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Metamemory is a multifaceted concept, which deals with an individual's knowledge and control of his or her own memory system. The ability to monitor memory performance accurately was examined in 16 patients with Alzheimer's disease (AD), 6 patients with frontotemporal lobe dementia (FTD) and 16 elderly subjects. Participants made global memory predictions(More)
Dopa-responsive dystonia is a childhood-onset dystonic disorder, characterized by a dramatic response to low dose of L-Dopa. Dopa-responsive dystonia is mostly caused by autosomal dominant mutations in the GCH1 gene (GTP cyclohydrolase1) and more rarely by autosomal recessive mutations in the TH (tyrosine hydroxylase) or SPR (sepiapterin reductase) genes.(More)
BACKGROUND Hereditary spastic paraplegias are disorders that are very heterogeneous, both clinically and genetically. The atlastin1 gene has recently been implicated in SPG3A, a form of autosomal dominant pure spastic paraplegia. Atlastin1 mutations have been identified in 8 families so far. OBJECTIVES To determine the relative frequency, phenotype, and(More)
OBJECTIVES To propose a neuropsychological study of the various aspects of self-consciousness (SC) in Alzheimer's disease. METHODS Forty-five patients with probable mild or moderate AD were included in the study. Severity of their dementia was assessed by the Mini Mental State (MMS). Fourteen questions were prepared to evaluate SC. RESULTS No(More)
OBJECTIVE To study event-related potentials and particularly the P300 wave in a group of patients with amyotrophic lateral sclerosis (ALS), as changes in the P300 wave have been observed in many cases of brain damage-inducing cognitive impairment. DESIGN Case series. SETTING University hospital. PATIENTS Twenty patients with ALS were compared with 20(More)
In Parkinson's disease (PD), apathy (or loss of motivation) is frequent. Nevertheless, the contribution of attentional disorders to its genesis is still not clearly known. We want to determine the relation existing between apathy and attentional disorders by using P300a (or novelty P3) as a marker of the attentional process. The study included 25 patients(More)
In Alzheimer's disease (AD), brain lesions are marked by severe neuronal loss and retinal degeneration was previously mentioned in affected patients. Mild cognitive impairment (MCI) is a clinical syndrome that could be an early phase of AD. In this study, using optical coherence tomography (OCT), the retinal nerve fiber layer (RNFL) thickness was assessed(More)
Long latency event-related auditory evoked potentials, particularly the P300 wave, constitute an objective electrophysiological index of cognitive function. For this reason, these potentials have been studied in a series of 101 patients with multiple sclerosis (MS), classified according to McAlpine's criteria into definite, probable and possible cases. The(More)
BACKGROUND Recent clinical and research reports suggest that the cerebellum may contribute to the modulation of higher cognitive functions. MATERIAL AND METHODS We included 15 consecutive patients with isolated cerebellar infarcts (4 superior cerebellar artery territory infarcts, 1 anterior inferior cerebellar artery territory infarct and 10 posterior(More)
In Alzheimer's disease, neuropathological hallmarks include the accumulation of beta-amyloid peptides (Abeta) in senile plaques, phosphorylated tau in neurofibrillary tangles and neuronal death. Abeta is the major aetiological agent according to the amyloid cascade hypothesis. Translational control includes phosphorylation of the kinases mammalian target of(More)