title: FTLD-TDP and progressive supranuclear palsy in comorbidity-a report of two cases with different clinical presentations.
abstract: Frontotemporal lobar degeneration with transactive response DNA-binding protein 43 (FTLD-TDP) and progressive supranuclear palsy (PSP) are distinct neurodegenerations with different clinical presentations. We report two cases with FTLD-TDP and PSP in comorbidity: a patient with amnestic dementia developing frontal lobe dementia, Parkinsonism and supranuclear gaze palsy and a patient with cerebellar ataxia and nystagmus developing akinesia, rigidity, and subcortical dementia. Neuropathological examination revealed neuronal and glial tau pathology together with ubiquitin, and phospho-TDP-43-immunoreactivities in the hippocampus, striatum, mesencephalon, and frontal and temporal cortices. Clinical and neuropathological correlations in atypical neurodegenerations are crucial to describe new entities of overlapping syndromes.
authors.affiliation: a Department of Neurology , Thomayer Hospital , Prague , Czech Republic.
authors.forename: Kateřina
authors.initials: K
authors.lastname: Storey
authors.affiliation: a Department of Neurology , Thomayer Hospital , Prague , Czech Republic.
authors.forename: Silvie
authors.initials: S
authors.lastname: Johanidesová
authors.affiliation: b Department of Pathology and Molecular Medicine , Thomayer Hospital , Prague , Czech Republic.|c Department of Pathology, First Faculty of Medicine , Charles University, and General University Hospital , Prague , Czech Republic.|d Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine , Charles University, and General University Hospital , Prague , Czech Republic.
authors.forename: Radoslav
authors.initials: R
authors.lastname: Matěj
authors.affiliation: e Department of Radiology , Na Homolce Hospital , Prague , Czech Republic.|f Department of Neurology, Third Faculty of Medicine , Charles University , Prague , Czech Republic.
authors.forename: Jiří
authors.initials: J
authors.lastname: Keller
authors.affiliation: b Department of Pathology and Molecular Medicine , Thomayer Hospital , Prague , Czech Republic.|c Department of Pathology, First Faculty of Medicine , Charles University, and General University Hospital , Prague , Czech Republic.
authors.forename: Zdeněk
authors.initials: Z
authors.lastname: Rohan
authors.affiliation: a Department of Neurology , Thomayer Hospital , Prague , Czech Republic.|d Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine , Charles University, and General University Hospital , Prague , Czech Republic.
authors.forename: Robert
authors.initials: R
authors.lastname: Rusina
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000378:metabolism*; D057180:Frontotemporal Dementia / Q000150:complications* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D013494:Supranuclear Palsy, Progressive / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D025801:Ubiquitin / Q000378:metabolism
chemical_list: D004268:DNA-Binding Proteins; C000598624:TARDBP protein, human; D025801:Ubiquitin
keywords: Progressive supranuclear palsy; TDP-43 inclusions; dementia; frontotemporal lobar degeneration; tauopathy