Pick's disease: clinicopathologic characterization of 21 cases. Academic Article uri icon

abstract

  • 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.

published proceedings

  • J Neurol

altmetric score

  • 19.9

author list (cited authors)

  • Choudhury, P., Scharf, E. L., Paolini, M. A., Graff-Radford, J., Alden, E. C., Machulda, M. M., ... Boeve, B. F

citation count

  • 5

complete list of authors

  • 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

publication date

  • September 2020