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Severe cortical involvement in MV2 Creutzfeldt–Jakob disease: An autopsy case report
Author(s) -
Ishihara Kenji,
Sugie Masayuki,
Shiota Junichi,
Kawamura Mitsuru,
Kitamoto Tetsuyuki,
Nakano Imaharu
Publication year - 2006
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.2006.00705.x
Subject(s) - spongiosis , basal ganglia , pathology , cerebral cortex , hyperintensity , cerebellum , autopsy , neuropathology , thalamus , pathological , medicine , cerebrum , neuroscience , magnetic resonance imaging , anatomy , disease , central nervous system , psychology , radiology
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.