Creutzfeldt-Jakob Disease with a Five-Year Clinical Course, Multicentric Cerebellar Prion Plaques and Prior History of Biopsy-Proven Primary Angiitis of the Central Nervous System: A Case for Iatrogenic Exposure?
Viruses
; 12(12)2020 12 08.
Article
en En
| MEDLINE
| ID: mdl-33302561
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative disease that can arise spontaneously, genetically, or be acquired through iatrogenic exposure. Most patients die within a year of symptom onset. It is rare, affecting 1-2 per million per year, and the majority of cases are sporadic. Primary angiitis of the central nervous system (PACNS) is also rare, affecting 2.4 per million per year. We present a case of an unusually long clinical course of CJD, almost five years, which began with symptoms of apraxia. The patient had biopsy-proven PACNS 16 years prior to clinical presentation, and the site of biopsy was the left parietal lobe. Autopsy revealed multicentric prion plaques in the cerebellum, in the setting of normal genetic testing. The presence of plaques in the cerebellum, and prior neurosurgery, raises the possibility of iatrogenic exposure. We present the details of this case, including pathology from the original biopsy and final autopsy, as well as a review of relevant cases in the literature.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Priones
/
Cerebelo
/
Síndrome de Creutzfeldt-Jakob
/
Vasculitis del Sistema Nervioso Central
Tipo de estudio:
Prognostic_studies
Límite:
Humans
/
Middle aged
Idioma:
En
Revista:
Viruses
Año:
2020
Tipo del documento:
Article
País de afiliación:
Canadá
Pais de publicación:
Suiza