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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