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Motor neuron diseases are characterized by weakness and wasting of affected muscles, without accompanying sensory changes. Motor neuron disease in adults generally commences between 30 and 60 years of age. There is degeneration of the anterior horn cells in
Classification
Five varieties have been distinguished on clinical grounds.
Primary Lateral Sclerosis: There is a purely upper motor neuron deficit in the limbs.
Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease): A mixed upper and lower motor neuron deficit is found in the limbs. This disorder is sometimes associated with dementia or parkinsonism.
Symptoms and Signs: Difficulty in swallowing, chewing, coughing, breathing, and talking (dysarthria) occur with bulbar involvement. In progressive bulbar palsy, there is
The disorder is progressive and usually fatal within 3B5 years; death usually results from pulmonary infections. Patients with bulbar involvement generally have the poorest prognosis.
Treatment
Riluzole, which reduces the presynaptic release of glutamate, may Symptomatic and supportive measures may include prescription of anticholinergic drugs (such as trihexyphenidyl, amitriptyline, or atropine) if Spasticity may be helped by baclofen or diazepam. A semiliquid