INTRODUCTION The motor features of corticobasal syndrome (CBS) are well recognized but the fact that many, if not all, affected patients develop cognitive impairment is still underrecognized. The dementia of CBS overlaps most with a language variant of frontotemporal dementia: progressive nonfluent aphasia (PNFA). The aim of this study was to determine the usefulness of Addenbrooke's Cognitive ...

In the two nematode species Caenorhabditis elegans and Pristionchus pacificus the vulva equivalence group in the central body region is specified by the Hox gene lin-39. C. elegans lin-39 mutants are vulvaless and the vulval precursor cells fuse with the surrounding hypodermis, whereas in P. pacificus lin-39 mutants the vulval precursor cells die by apoptosis. Mechanistically, LIN-39 might inhi...

Anomia, or impaired word retrieval, is the most widespread symptom of aphasia, an acquired language impairment secondary to brain damage. In the last decades, functional neuroimaging techniques have enabled studying the neural basis underlying anomia and its recovery. The present study aimed to explore maladaptive plasticity in persistent verb anomia, in three male participants with chronic non...

BACKGROUND The language profile of behavioral variant frontotemporal dementia (bvFTD) remains to be fully defined. OBJECTIVE We aimed to quantify the extent of language deficits in this patient group. METHODS We assessed a cohort of patients with bvFTD (n = 24) in relation to patients with semantic variant primary progressive aphasia (svPPA; n = 14), nonfluent variant primary progressive ap...

BACKGROUND We investigated the demographic, clinical, and neuropsychological characteristics of frontotemporal dementia (FTD) from the Clinical Research Center for Dementia of South Korea (CREDOS)-FTD registry. METHODS A total of 200 consecutive patients with FTD recruited from 16 neurological clinics in Korea were evaluated by cognitive and functional assessments, a screening test for aphasi...

Background Interoception (the perception of internal bodily sensations) is strongly linked to emotional experience and sensitivity to the emotions of others in healthy subjects. Interoceptive impairment may contribute to the profound socioemotional symptoms that characterize frontotemporal dementia (FTD) syndromes, but remains poorly defined. Methods Patients representing all major FTD syndro...

OBJECTIVE To assess discourse in patients with frontotemporal dementia (FTD). METHODS The authors asked patients with progressive nonfluent aphasia (PNFA), patients with semantic dementia (SemD), and nonaphasic patients with a disorder of social comportment and executive functioning (SOC/EXEC) to narrate the story of a wordless children's picture book. RESULTS The authors found significant ...

Impaired semantic memory is ubiquitous in frontotemporal dementia (FTD), including patients with semantic dementia (SD), progressive nonfluent aphasia (PNFA) and nonaphasic FTD patients with a deficit in executive and social functioning (EXEC/SOC). One hypothesis attributes this to the degradation of specific categories of knowledge in semantic memory. This study explores the alternate hypothes...

OBJECTIVE To learn if oral steroid treatment can alter the signs of primary progressive aphasia (PPA). Many patients with PPA have had a vasectomy and there is a possible link between vasectomy and autoimmune diseases. If PPA is, at least in part, an autoimmune disease, patients might improve with immunosuppressant treatment. DESIGN Case report. SETTING Cognitive and memory clinic. PATIEN...

Corticobasal degeneration (CBD) is a distinct neurodegenerative disorder characterized by widespread neuronal and glial accumulation of abnormally phosphorylated tau protein. Patients with CBD often present with corticobasal syndrome (CBS) showing impairment of the motor system, cognition, or both. Several studies demonstrate that they may also present with progressive supranuclear palsy syndro...