Disease Management

Therapy is designed to enhance muscle function, especially those which support breathing, swallowing, and speech, in order to avoid malnutrition, recurrent aspiration, or choking. Two drugs that may delay disease progression include riluzone and gabapentin, both of which prevent cell toxicity through modulation of the excitotoxin glutamate. Respiratory insufficiency is suggested by atrophy of the deltoids and trapezii, and a decrease in forced vital capacity by 50 percent increases the risk of respiratory failure. Pulmonary function testing, respiratory therapy, incentive spirometry, and intermittent positive-pressure breathing, intermittent negative-pressure (external) ventilation, and long-term assisted ventilation all can enhance the quality of life as diaphragm weakness progresses.

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