Syringomyelia

Background

Sensory dermatome by spinal level.

• 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 canal 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

Types

• Congenital
  • Secondary to Arnold Chiari malformation
  • Associated with hydrocephalus
• Acquired
  • Secondary to an insult to the brain or spinal cord
  • Examples: stroke, trauma, meningitis, hemorrhage, or tumor

Clinical Features

• Insidious onset
• "Cape-like" distribution of:
  • Paralysis
  • Paresthesia
  • Pain
• Stiffness of the back, shoulders, and extremities
• Loss of ability to sense extreme hot or cold in the hands
• Proprioception and vibration- spared due to sparing of the dorsal column and medial lemniscus

Differential Diagnosis

• Brain tumor
• Guillain-Barré Syndrome
• Acute myelopathy
• Brain stroke
• Spinal cord stroke
• Central pontine myelinolysis
• Pediatric ependymoma
• Diabetic neuropathy
• Multiple Sclerosis
• Transverse Myelitis
• Myasthenia Gravis

Evaluation

An idiopathic syrinx on MRI.

• Imaging
  • CT to rule out mass, bleed, and hydrocephalus
  • MRI brain and spine will make the diagnosis though this may not be indicated in the ED setting

Management

• Ensure no respiratory involvement and consider intubation if severe
• Analgesia

Disposition

• Assuming pain is controlled and no concern for respiratory compromise, discharge home.
• Neurology follow-up- to create and monitor pain management plan
• Neurosurgery- for potential surgical resection

See Also

• Epidural compression syndromes