Abstract
Semantic dementia (SD) is a clinical variant of frontotemporal lobar degeneration (FTLD) characterized by progressive deterioration of semantic memory with relative sparing of other cognitive functions. It is associated with mainly left anterior temporal atrophy, and is also referred to as “left-temporal lobe variant” of FTLD. Recently, patients with mainly right-sided atrophy, or “right-temporal lobe variant”(RTLV), have been described. While some authors have reported that the initial and most significant deficit in these right-sided cases is a difficulty in recognizing famous people, others have observed that major behavioral abnormalities are the presenting symptoms. Here we report a detailed neuropsychological, language, behavioral and neuroimaging assessment of JT, a case of right temporal lobe variant of FTLD. JT showed early and prominent behavioral changes accompanied by a severe impairment in recognizing foods by their look, flavor or name. Later she also developed a difficulty in recognizing familiar people and objects. Standardized caregiver questionnaires of JT's pre- and post-morbid personality and interpersonal functioning showed that she went from being a flexible, dominant, extraverted, person to showing rigid, submissive and introverted behaviors. Her levels of neuroticism significantly increased, while her scores on agreeableness and cognitive and emotional empathy dropped. Voxel-based morphometry (VBM) showed most significant atrophy in the right amygdala/anterior hippocampal complex and collateral sulcus, extending to the right insula. We discuss the atypical cognitive and behavioral features of this case of RTLV of FTLD and stress the importance of behavioral changes and atypical semantic deficits for early diagnosis.
Highlights
Semantic dementia (SD) is a clinical syndrome characterized by a progressive loss of conceptual knowledge
Neuropsychological and Functional Evaluation: JT’s performance in all tests of visuospatial functioning, was average with the exception of the VOSP number location. Her poor performance on this test appeared to be due to her inadequate comprehension of the rules of the test. She performed in the normal range on a facial discrimination task (Benton Facial Recognition Test; see below)
Behavioral and anatomical features of JT, a case of right temporal lobe variant (RTLV) of frontotemporal lobar degeneration (FTLD)
Summary
Semantic dementia (SD) is a clinical syndrome characterized by a progressive loss of conceptual knowledge. Patients typically present in the presenium with difficulty in recalling and understanding words, and later show impairment in recognizing objects (Lambon Ralph et al, 1998, 1999; Hodges et al, 1992). Surface dyslexia is part of the SD clinical picture, as patients have greater difficulty reading words with irregular pronunciations (such as “yacht”) compared to regularly spelled words (such as “context”). Neuroimaging findings in SD indicate that the anterior temporal lobe is the site of most prominent atrophy and/or hypometabolism, providing a powerful model for studying this brain region rarely hit by other neurological diseases (Mummery et al, 2000; Galton et al, 2001; Chan et al, 2001; Rosen et al, 2002a). Voxel-based Morphometry studies (VBM) and region of interest analysis on MRI scans have shown that the hippocampal/amygdala
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