Pick's disease: clinicopathologic characterization of 21 cases.
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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 54years, with PPA cases (median=52years) presenting earlier than bvFTD (median=59). Median disease duration (onset-death) overall was 10years and did not differ significantly between bvFTD (median=9.5years) 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.
Choudhury, P., Scharf, E. L., Paolini, M. A., Graff-Radford, J., Alden, E. C., Machulda, M. M., ... Boeve, B. F.
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Choudhury, Parichita||Scharf, Eugene L||Paolini, Michael A||Graff-Radford, Jonathan||Alden, Eva C||Machulda, Mary M||Jones, David T||Fields, Julie A||Murray, Melissa E||Graff-Radford, Neill R||Constantopoulos, Eleni||Reichard, Ross R||Knopman, David S||Duffy, Joseph R||Dickson, Dennis W||Parisi, Joseph E||Josephs, Keith A||Petersen, Ronald C||Boeve, Bradley F