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SYRINGOMYELIA

Definition
  • Pathology: Cavitation & gliosis
  • Location
    • Spinal cord: Usually cervical or thoracic
    • Medulla
  • Course: Chronic
Clinical syndrome
  • Epidemiology
    • Onset: Most commonly between ages 25 to 40
    • Males somewhat > Females
  • Clinical features: Variable depending on anatomical involvement
    • Anterior horns
      • Weakness & Wasting: Especially in hands & arms
      • Fasciculations
    • Posterior horns & Decussating sensory fibers
      • Sensory loss: Pain & Temperature
      • Distribution: "Suspended" sensory loss
        • Involves: Arms & Trunk
        • Spares: Legs
        • Involvement of sacral areas may suggest association with neoplasm
      • Pain: Occasional; Boring or lancinating
      • Large fiber sensations: Usually preserved
      • Tendon reflexes: Reduced or absent in arms
    • Autonomic pathways
      • Horner's syndrome
      • Skin: Trophic changes
      • Neurogenic bladder
    • Corticospinal tracts: Variable involvement
      • Spastic paraparesis: Legs > Arms
    • Skeletal
      • Scoliosis
    • Cranial nerve involvement: With syringobulbia
      • Most often unilateral
      • XII: Tongue weakness & hemiatrophy
      • IX - X: Dysphagia; Dysarthria
      • XI: Weakness & wasting of sternomastoid & trapezius
      • VII: Facial paresis
      • Descending tract of V
        • Reduced pain & temperature on lateral face
        • "Onion skin" distribution
        • Reduced corneal sensation & reflex
  • Course: Very slow progression
  • Radiology: A; B
  • Surgical treatment: Posterior fossa decompression1
  • Variant syndrome: Presyrinx2
    • Spinal cord edema
      • Caused by alterations in CSF flow at foramen magnum
      • Location: Cervical or Thoracic
    • Associated with disorders that disrupt CSF flow
      • Chiari I & II malformations
      • Trauma
      • Subarachnoid hemorrhage
      • Bacterial meningitis
      • Cervical stenosis
      • Posterior fossa arachnoid cyst
      • Spinal arachnoiditis
      • Neurosarcoidosis
    • Clinical
      • Onset age: 1 year to Adult; Childhood with Chiari malformation
      • Course
        • Onset may be acute or subacute
        • Some with chronic progression
        • MRI change may be asymptomatic
      • Weakness: Arms or Quadriparesis
      • Headache
      • Bulbar: Dysphagia & Gagging
    • MRI
      • T2 prolongation
      • T1 prolongation
      • Cord enlargement without enhancement
    • Treatment: Reversible with surgery
Associated disorders
   



Bramwell



T1                                  T2

Return to Spinal disorders

References
1. Childs Nerv Syst 2009;25:453-459
2. Neurology 2008;71:351-356

2/22/2010

 

Link to actual page on Washington University School of Medicine website:

 http://neuromuscular.wustl.edu/spinal/syrinx.htm

 

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