| Popis: |
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 01/01/2011 and 31/10/2021. Fifty patients with N4GT (30 PSP, 20 CBD) were prospectively recruited and followed at Mayo Clinic, Minnesota. MR 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-PET 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, cognitive impairment followed by pyramidal tract signs and 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 before death. Most GGT patients had asymmetric frontotemporal atrophy with matching hypometabolism. GGT patients had more gray matter atrophy in temporal lobes, normal MR-parkinsonism index, and larger midbrain volumes. CONCLUSIONS: Female sex, lower motor neuron involvement in the context of a frontotemporal dementia syndrome and asymmetric brain atrophy with preserved midbrain might be suggestive of underlying GGT. |
