Objective: We report two cases of semantic dementia (SD), the temporal variant of frontotemporal dementia (FTD), with right and left temporal predominance of atrophy. SD is a disorder of semantics, with well preserved phonological and syntactic aspects of language, and non-semantic skills, and behavioral changes qualitatively distinguishable from those of the frontal variant of FTD (fvFTD).
Materials, Methods, and Results: M.V., a right-handed 79-year-old-woman, was a housewife, with 5 years of schooling, a 8-year history of impairment of semantics, and normal neurological examination and EEG. MRI scans and HMPAOtc99 SPECT r(CBF) showed bilateral temporal atrophy, more marked on the left. Neuropsychological examination revealed a total loss of verbal fluency with grossly impaired performance in all kind of word fluency test, and a better performance in initial-letter word generation than category or word association generation. Language comprehension and sentence generation were impaired with no paraphasic errors, while visuo-spatial tasks were within normal limits. N.V., a right-handed 79-year-old-woman, was a housewife, with 5 years of schooling, 10 year-history of progressive deterioration of language, EEG normal. CT scans and SPECT r(CBF) showed bilateral fronto-temporal atrophy, with a right predominance. The neuropsychological examination revealed impairment of verbal fluency and sentence generation. No disorder was present in language. She performed surprisingly poorly on formal test of copying. Discrimination between abstract figures was normal. Both patients performed normally on word span, episodic memory, selective attention, and problem-solving. We rated psychiatric symptoms with Neuropsychiatric Inventory, and M.V. exhibited an apathetic pattern of behavior with depression, verbal and motor perseveration of the “recurrent” type. She showed exaggerated prodigality. N.V. showed a stereotypic pattern of behavior with sexual disinhibition and altered eating habits, with bulimia and a “sweet tooth” typical of SD. She was socially inept, agitated, and profoundly irritable.
Conclusion: In conclusion, a predominance of left temporal atrophy involves language disorder, which includes phonological skills, apathy and perseveration, while right temporal atrophy predominance involves impaired constructional performance with a stereotypic pattern of behavior. The behavioral changes of these SD patients seem to be more linked to the pattern of perfusion than to hemispheric predominance, with neuropsychological features substantially fulfil the quality and quantity of atrophy.
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