Is knowledge of famous people compromised in mild cognitive impairment?

Objective: : This study addressed the issue of whether person naming deficits in mild cognitive impairment (MCI) occurred with deficits in person semantic knowledge and whether person knowledge was more impaired than general semantics.

Background: : Recent definitions of MCI are beginning to encompass cognitive impairments outside the domain of episodic memory. Increasing evidence suggests that semantic memory may also be compromised in this patient group, including tasks of person naming and identification.

Relapsing encephalopathy in a patient with α-methylacyl-CoA racemase deficiency.

α-Methylacyl-CoA racemase (AMACR) deficiency is a rare disorder of fatty acid metabolism which has recently been described in three adult cases. We have identified a further patient with clinical features of a relapsing encephalopathy, seizures and cognitive decline over a 40 year period. Biochemical studies revealed grossly elevated plasma pristanic acid levels, and a deficiency of AMACR in skin fibroblasts.

Naming of objects, faces and buildings in mild cognitive impairment.

Accruing evidence suggests that the cognitive deficits in very early Alzheimer's Disease (AD) are not confined to episodic memory, with a number of studies documenting semantic memory deficits, especially for knowledge of people. To investigate whether this difficulty in naming famous people extends to other proper names based information, three naming tasks - the Graded Naming Test (GNT), which uses objects and animals, the Graded Faces Test (GFT) and the newly designed Graded Buildings Test (GBT) - were administered to 69 participants (32 patients in the early prodromal stage of AD, so-called Mild Cognitive Impairment (MCI), and 37 normal control participants). Patients were found to be impaired on all three tests compared to controls, although naming of objects was significantly better than naming of faces and buildings.

The hippocampal region is involved in successful recognition of both remote and recent famous faces.

There is currently a debate regarding the precise role of medial temporal regions in memory, in particular regarding the time scale of their involvement in conscious recollection of information stored in long-term memory. Using event-related fMRI, we have attempted to contribute to this debate by identifying brain regions associated with the successful recognition of famous faces from two different periods: "Old" faces of people who became famous in the 1960s-1970s and "Recent" faces of people who became famous in the 1990s. We demonstrate that the hippocampus is involved in the successful recognition of famous faces from both periods and does not appear to distinguish between these two periods.

Dissociating person-specific from general semantic knowledge: roles of the left and right temporal lobes.

The cognitive architecture and neural underpinnings of different semantic domains remains highly controversial. We report two patients with focal temporal lobe atrophy who presented with contrasting and theoretically informative dissociations of person-specific versus general semantic knowledge. Subject J.

Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications.

Objective: To characterize presenting symptomatology in patients with semantic dementia (SD) and to investigate whether left and right temporal variants of the disease have distinct behavioral and cognitive profiles.

Methods: Retrospective examination of case notes was performed in 47 consecutive referrals of patients with a clinical diagnosis of SD. Patients (and informants) were interviewed and underwent neuropsychological testing within 6 months of assessment.

Mild cognitive impairment: a clinically useful but currently ill-defined concept?

The term mild cognitive impairment (MCI) has gained wide currency among clinicians, and particularly dementia researchers, to denote patients with memory deficits who do not yet fulfil the criteria for dementia, but are at high risk of conversion. MCI is therefore regarded as the prodromic or pre-dementia stage of Alzheimer's disease. The accurate categorization of these subjects has far-reaching implications, both for research in this field and for those individuals who fall within this diagnostic group.

Is knowledge of famous people disproportionately impaired in patients with early and questionable Alzheimer's disease?

Twenty-two patients with early dementia of Alzheimer's type (DAT) and 31 controls were administered tests of person-specific semantics (Experiment 1). DAT patients were impaired on all test components. In Experiment 2, 31 DAT patients, 28 questionable DAT (QDAT) patients, and 42 controls were administered the Graded Naming Test (GNT) and the newly designed Graded Faces Test (GFT), matched for difficulty with the GNT.