Diagnosis
- Glioma
- guillain-barré syndrome
- Acute myelopathy
- brain stroke
- spinal cord stroke
- central pontine myolinolysis
- malingering
- Pediatric Ependymoma
- Diabetic Neuropathy
- Multiple Sclerosis
- Transverse Myelitis
- Myasthenia Gravis
Evaluation
- Thorough history is going to be very important
- Neuro Exam
- cranial nerves
- sensation- including hot and cold
- proprioception and vibration- spared in syringomyelia due to sparing of the dorsal column and medial lemniscus
- strength, especially in distal muscle groups
Management
Disposition
- Assuming pain is controlled and no concern for respiratory compromise, discharge home.
- Neurology followup- to create and monitor pain management plan
- Neurosurgery- for potential surgical resection
