Optimizing care of patients with ALS. Steps to early detection and improved quality of life.

Primary care physicians should be alert for early, purely motor neurologic signs and symptoms of ALS, a progressive and ultimately fatal motor neuron disease. Because many neuromuscular disorders mimic ALS, careful differential diagnosis is essential. All patients with signs of motor neuron dysfunction need prompt referral to a neurologist at a regional ALS care center to ensure that they receive an accurate diagnosis and disease-specific multidisciplinary care. Once the diagnosis is confirmed, primary care physicians should work closely with the ALS center to ensure continuity of care and provide emotional support for both patient and family. Patients may also benefit from enrollment in treatment trials and the ALS CARE database. Riluzole, the only medication approved for treatment of ALS, has been shown to slow disease progression and prolong survival. Such benefits have provided new hope to patients and have spurred investigators to search for other, more effective medications for use alone or in combination.