Primary progressive aphasia (PPA) corresponds to the gradual degeneration of language which can occur as nonfluent/agrammatic PPA, semantic variant PPA or logopenic variant PPA. We describe the clinical evolution of a patient with PPA presenting jargon aphasia as a late feature. At the onset of the disease (ten years ago) the patient showed anomia and executive deficits, followed later on by ph...

OBJECTIVE To describe the clinical, neuropsychologic, and radiologic features of a family with a C31LfsX35 mutation in the progranulin gene CCDS11483.1). DESIGN Case series. PATIENTS A large British kindred (DRC255) with a PGRN mutation was assessed. Affected individuals presented with a mean age of 57.8 years (range, 54-67 years) and a mean disease duration of 6.1 years (range, 2-11 years)...

Background: Hippocampal atrophy on MRI is an early characteristic of Alzheimer’s disease (AD). However, hippocampal atrophy may also occur in other dementias, like frontotemporal lobar degeneration (FTLD). Aim: To investigate hippocampal atrophy on MRI in FTLD and its three clinical subtypes, in comparison to AD, using volumetry and a visual rating scale. Methods: Forty-two patients with FTLD (...

A 56-year-old man was diagnosed with follicular nonHodgkin’s lymphoma. Four months prior to admission, he presented a right headache attributed to rhinosinusitis. Biopsy showed mucormycosis. There was no affectation intracranial in the brain magnetic resonance imaging (MRI), but the patient became blind due to involvement of both central retinal arteries. He underwent debridement of the paranas...

Most of us would like to believe that the different patterns of language breakdown observed in aphasic patients reflect the way that the human mind and brain are organized for language. However, because so much modern research on aphasia has been carried out in English, it is difficult to separate universal mechanisms from language-specific content. Crosslinguistic com-parisons permit us to dis...

Case Report A 56-year-old man was seen for speech difficulties. He had no past medical history or known familial disease. He studied Portuguese for 4 years and then went to live in France, where he learned French for 3 years. Afterward, he started working as a locksmith. At age 21 he spent 2 years in Saudi Arabia and Iraq, where he spoke French, and then came back to France until turning 42. He...

Primary Progressive Aphasia (PPA) is a behaviorally focal dementia syndrome with deterioration of language functions but relative preservation of other cognitive domains for at least the first two years of disease. In this study, PPA patients with impaired word finding but intact comprehension of conversational speech and their matched control subjects were examined using voxel-based morphometr...

Fluent primary progressive aphasia (fPPA) is very often confused with semantic dementia (SD), due to the ambiguity of the latter term. We present a case of fPPA with neuropsychological and neuroimaing data, not fulfilling the diagnostic criteria for SD. The language disturbance profile is presented, resembling transcortical sensory aphasia. The results of both standardized and experimental test...

The neuropsychological features of the primary progressive aphasia (PPA) syndromes continue to be defined. Here we describe a detailed neuropsychological case study of a patient with a mutation in the progranulin (GRN) gene who presented with progressive word-finding difficulty. Key neuropsychological features in this case included gravely impoverished propositional speech with anomia and prolo...

The nematode Pristionchus pacificus shows two forms of phenotypic plasticity: dauer formation and dimorphism of mouth form morphologies. It can therefore serve as a model for studying the evolutionary mechanisms that underlie phenotypic plasticity. Formation of dauer larvae is observed in many other species and constitutes one of the most crucial survival strategies in nematodes, whereas the mo...