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Publicatie

Early-Onset Creutzfeldt-Jakob Disease Mimicking Immune-Mediated Encephalitis

Tijdschriftbijdrage - Tijdschriftartikel

Objectives: The objective of this study is to explore the clinical, radiological, and pathological manifestations of a rare subtype of prion disease and their implication for differential diagnosis in case of an early onset neuropsychiatric deterioration.

Methods: We discuss a patients' clinical history, as well as the string of investigations and symptomatological evolution that finally led to a pathological diagnosis.

Results: Our patient had the extremely rare VV1 type sporadic Creutzfeldt-Jakob disease (sCJD). We explain the differential diagnosis of progressive encephalomyelitis with rigidity and myoclonus and its implications for treatment.

Conclusion: sCJD, especially the VV1 subtype, can present at an early age with an insidious psychiatric onset. Classical findings of prion disease-14-3-3 protein, PSWC on electroencephalography, and magnetic resonance imaging patterns-are not always present. The presence of neural autoantibodies does not always implicate pathogenicity in the presence of other neurological/neurodegenerative conditions.

Tijdschrift: Front Neurol
ISSN: 1664-2295
Issue: APR
Volume: 9
Jaar van publicatie:2018
Trefwoorden:Creutzfeldt-Jakob disease, Dementia, Glycine receptor antibodies, Progressive encephalomyelitis with rigidity and myoclonus, VV1 type
BOF-keylabel:ja
BOF-publication weight:1
CSS-citation score:1
Auteurs:International
Authors from:Hospital, Higher Education
Toegankelijkheid:Open