Severe cortical involvement in MV2 Creutzfeldt?Jakob disease: An autopsy case report
| Autor: | Jun Ichi Shiota, Kenji Ishihara, Mitsuru Kawamura, Masayuki Sugie, Imaharu Nakano, Tetsuyuki Kitamoto |
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| Rok vydání: | 2006 |
| Předmět: |
Pathology
medicine.medical_specialty Cerebellum Prions Blotting Western Neuropathology Creutzfeldt-Jakob Syndrome Pathology and Forensic Medicine Basal ganglia medicine Humans Pathological Aged Cerebral Cortex business.industry General Medicine medicine.disease Magnetic Resonance Imaging Hyperintensity medicine.anatomical_structure Cerebral cortex Female Autopsy Neurology (clinical) business Spongiosis |
| Zdroj: | Neuropathology. 26:433-437 |
| ISSN: | 1440-1789 0919-6544 |
| DOI: | 10.1111/j.1440-1789.2006.00705.x |
| Popis: | MV2 type sporadic Creutzfeldt-Jakob disease (sCJD) is reported to have a long duration and marked involvement of the cerebral deep gray matter. We describe an autopsied long-surviving sCJD case of MV2. In the early stages, the patient exhibited memory impairment, attention deficit and semantic memory disorder. Diffusion-weighted MRI showed abnormal hyperintensity signals along the cerebral cortex, sparing the thalami and basal ganglia. Pathological observations included: severe spongiosis throughout the cerebral cortex, several kuru plaques and plaque-like PrP deposits in the cerebellum, with only minimal degeneration in the thalami and basal ganglia. Our case suggests that MV2 has a wide clinicopathological spectrum, which ranges from "VV2" to "MM2" type. |
| Databáze: | OpenAIRE |
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