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P4‐257: Discriminant validity for the proposed NINDS‐CSN harmonization vascular cognitive impairment neuropsychological assessment protocols
Author(s) -
Nyenhuis David,
Geary Elizabeth,
Rizkalla Mireille N.,
Ganda Anoop,
Honjo Kie,
Pedelty Laura,
Black Sandra E.,
Stuss Donald T.,
Stebbins Glenn T.
Publication year - 2009
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2009.04.622
Subject(s) - neuropsychology , stroke (engine) , neuropsychological assessment , cognition , medicine , montreal cognitive assessment , psychology , cognitive impairment , physical therapy , psychiatry , mechanical engineering , engineering
a marked demyelination of the white matter and/or cortical involvement with brain atrophy, demonstrated by magnetic resonanz imaging is present. DMS type 3 is associated with older age with or without major motor disability. In these cases we found no clear correlation between the course and severity of MS and the dementia syndrome itself. Conclusions: In ptients with MS the development of dementia is very heterogenous. DMS type 1 and 2 are directly associated with the progression of the chronic autoimmune disease and the involvement of cortical and/or white matter structures. In DMS type 3 cases a comorbid condition with Alzheimer Dementia is discussed. It seems as DMS is not as rare as described in literature. Further epidemiologic studies are necessary to evaluate the incidence of DMS in a greater population homozygosity at the 129 codon and proved a new four OPRI on one allele with the following repeat order: R1R2R2R3R3R2R2R3R4. At autopsy, diffuse spongiosity, neuronal loss and gliosis were seen but pathological prion protein deposition was seen in the molecular layer of the cerebellum only as atypical, plaque-like patches. Conclusions: Our patient is the seventh published one with 4 OPRI of the PRNP: the order of the repeats is unique to this case and the clinical picture was atypical because of the prominent amyotrophy. The neuropathological findings with plaque-like prion protein deposits in the cerebellar molecular layer are also atypical. Our case emphasizes the heterogeneity of genetic prion diseases.