Abstract
The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.
Similar content being viewed by others
Author information
Authors and Affiliations
Additional information
Received: 9 November 1999 / Revised, accepted: 10 December 1999
Rights and permissions
About this article
Cite this article
Knirsch, U., Bachus, R., Gosztonyi, G. et al. Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism. Acta Neuropathol 100, 342–346 (2000). https://doi.org/10.1007/s004010000185
Issue Date:
DOI: https://doi.org/10.1007/s004010000185