title: Right temporal lobe variant of frontotemporal dementia.
abstract: We present two women with the right temporal lobe variant (RTLV) of frontotemporal dementia (FTD) and analyse the clinical features that are determined by the anatomical distribution of atrophy. Each of our patients displayed different clinical and radiological profiles which were in line with findings reported by other authors. One of two patients carries a novel mutation in the granulin gene. FTD is heterogeneous with regard to clinical manifestation, genetics, distribution of cortical atrophy and underlying disease. Its clinical manifestations are related to the distribution of the cortical atrophy. The RTLV of FTD is an uncommon entity. There is no consensus about its name despite the fact that its clinical and radiological features are well-defined and distinguish it from other types of FTD including semantic dementia.
authors.affiliation: Unit for Behavioral Neurology and Dementia, Hospital San Vicente, 137 San Vicente del Raspeig, Alicante 03690, Spain. Electronic address: gloriagcaballero@yahoo.es.
authors.forename: G
authors.initials: G
authors.lastname: González-Caballero
authors.affiliation: Unit for Behavioral Neurology and Dementia, Hospital San Vicente, 137 San Vicente del Raspeig, Alicante 03690, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Abellán-Miralles
authors.affiliation: Unit for Behavioral Neurology and Dementia, Hospital San Vicente, 137 San Vicente del Raspeig, Alicante 03690, Spain.
authors.forename: M J
authors.initials: MJ
authors.lastname: Sáenz-Sanjuan
mesh_terms: D000368:Aged; D018888:Aphasia, Primary Progressive / Q000209:etiology / Q000235:genetics / Q000473:pathology; D001284:Atrophy; D005260:Female; D057180:Frontotemporal Dementia / Q000235:genetics / Q000473:pathology* / Q000523:psychology; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D009460:Neurologic Examination; D000077153:Progranulins; D013702:Temporal Lobe / Q000473:pathology*
chemical_list: C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins
keywords: Frontotemporal dementia; Novel GRN mutation; Right temporal lobe variant; Semantic variant of primary progressive aphasia; Temporal lobe atrophy