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Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: an autopsy case report

Kenji Ishihara1, Masayuki Sugie, Jun-Ichi Shiota

  • 1Department of Neurology, Showa University School of Medicine, Hatanodai 1-5-8, Shinagawa-ku, Tokyo 142-8666, Japan. k-ishihara@mvj.biglobe.ne.jp

Neuropathology : Official Journal of the Japanese Society of Neuropathology
|November 4, 2006
PubMed

Insights

This study details a rare, long-surviving case of MV2 type sporadic Creutzfeldt-Jakob disease (sCJD). The findings reveal a broader clinicopathological spectrum for MV2 sCJD than previously understood.

Area of Science:

  • Neurology
  • Prion Diseases
  • Neurodegenerative Disorders

Background:

  • Sporadic Creutzfeldt-Jakob disease (sCJD) encompasses various subtypes with distinct clinical and pathological features.
  • The MV2 subtype is typically associated with prolonged disease duration and significant deep gray matter involvement.

Observation:

  • A case of long-surviving MV2 sCJD presented with early memory, attention, and semantic deficits.
  • Diffusion-weighted MRI revealed cortical hyperintensities but spared the thalami and basal ganglia.
  • Autopsy showed severe cerebral cortical spongiosis, cerebellar PrP deposits, and minimal thalamic/basal ganglia degeneration.

Findings:

  • The observed clinicopathological presentation expands the known spectrum of MV2 sCJD.
  • This case bridges features typically seen in VV2 and MM2 sCJD subtypes.
  • The findings challenge the presumed strict localization of pathology in MV2 sCJD.

Implications:

  • Understanding the wide spectrum of MV2 sCJD is crucial for accurate diagnosis and patient management.
  • This case highlights the importance of integrating clinical, imaging, and pathological data in sCJD classification.
  • Further research is needed to elucidate the mechanisms underlying the diverse presentations of MV2 sCJD.

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