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Pick's disease: clinicopathologic characterization of 21 cases.
Journal of Neurology ( IF 4.8 ) Pub Date : 2020-05-21 , DOI: 10.1007/s00415-020-09927-9
Parichita Choudhury 1 , Eugene L Scharf 1 , Michael A Paolini 2 , Jonathan Graff-Radford 1 , Eva C Alden 3 , Mary M Machulda 3 , David T Jones 1 , Julie A Fields 3 , Melissa E Murray 4 , Neill R Graff-Radford 5 , Eleni Constantopoulos 6 , Ross R Reichard 6 , David S Knopman 1 , Joseph R Duffy 1 , Dennis W Dickson 4 , Joseph E Parisi 1, 6 , Keith A Josephs 1 , Ronald C Petersen 1 , Bradley F Boeve 1
Affiliation  

BACKGROUND Pick's disease (PiD) is a unique subtype of frontotemporal lobar degeneration characterized pathologically by aggregates of 3-Repeat tau. Few studies have examined the clinical variability and disease progression in PiD. We describe the clinical features, neuropsychological profiles and coexistent pathologies in 21 cases of autopsy-confirmed PiD. METHODS This study was a retrospective analysis of patients with Pick's disease evaluated at Mayo Clinic, Rochester or Jacksonville (1995-2018), and identified through an existing database. RESULTS Twenty-one cases with sufficient clinical data were identified. Behavioral variant FTD (bvFTD; 12/21) was the most common phenotype, followed by primary progressive aphasia (PPA; 7/21), corticobasal syndrome (CBS; 1/21) and amnestic dementia (1/21). Median age at disease onset was 54 years, with PPA cases (median = 52 years) presenting earlier than bvFTD (median = 59). Median disease duration (onset-death) overall was 10 years and did not differ significantly between bvFTD (median = 9.5 years) and PPA (median = 13). Age at death was not significantly different in PPA (median = 66) compared to bvFTD (median = 68.5). A third of the cases (n = 7/21) demonstrated pure PiD pathology, while the remainder showed co-existent other pathologies including Alzheimer's type (n = 6), cerebral amyloid angiopathy (n = 3), combined Alzheimer's and amyloid angiopathy (n = 4), and Lewy body disease (n = 1). CONCLUSIONS Our study shows that bvFTD and PPA are the most common clinical phenotypes associated with PiD, although rare presentations such as CBS were also seen. Coexisting non-Pick's pathology was also present in many cases. Our study highlights the clinical and pathologic heterogeneity in PiD.

中文翻译:

皮克氏病:21例的临床病理特征。

背景匹克氏病(PiD)是额颞叶变性的一种独特亚型,病理上以3-重复tau的聚集体为特征。很少有研究检查PiD的临床变异性和疾病进展。我们描述了21例经过尸检确认的PiD的临床特征,神经心理特征和共存的病理。方法本研究是对Mayo Clinic,Rochester或Jacksonville(1995-2018)评估的Pick病患者的回顾性分析,并通过现有数据库进行鉴定。结果确定了21例具有足够临床数据的病例。行为变异性FTD(bvFTD; 12/21)是最常见的表型,其次是原发性进行性失语症(PPA; 7/21),肾上腺皮质综合征(CBS; 1/21)和失忆性痴呆(1/21)。发病年龄中位数为54岁,PPA病例(中位数= 52岁)的出现早于bvFTD(中位数= 59)。中位疾病持续时间(发病死亡)总体为10年,bvFTD(中位= 9.5年)和PPA(中位= 13)之间无显着差异。与bvFTD(中位数= 68.5)相比,PPA(中位数= 66)的死亡年龄无显着差异。三分之一的病例(n = 7/21)表现为单纯的PiD病理,而其余病例则并存其他病理,包括阿尔茨海默氏病(n = 6),脑淀粉样血管病(n = 3),合并的阿尔茨海默氏病和淀粉样血管病( n = 4)和路易体病(n = 1)。结论我们的研究表明bvFTD和PPA是与PiD相关的最常见的临床表型,尽管也很少见到诸如CBS的表现。在许多情况下,非皮克氏病也并存。
更新日期:2020-05-21
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