TY - JOUR
T1 - Pick’s disease
T2 - clinicopathologic characterization of 21 cases
AU - Choudhury, Parichita
AU - Scharf, Eugene L.
AU - Paolini, Michael A.
AU - Graff-Radford, Jonathan
AU - Alden, Eva C.
AU - Machulda, Mary M.
AU - Jones, David T.
AU - Fields, Julie A.
AU - Murray, Melissa E.
AU - Graff-Radford, Neill R.
AU - Constantopoulos, Eleni
AU - Reichard, Ross R.
AU - Knopman, David S.
AU - Duffy, Joseph R.
AU - Dickson, Dennis W.
AU - Parisi, Joseph E.
AU - Josephs, Keith A.
AU - Petersen, Ronald C.
AU - Boeve, Bradley F.
N1 - Publisher Copyright:
© 2020, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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.
AB - 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.
KW - Frontotemporal dementia
KW - Pick’s disease
KW - Primary progressive aphasia
UR - http://www.scopus.com/inward/record.url?scp=85084983161&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85084983161&partnerID=8YFLogxK
U2 - 10.1007/s00415-020-09927-9
DO - 10.1007/s00415-020-09927-9
M3 - Article
C2 - 32440921
AN - SCOPUS:85084983161
SN - 0340-5354
VL - 267
SP - 2697
EP - 2704
JO - Journal of Neurology
JF - Journal of Neurology
IS - 9
ER -