Syringomyelia
Tubular cavitation of spinal cord, usually cervical, then other segments; more frequent in men; sometimes familial.
Onset age 20 to 40. Slow progression; more rapid if brainstem affected (syringobulbia).
Chiari type 1 malformation in more than two thirds of cases; also seen with intramedullary spinal cord tumor or trauma.
Clinical Features
Lower cervical region: loss of pain, temperature perception; preserved light touch, vibratory sense (dissociated sensory loss) in “shawl” distribution. Cavity may enlarge to posterior columns (loss of position and vibratory sense in feet), anterior horns (weakness, wasting of hand muscles, fasciculations, loss of tendon reflexes in arms), lateral columns (spastic paraparesis, hyperreflexia in legs, Babinski signs), autonomic tracts (sphincter symptoms). Deep, aching pain.
Brainstem (syringobulbia): dysphagia, pharyngeal and palatal weakness, atrophy of tongue, loss of pain and temperature sense in distribution of trigeminal nerve, nystagmus.Related posts:
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