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Assessment confirmed weakness of all limbs, more marked on the right side, with muscle atrophy. Reflexes were hyperactive and fasciculations were observed in the right leg.
Within two years, the patient became severely disabled because of generalized weakness. She experienced no sensory problems, intellectual deterioration or skin breakdown in spite of her degree of immobility. Difficulty in swallowing necessitated the use of a gastrostomy tube for feeding. The patient died of respiratory failure approximately three years after reporting the initial symptoms.
* ALS = amyotrophic lateral sclerosis; LMN = lower motor neuron; UMN = upper motor neuron; EMG = electromyographic...
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