The right temporal lobe variant of frontotemporal dementia : Cognitive and neuroanatomical profile of three patients

• Published in: Journal of neurology Vol. 253; no. 11; pp. 1447 - 1458
• Main Authors: JOUBERT, Sven, FELICIAN, Olivier, BARBEAU, Emmanuel, RANJEVA, Jean-Philippe, CHRISTOPHE, Marion, DIDIC, Mira, PONCET, Michel, CECCALDI, Mathieu
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01-11-2006; Springer Nature B.V; Springer Verlag
• Subjects: Aged; Biological and medical sciences; Cognition Disorders - etiology; Cognition Disorders - pathology; Cognition Disorders - physiopathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Dementia - pathology; Dementia - physiopathology; Female; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Life Sciences; Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Nervous system (semeiology, syndromes); Neurology; Neurons and Cognition; Neuropsychological Tests; Temporal Lobe - pathology; Temporal Lobe - physiopathology; Human; Temporal lobe; Nervous system diseases; Central nervous system disease; Degenerative disease; Frontotemporal dementia; Cerebral disorder
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-006-0232-x

The right temporal variant of frontotemporal lobar degeneration (Rtv-FTLD) is a focal degenerative condition affecting predominantly the right temporal lobe. The aim of this study was to further characterize the profile of cognitive impairment and the neuroanatomical basis of Rtv-FTLD patients without behavioural disturbances. A group of three patients with this syndrome had a detailed neuropsychological assessment, along with Voxel-Based Morphometry (VBM) of their brain to determine location of cortical atrophy. VBM analyses showed a pattern of atrophy that was predominant in the right hemisphere and concerned primarily the right anterior temporal lobe region. Patients carried out a test of famous people in which their ability to recognize, name and provide semantic information about famous persons from their faces, their voices and their names was investigated. They all showed a severe defect in recognizing, naming and identifying famous people irrespective of modality. Therefore, their inability to recognize famous people resulted from a multimodal defect (semantic). These results highlight the semantic nature of the defect, and suggest that the anterior right temporal lobe may have a prominent role in processing person-based semantic knowledge. This study helps in further understanding the neuropsychological profile of patients with Rtv-FTLD.