Motor neuron disease with parkinsonism

Objective: To report clinical characteristics of patients with combined features of parkinsonism and motor neuron disease (MND). Design: Medical chart review. Setting: University medical center. Patients: Thirteen patients, identified by computer-assisted search, who had diagnoses of both parkinsonism and MND. Results: Median age was 68 years. There were 7 men and 6 women. All had clinical and electrodiagnostic evidence of both upper and lower motor neuron degeneration. One or more clinical manifestations of parkinsonism were observed in all patients either before the diagnosis of MND (n=2), at the time of initial evaluation (n=10), or after the diagnosis of MND (n=1). The median time from symptom onset to presentation was 18 months. Improvement was seen in 5 of the 11 patients treated with levodopa. Dementia and autonomic dysfunction were absent in all patients. Postmortem neuropathological evaluation, available in 1 patient, demonstrated degeneration of the substantia nigra with Lewy bodies, mild pallor of the medullary pyramids, and neurogenic atrophy of the skeletal muscle. Conclusion: The onset of MND and parkinsonism within a relatively short period in most of our patients favors a common pathogenic mechanism over coincidental occurrence of 2 unrelated diseases. In patients with MND, it is important to recognize signs of parkinsonism that levodopa might alleviate.