Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy

作者:Clerc Marie Therese*; Deprez Manuel; Leuba Genevieve; Lhermitte Benoit; Lopez Ursula; von Gunten Armin
来源:Neurocase, 2015, 21(1): 1-15.
DOI:10.1080/13554794.2013.841953

摘要

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.

  • 出版日期2015-1-2