Motor neuron disease, parkinsonism and dementia. Report of a case with diffuse Lewy body-like intracytoplasmic inclusions

Abstract

The clinicopathological findings in a 49-year-old man who presented multisystemic neurological degenerative disease are reported. The patient presented, at the age of 36, distal upper limb amyotrophy and 8 years later pyramidal signs with fasciculations. In his last year of life, he suffered extrapyramidal hypertonus and mental deterioration. Neuropathological examination showed anterior spinal root and cerebral atrophy. Myelin pallor was mild and predominated on posterior cords. Anterior horn neuronal loss was noted in the spinal cord as well as Alzheimer-type changes in the brain. Inclusion bodies consistent with Lewy bodies were diffusely apparent. The peculiar clinical progression in this case and the extension of neuropathological lesions with inclusion bodies mainly in the substantia nigra and cerebral cortex are an interesting subject of discussion.