The many faces of globular glial tauopathy: A clinical and imaging study

作者：Marina Buciuc, Shunsuke Koga, Nha Trang Thu Pham, Joseph R. Duffy, David S. Knopman, Farwa Ali, Bradley F. Boeve, Jon Graff‐Radford, Hugo Botha, Val J. Lowe, Aivi T. Nguyen, Ross R. Reichard, Dennis W. Dickson, Ronald C. Petersen, Jennifer L. Whitwell, Keith A. Josephs · 发表于：European Journal of Neurology · 年份：2022 · DOI：10.1111/ene.15603 · 被引用次数：25 · 研究领域：Parkinson's Disease Mechanisms and Treatments、Alzheimer's disease research and treatments、Neurological and metabolic disorders

Abstract Background Globular glial tauopathy (GGT) has been associated with frontotemporal dementia syndromes; little is known about the clinical and imaging characteristics of GGT and how they differ from other non‐globular glial 4‐repeat tauopathies (N4GT) such as progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD). Methods For this case–control study the Mayo Clinic brain banks were queried for all cases with an autopsy‐confirmed diagnosis of GGT between 1 January 2011 and 31 October 2021. Fifty patients with N4GT (30 PSP, 20 CBD) were prospectively recruited and followed by the Neurodegenerative Research Group at Mayo Clinic, Minnesota. Magnetic resonance imaging was used to characterize patterns of gray/white matter atrophy, MR‐parkinsonism index, midbrain volume, and white matter hyperintensities. 18 F‐Fluorodeoxyglucose‐, 11 C Pittsburg compound‐, and 18 F‐flortaucipir‐positron emission tomography scans were reviewed. Results Twelve patients with GGT were identified: 83% were women compared to 42% in NG4T ( p = 0.02) with median age at death 76.5 years (range: 55–87). The most frequent clinical features were eye movement abnormalities, parkinsonism, behavioral changes followed by pyramidal tract signs and motor speech abnormalities. Lower motor neuron involvement was present in 17% and distinguished GGT from NG4T ( p = 0.035). Primary progressive apraxia of speech was the most frequent initial diagnosis (25%); 50% had a Parkinson‐plus syndrome befor...