1. What is the pathology behind ALS?
2. What is the clinical presentation of ALS?
3. What are predictors of poor survival?
4. What are the EMG findings of ALS?
Answers:
1. Degeneration of anterior horn cells.
2. Asymmetric atrophy, weakness, fasciculations, dysphagia, dysarthria, pseudobulbar signs (crying, laughing), sensation spared, EOMI, upper and lower motor neuron signs, B/B spared.
3. Older age, worse severity at onset, poor pulmonary function.
4. SNAP and CMAP normal. EMG showing abn activity in 2 muscles from 2 different nerve roots in 3 different body regions (brainstem, cervical, thoracic, lumbar), increased jitter and fiber density.
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