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[Chronic encephalitis with mesencephalic predominance. A clinico-pathologic case].

F Dubas1, I Pénisson-Besnier, A Pouplard-Barthelaix

  • 1Service de Neurologie A, CHU, Angers.

Revue Neurologique
|January 1, 1990
PubMed
Summary

A rare case of polioencephalomyelitis presented with progressive supranuclear ophthalmoplegia, dementia, and midbrain involvement. Post-mortem analysis revealed subacute encephalitis, suggesting a link between this neurological condition and specific brainstem lesions.

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Presents a rare case of a 70-year-old woman with progressive supranuclear ophthalmoplegia, axial akinesia, and dementia.
  • Initial CT scan revealed mild cortico-subcortical atrophy.
  • Cerebrospinal fluid (CSF) analysis showed high immunoglobulins with an oligoclonal pattern, but no cell reaction.

Observation:

  • The patient exhibited eyelid opening apraxia, a key feature of progressive supranuclear ophthalmoplegia.
  • Neuropathological examination was limited to the central nervous system (CNS).

Findings:

  • Post-mortem examination revealed subacute encephalitis.
  • The encephalitis was localized to the tectal, pretectal, subthalamic areas, and Ammon's horns.
  • These findings strongly suggest polioencephalomyelitis, potentially with an underlying cancer, characterized by unusual midbrain involvement.

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Implications:

  • Highlights an exceptional presentation of polioencephalomyelitis with significant midbrain and hippocampal involvement.
  • Suggests the need for considering polioencephalomyelitis in cases of progressive supranuclear ophthalmoplegia with specific neuropathological findings.
  • Underscores the importance of post-mortem examinations in understanding rare neurological diseases.