title: Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy.
abstract: A 57-year-old male with no family history was diagnosed with semantic dementia. He also showed some unusual cognitive features such as episodic memory and executive dysfunctions, spatial disorientation, and dyscalculia. Rapidly progressive cognitive and physical decline occurred. About 1.5 years later, he developed clinical features of a corticobasal syndrome. He died at the age of 60. Brain autopsy revealed numerous 4R-tau-positive lesions in the frontal, parietal and temporal lobes, basal ganglia, and brainstem. Neuronal loss was severe in the temporal cortex. Such association of semantic dementia with tauopathy and corticobasal syndrome is highly unusual. These findings are discussed in the light of current knowledge about frontotemporal lobar degeneration.
authors.affiliation: a Service Universitaire de Psychiatrie de l'âge avancé, Département de Psychiatrie , Centre Hospitalier Universitaire Vaudois , Lausanne , Switzerland.
authors.forename: Marie-Therese
authors.initials: MT
authors.lastname: Clerc
authors.forename: Manuel
authors.initials: M
authors.lastname: Deprez
authors.forename: Genevieve
authors.initials: G
authors.lastname: Leuba
authors.forename: Benoît
authors.initials: B
authors.lastname: Lhermitte
authors.forename: Ursula
authors.initials: U
authors.lastname: Lopez
authors.forename: Armin
authors.initials: A
authors.lastname: von Gunten
mesh_terms: D001921:Brain / Q000473:pathology; D057180:Frontotemporal Dementia / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D024801:Tauopathies / Q000150:complications / Q000473:pathology
keywords: FTLD; corticobasal syndrome; semantic dementia; tauopathy