Diagnosis of Amyotrophic Lateral Sclerosis

TREATMENT

To date, riluzole is the sole Food and Drug Administration (FDA)-approved drug for the treatment of ALS. Several randomized trials have demonstrated that riluzole prolongs the life of ALS patients by 2 to 3 months. Until more effective drug therapies are discovered, symptomatic control and emotional support are the primary therapeutic goals because these diseases usually progress relentlessly to death in 2 to 10 years. Fortunately, there have been significant advances in the realm of supportive therapy for ALS.

Feeding difficulties are frequently related to an inability to move food about in the mouth and to swallow effectively. Some patients may manage food prepared in a blender. Instructing the patient in chin tuck and other mechanical maneuvers can facilitate safe swallowing. When oral intake becomes unsafe or ineffective at maintaining weight, a feeding gastrostomy tube can be placed.

Numerous adaptive devices and machines can facilitate functional independence. Mobility can be maintained with scooters and motorized wheelchairs. Transfers can be facilitated with ramps, lifts, and boards. Grab bars, commodes, and structural adjustments to the home environment can improve access. Several adaptive devices, including strategic foot bracing, arm boards and foam grips can enhance function (see Plate F). Physical therapy regimens can maintain flexibility and avoid frozen joints.

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