Emergency Diagnosis and Management

Patients with ALS most often will not present to the emergency department (ED) undiagnosed unless there is extremely rapid disease progression or a long period without medical care. Emergency management usually is required for acute respiratory failure, aspiration pneumonia, choking episodes, or trauma related to extremity weakness. A vital capacity drop below 25 mL/kg increases the risk of aspiration pneumonia and respiratory failure. Blood gas determination does not reliably predict impending respiratory failure, since mild hypoxia and hypercarbia may exist throughout the disease course. Although no acute therapies exist for worsening ALS, therapies that optimize pulmonary function (e.g., nebulized medications, steroids, antibiotics, intubation) are indicated. Because the need for long-term ventilatory assistance rarely reverses, it is important to establish the patient's preference regarding intubation via a living will or the power of attorney for health care. Hospitalization is indicated with impending respiratory failure, pneumonia, the inability to control secretions, or a worsening overall status that requires social service intervention for long-term placement.

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