Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease

He, Dian; Cai, Gang; Li, Yan; Ling, Qi; Xiao, Kang; Liu, ChunTing; Chu, Lan; Wu, Shan; Liu, Fang; You, Mingyao; Jiang, Weijia

doi:10.1097/wad.0000000000000242

Alzheimer Disease &Amp; Associated Disorders

2018

DOI: 10.1097/wad.0000000000000242

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Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease

Dian He

Gang Cai

Yan Li

et al.

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Cited by 2 publications

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Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports

Lu,

Cui,

Zhang

2023

JIR

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Primary angiitis of the adult central nervous system (PACNS) is an increasingly recognized but limited disease. Using previous case reports, we sought to summarize the clinical symptoms, imaging manifestations, treatment, and prognosis of patients with biopsy-confirmed PACNS to guide clinical diagnosis and management. Methods: We searched the Web of Science database for studies published from January 2000 to April 2023, with the language set to English and the document type limited to [Article or Review or Letter or Editorial Material]. A systematic review of all case reports met the inclusion and exclusion criteria was performed. These patients' clinical, pathological, and imaging characteristics were analyzed, and treatment and prognostic data were summarized. Results: We analyzed 69 articles, including 76 patients with biopsy-confirmed PACNS. And 57.9% of the patients were male, the median age at presentation was 47.0 years, and focal neurological deficits were the most common symptom in patients (78.9%), followed by headache (52.6%). The median duration of biopsy was 1.1 months, of which 49 (64.5%) patients were lymphocytic, 13 (17.1%) were granulomatous, 11 (14.5%) were amyloidotic, and 3 (3.9%) were necrotizing PACNS. Relapse events occurred in 41 (53.9%) patients, including 34 (44.2%) relapses and 8 (10.5%) deaths. Univariate logistic regression analysis revealed that symptomatic epilepsy, prolonged biopsy time window, and CD20 expression in pathological tissues might be independent risk factors for recurrent events in patients (HR=4.

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Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports

Lu,

Cui,

Zhang

2023

JIR

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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?

Jeon

Joseph

Jansen

et al. 2020

Viruses

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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.

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Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease

Cited by 2 publications

References 10 publications

Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports

Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports

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?

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