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

被引：8

作者：

Wiels, Wietse A. ^{[1
]}

Du Four, Stephanie ^{[1
]}

Seynaeve, Laura ^{[1
]}

Flamez, Anja ^{[1
]}

Tousseyn, Thomas ^{[2
,3
]}

Thal, Dietmar ^{[2
,4
]}

D'Haeseleer, Miguel ^{[1
,5
]}

机构：

[1] Vrije Univ Brussel, Univ Ziekenhuis Brussel, Ctr Neurosci, Dept Neurol, Brussels, Belgium

[2] Univ Ziekenhuis Leuven, Dept Pathol, Leuven, Belgium

[3] Univ Zekenhuis Leuven, Dept Imaging & Pathol, Tansfat Cell & Tissue Res Lab, Leuven, Belgium

[4] Katholieke Univ Leuven, Dept Neurosci, Lab Neuropathol, Leuven, Belgium

[5] Natl Multiple Sclerose Centrum, Melsbroek, Belgium

来源：

FRONTIERS IN NEUROLOGY | 2018年 / 9卷

关键词：

Creutzfeldt-Jakob disease; VV1; type; progressive encephalomyelitis with rigidity and myoclonus; glycine receptor antibodies; dementia; NEURONAL ANTIBODIES; SUBTYPE; VV1;

D O I：

10.3389/fneur.2018.00242

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

100204 [神经病学];

摘要：

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.

引用

页数：5

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