A Unique Case of Genetic Creutzfeldt-Jakob Disease (E200K Mutation) with CSF-restricted Anti Myelin Oligodendrocyte Glycoprotein Antibody.

Takahiro Shimizu, Emiko Horiuchi, Takashi Matsukawa, Tsutomu Yasuda, Hideji Hashida

Abstract readCase Reports

In one paragraph

Article in Internal medicine (Tokyo, Japan), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 authors.

Takahiro ShimizuDepartment of Neurology, Japanese Red Cross Medical Center, Japan.

Emiko HoriuchiDepartment of Neurology, Japanese Red Cross Medical Center, Japan.

Takashi MatsukawaDepartment of Neurology, Graduate School of Medicine, The University of Tokyo, Japan.

Tsutomu YasudaDepartment of Neurology, Japanese Red Cross Medical Center, Japan.

Hideji HashidaDepartment of Neurology, Japanese Red Cross Medical Center, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder that can share clinicoradiologic features with autoimmune cortical encephalitis (CE); however, the pathological significance of myelin oligodendrocyte glycoprotein (MOG)-IgG, which is one of the causes of CE, has not yet been explored in the context of CJD. We herein present the case of a man in his mid-70s with a family history of CJD and rapidly progressive cognitive decline. Genetic testing confirmed the E200K mutation in PRNP. CSF analysis revealed CSF-restricted MOG-IgG that became undetectable after corticosteroid therapy without clinical improvement. This case highlights the need for cautious interpretation of the CSF-restricted MOG-IgG in CJD.

Indexed as

AutoantibodiesCreutzfeldt-Jakob SyndromeMutationMyelin-Oligodendrocyte GlycoproteinAgedHumansImmunoglobulin GMalePrion ProteinsAutoantibodiesImmunoglobulin GMOG protein, humanMyelin-Oligodendrocyte GlycoproteinPrion ProteinsPRNP protein, humancortical encephalitisCreutzfeldt-Jakob diseaseMOG antibody-associated diseasemyelin oligodendrocyte glycoprotein (MOG)-IgG

Identifiers

PMID40603096

DOI10.2169/internalmedicine.5715-25

PMCPMC12900607

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.