- Uncurable motor neuron degenerative disease.
- May present to ED as:
- Abnormal gait (falls)
- "Radiculopathy" (eps. cervical)
- "Footdrop"/"Wristdrop" Saturday night palsy.
- Bulbar "event" (e.g. slurred speech or aspiration).
- M:F = 2:1, any age (incidence rising with age).
- 80% present with limb weakness ("amyotrophy" = muscle fibre atrophy), NB foot drop or loss of finger dexterity.
- 20% with bulbar symptoms, slurred speech, poor swallow, aspiration.
- Initial flaccid, then ±spastic cramps.
- Later cognitive and emotional fluctuations.
- Mixed UMN and LMN signs in different body regions.
- No sensory signs in ED
Very early presentation may "mimic" others:
- EMG - Normal sensory, abnormal motor conduction.
- If suspected, admit neurology for workup and management plan.
- Consider, respiratory support, analgesia etc.