Syringomyelia

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
• Notable Labs
  • inflammatory markers
  • LP
• 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
• Neurology consult
• pain management including some combination of NSAIDs, narcotics, and nerve modulators such as Gabapentin/lyrica

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

References

Authors

Jay, Anthony Lucero, Michael Holtz