Abstract
Thirty-one cases of motor neuron disease (MND) were diagnosed in residents of Lehigh County, Pennsylvania, in 1968–1975. Independently reviewed by two neurologists, these cases satisfy explicit diagnostic criteria for one of the three variants of MND—amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), and progressive bulbar palsy (PBP). The annual incidence of MND adjusted for sex and age to the 1950 United States population was 1.32 cases per 100,000 population per year; this rate was almost identical to the only other reported rate for an area of the continental United States. The point prevalence for Lehigh county on 15 November, 1975 was 6.4 cases per 100,000 population.
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