Syringomyelia: Difference between revisions
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==Background== | ==Background== | ||
[[File:Dermatoms alt.png|thumb|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. | *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 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. | *Typically occurs in the C2 and T9 distribution. | ||
*Most commonly occurs in patients with a pre-existing Chiari malformation. | *Most commonly occurs in patients with a pre-existing Chiari malformation. | ||
*Usually seen in young adults | *Usually seen in young adults | ||
*Most commonly insidious in onset but the effects of the cyst may manifest suddenly after coughing or sneezing | *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== | ==Clinical Features== | ||
*Insidious onset | |||
*"Cape-like" distribution of: | *"Cape-like" distribution of: | ||
**Paralysis | **[[Weakness|Paralysis]] | ||
** | **[[Paresthesia]] | ||
**Pain | **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== | ==Differential Diagnosis== | ||
*[[ | *[[Brain tumor]] | ||
*[[ | *[[Guillain-Barré Syndrome]] | ||
*Acute myelopathy | *Acute myelopathy | ||
* | *Brain [[stroke]] | ||
* | *Spinal cord [[stroke]] | ||
*[[Central pontine myelinolysis]] | |||
*[[ | *Pediatric ependymoma | ||
*Pediatric | *[[Diabetic neuropathy]] | ||
*Diabetic | |||
*[[Multiple Sclerosis]] | *[[Multiple Sclerosis]] | ||
*[[Transverse Myelitis]] | *[[Transverse Myelitis]] | ||
*[[ | *[[Myasthenia Gravis]] | ||
==Evaluation== | ==Evaluation== | ||
[[File:Syringomyelia (with arrow).png|thumb|An idiopathic syrinx on MRI.]] | |||
*Imaging | |||
**[[head CT|CT]] to rule out [[intracranial mass|mass]], [[ICH|bleed]], and [[hydrocephalus]] | |||
**[[brain MRI|MRI brain]] and spine will make the diagnosis though this may not be indicated in the ED setting | |||
==Management== | ==Management== | ||
*Ensure no respiratory involvement and consider intubation if severe | |||
*[[Analgesia]] | |||
==Disposition== | ==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== | ==See Also== | ||
*[[Epidural compression syndromes]] | |||
==External Links== | ==External Links== | ||
Latest revision as of 11:32, 24 October 2020
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 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
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- 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