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Related Experiment Videos

Multiple system atrophy with remarkable frontal lobe atrophy.

M Konagaya1, M Sakai, Y Matsuoka

  • 1Department of Neurology, Suzuka National Hospital, Mie, Japan.

Acta Neuropathologica
|April 20, 1999
PubMed
Summary

Autopsy revealed multiple system atrophy (MSA) with significant frontal lobe atrophy and glial cytoplasmic inclusions (GCIs). This case highlights potential cerebral lobe involvement in MSA progression.

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Area of Science:

  • Neuropathology
  • Neurodegenerative Diseases

Background:

  • Multiple System Atrophy (MSA) is a rare, sporadic neurodegenerative disorder.
  • Characterized by autonomic dysfunction, parkinsonism, and cerebellar or pyramidal signs.

Observation:

  • Autopsy of a 65-year-old female with a 13-year history of parkinsonism, dysautonomia, and progressive motor aphasia.
  • Gross examination revealed severe cerebral atrophy, predominantly in the frontal lobe, striatum, pons, and cerebellum (brain weight 810g).
  • Microscopic analysis showed laminar gliosis, sponginess, and neuronal loss in specific cortical layers of the frontal and parietal lobes.

Findings:

  • Abundant glial cytoplasmic inclusions (GCIs) were observed in deep cortical layers and white matter, particularly in the frontal and parietal lobes.
  • Severe degeneration of the olivopontocerebellar and striatonigral systems was noted.

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  • Absence of Pick bodies, Lewy bodies, and significant neurofibrillary tangles confirmed the diagnosis of MSA.

    • Implications:

    • This case demonstrates significant frontal lobe atrophy in MSA, suggesting involvement of cerebral lobes beyond typical affected areas.
    • The findings expand the understanding of MSA's neuropathological spectrum.
    • Highlights the need to consider cerebral involvement in MSA, especially with progressive aphasia.