Presenile non-Alzheimer dementia with motor neuron disease and laminar spongiform degeneration

Masaru Mimura, Itaru Tominaga, Haruo Kashima, Masao Honda, Kenji Kosaka, Yuji Kato

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)


We describe the clinicopathological findings in three autopsy cases of presenile dementia with motor neuron disease. These patients had a relatively rapid course involving dementia and muscle weakness with a distal pattern of atrophy in the upper extremities. Postmortem examination revealed features of motor neuron disease and spongiform cortical degeneration. The latter change was most marked in the second layer of the frontal or temporal cortex and included minimal to mild neuronal cell loss and mild to moderate gliosis. In this report we relate these patients' laminar spongiform degeneration to three other conditions; frontal lobe dementia, primary progressive aphasia and dementia lacking a distinctive histology. These three conditions and presenile dementia with motor neuron disease may fall within the spectrum of the non-Alzheimer type frontotemporal degenerative dementia.

Original languageEnglish
Pages (from-to)19-26
Number of pages8
Issue number1
Publication statusPublished - 1998
Externally publishedYes


  • Frontal lobe dementia
  • Motor neuron disease
  • Presenile dementia
  • Primary progressive aphasia
  • Spongiform degeneration

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology


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