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BACKGROUND Neurodegeneration associated with brain iron accumulation (NBIA) comprises a heterogeneous group of disorders in which disruption of cellular mechanisms leads to accumulation of iron in the basal ganglia. This group includes patients with recently discovered mutations in the PLA2G6 gene encoding a calcium-independent phospholipase A2 enzyme that(More)
This study investigated the influence of age at onset on cognitive performance, neuropathological and neurochemical features in autopsy-confirmed sporadic Lewy body variant (LBV) and in Alzheimer's disease (AD). We compared 28 early-onset (< or = 70 years) LBV subjects with 28 matched late-onset (> 70 years) subjects. Similarly, we examined the same(More)
BACKGROUND Olfactory abnormalities are reported in Alzheimer's disease and Parkinson's disease. Anosmia appears to be common in dementia with Lewy bodies but not in pure Alzheimer's disease. OBJECTIVE To determine whether anosmia improves discrimination between the Lewy body variant (LBV) of Alzheimer's disease and "pure" Alzheimer's disease. METHODS(More)
Unit activity of the cingulate cortex and the anteroventral (AV) nucleus of the thalamus in rabbits was recorded during reversal training following differential conditioning of a locomotory (wheel rotation) avoidance response. The positive and negative conditional stimuli (CS+ and CS-) were pure tones (1 kHz and 8 kHz), and the unconditional stimulus was(More)
Bilateral electrolytic lesions of the anteroventral (AV) nucleus of the thalamus given after training impaired retention performance (extinction and reacquisition) of rabbits in a differential avoidance conditioning task. In addition, the lesions abolished the excitatory, discriminative multiple-unit discharges that had developed in the cingulate and(More)
Multiple-unit activity of the cingulate cortex and the anteroventral (AV) nucleus of the thalamus was recorded during discriminative conditioning of an avoidance response (locomotion) in rabbits. The results indicated a greater unit response in cingulate cortex to the positive conditional stimulus (CS+; a tone paired with a footshock unconditional stimulus(More)
Neurons in deep laminae of the rabbit cingulate cortex develop discriminative activity at an early stage of behavioral discrimination learning, whereas neurons in the anteroventral nucleus of thalamus and neurons in the superficial cortical laminae develop such activity in a late stage of behavioral learning. It is hypothesized that early-forming(More)
In the current study, we explored how a person's physiological arousal relates to their performance in a challenging math situation as a function of individual differences in working memory (WM) capacity and math-anxiety. Participants completed demanding math problems before and after which salivary cortisol, an index of arousal, was measured. The(More)
OBJECTIVES To compare the diagnostic performance of clinical assessment against magnetic resonance imaging (MRI) diagnosed hip arthritis in a juvenile idiopathic arthritis (JIA) population. To determine the clinical and serological predictors of MRI diagnosed hip arthritis. METHODS A total of 34 JIA patients with established disease (mean disease duration(More)
Multiple-unit activity of the cingulate cortex and the anteroventral (AV) nucleus of the thalamus in rabbits was recorded during reversal training, following differential conditioning of a locomotory (wheel rotation) avoidance response. The CS+ and CS- were pure tones (1 or 8 kHz) and the UCS was a footshock delivered through the grid floor of the wheel.(More)