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Although the striatum has one of the highest densities of metabotropic glutamate receptor (mGluR) binding sites in the brain, little is known about their physiological role. In this study we characterized the contribution of mGluRs located in the ventral part of the striatum (the nucleus accumbens) to the control of extrapyramidal motor function. Activation(More)
OBJECTIVE To study the diagnostic value of transcranial magnetic stimulation (TMS) in a group of patients with lower motor neuron disease (LMND). Among LMND, several chronic immune mediate motor neuropathies may simulate amyotrophic lateral sclerosis (ALS). METHODS Forty patients with LMND were included TMS was performed at the first visit. The patients(More)
Current practice guidelines state that enteral feeding should be proposed and provided whenever ALS patients show significant dysphagia and/or weight loss. Percutaneous endoscopic gastrostomy (PEG) is usually available at ALS centres, but this method is not indicated in the case of patients with severe respiratory impairment. We undertook a study to assess(More)
It has been suggested previously that multifocal motor neuropathy (MMN) without conduction block (CB) or other features of demyelination is axonal in nature. Conventional transcranial magnetic stimulation (TMS) and the triple-stimulation technique (TST) performed on 10 MMN patients without CB revealed a proximal focal CB in 4 patients. In 3 other patients,(More)
BACKGROUND The optimal dose of intravenous immunoglobulin (IVIG) in acute exacerbation of myasthenia gravis remains unknown. Increasing the treatment duration might provide added efficacy. OBJECTIVE To determine the optimal dose of IVIG for treating myasthenia gravis exacerbation. DESIGN Randomized double-blind placebo-controlled multicenter trial(More)
OBJECTIVE To describe the phenotypic spectrum of dysferlin (DYSF) gene mutations (which cause dysferlinopathies, autosomal recessive muscular dystrophies) in patients with a dysferlin protein deficiency. DESIGN Clinical, biological, and pathological data from 40 patients were reviewed. The diagnosis of dysferlinopathy was based on the absence or strong(More)
Respiratory muscle involvement is one of the main prognostic factors in amyotrophic lateral sclerosis (ALS). Acute respiratory failure is sometimes the first manifestation of the disease, although onset can be more insidious. In the present retrospective study, it was proposed to review the clinical features and to assess the effects of non-invasive(More)
OBJECTIVE Among Parkinsonian syndromes, pyramidal signs suggesting cortico-spinal impairment are a hallmark of multiple system atrophy (MSA). Although it is crucial to diagnose correctly this disease to choose the appropriate treatment, the available diagnostic criteria lack sensitivity. Cortical excitability patterns assessed by transcranial magnetic(More)
BACKGROUND Charcot-Marie-Tooth disease type 1A (CMT1A) is a hereditary peripheral neuropathy that affects roughly one in 5000 births. No specific therapy currently exists for this degenerative disorder, which is characterised by distal progressive muscle atrophy and sensory loss, although ascorbic acid has been shown to reduce demyelination and improve(More)
In view of the conflicting results about the links between lower and upper motor neuron (LMN, UMN) dysfunction in amyotrophic lateral sclerosis (ALS), we undertook this study to correlate their changes over time. Single motor units (MUs) were characterized by their macro-MU potentials, twitch amplitude, and excitatory responses to transcranial magnetic(More)