A prospective study of 14 cases of motor neuron disease

Fourteen consecutive patients with motor neuron disease were studied during a period of one year. Seven patients were in the second decade of life, with eleven below the age of 40 years. Males accounted for 13 cases. Four had sustained some trauma in the past, one had poliomyelitis. Younger patients (average age 17.9 years) usually had an asymmetrical lower motor neuron lesion which was gradually progressive (the Norris score reached an average of 89.9 in a mean period of 36.9 months). A largely symmetrical distribution affecting both the upper and lower motor neurons and the lower cranial nerves with a rapidly downhill course (the Norris score fell to 80.3 in 12.9 months) were the features observed in older patients (average age 46.7 years). Cerebrospinal fluid protein was raised in four cases. Anti-neural antibodies were not demonstrable in any of the patients.