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Biomedical subjects

Nadine Le Forestier

Publications and source records attributed to Nadine Le Forestier.

4 recordsLinked to original sources

Neuropsychological changes in patients with primary lateral sclerosis.

Primary lateral sclerosis (PLS) has been defined as rare, and the neuropsychological changes remain poorly defined. We studied 20 patients with a diagnosis of PLS. We carried out an extensive psychometric testing including a general assessment (memory, language, attention, visual-constructional ability and praxis) and a more specific assessment of prefrontal and premotor cortex functions in order to characterize the neuropsychological profile of the patients compared to matched controls, and explore executive functions and premotor cortex functions. None of the PLS patients was demented but they all presented memory deficits reflecting an executive dysfunction. All patients but three had signs of premotor and/or prefrontal cortex deficits. The cognitive impairment in PLS, specifically related to a frontal lobe dysfunction, seems qualitatively similar to ALS. Our results suggest a patchy distribution of cortical involvement in PLS but it remains difficult to draw any definite conclusion as to the spatio-temporal progression of the disease into the different regions of the frontal lobe.

Adult↗

[Peripheral neuropathy in the elderly].

Peripheral neurologic deficits are commonly found during physical examination of older patients. Losses of vibratory sensation in the lower extremities and ankle reflexes are so common that they are often listed in geriatric textbooks as normal physical findings in very old people. In this population, the detection of peripheral neuropathy, which may lead to a serious disability, is an important contribution to the health care but there is very little information in the literature about its actual prevalence and etiology. The epidemiological, clinical, morphological, electrophysiological data on the peripheral nervous system abnormalities in elderly are reviewed. A strategy of investigations is proposed to improve the identification of the etiology of their peripheral neuropathy.

Aged↗

Glutamate levels in cerebrospinal fluid in amyotrophic lateral sclerosis: a reappraisal using a new HPLC method with coulometric detection in a large cohort of patients.

Glutamate is involved in the degeneration of motor neurons in amyotrophic lateral sclerosis (ALS). However, the aetiology of ALS appears heterogeneous, leading to the possibility that patient subgroups with different pathophysiology may exist. The concentration of glutamate in cerebrospinal fluid (CSF) is measured using a new HPLC method with coulometric detection in a large cohort of ALS patients and controls: 377 ALS patients, 88 neurological patients and 18 normal controls. In ALS patients, and only in these subjects, the existence of two groups was observed, one with normal glutamate concentrations and one (40.8% of ALS patients) with high glutamate concentrations. High glutamate concentrations were correlated with a spinal onset of the disease, more impaired limb function and a higher rate of muscle deterioration. These results suggest that elevations of CSF glutamate concentrations could reflect the intensity of cell insult in the spinal cord. It remains to be determined if the group of patients with high CSF glutamate concentrations represents a specific subgroup of patients in terms of mechanism of disease, or only in terms of the spatial extent of motor neuron insult.

Age Factors↗

Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy.

Electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991. Only 60% of CIDP patients fulfilled these criteria, which therefore appear poorly sensitive. We therefore sought to revise the electrophysiological criteria. We selected 40 CIDP patients and compared them with 35 patients with axonal polyneuropathy, 116 patients with Charcot-Marie-Tooth type 1A (CMT1A) disease, and 66 patients with immunoglobulin M (IgM) monoclonal gammopathy. The proposed electrophysiological criteria identified 90% of the CIDP patients, although 3% of patients with axonal polyneuropathy were falsely identified. For the CIDP patients, sensitivity and specificity were 90% and 97%, respectively. Of the patients with IgM monoclonal gammaglobulin of undetermined significance (MGUS) and CMT1A, 100% fulfilled these new criteria, whereas 90% and 97%, respectively, fulfilled the AAN criteria. These results suggest that the AAN criteria are more appropriate for IgM MGUS and CMT1A patients than for CIDP patients. We therefore propose new electrophysiological criteria for CIDP that appear to have better sensitivity.

Adult↗