Neuromuscular

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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
      • Loss of pain & temperature sensation
      • Distribution: Usually "suspended"
        • Involving arms & trunk
        • Sparing legs
      • Pain: Occasional; Boring or lancinating
      • Large fiber sensations: Usually preserved
    • Autonomic pathways
      • Horner's syndrome
      • Skin: Trophic changes
      • Neurogenic bladder
    • Corticospinal tracts: Variable involvement
      • Spastic paraparesis: Legs > arms
    • Other
      • Scoliosis
      • Tendon reflexes: Reduced or absent in arms
    • 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
Associated disorders
   

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4/25/2003