Intraneuronal Conglomerates in Sporadic Motor Neuron Disease

Abstract

IN THE ADULT, motor neuron disease or amyotrophic lateral sclerosis can be subdivided into several groups on the basis of clinical, pathologic, and epidemiologic features.¹ The histologic changes observed in the classic sporadic cases include nonspecific degeneration and loss of motor neurons in the spinal cord, brain stem, and cerebral cortex. Demyelination of the pyramidal tracts often accompanies the neuronal losses.² A familial variant further characterized by demyelination of the posterior columns and spinocerebellar tracts has been reported.³ In a few residual motor neurons from these cases, Hirano et al⁴ described accumulations of hyalinized material, occasionally with acidophilic cores that resemble Lewy bodies. In still another variant, the amyotrophic lateral sclerosis and Parkinsonism-dementia complex on Guam, neurofibrillary tangles have been observed in the anterior horn cells.⁵ This report presents light and electron microscopic observations from an unusual sporadic case of motor neuron disease characterized by