Delphine Boërio

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Non-invasive excitability studies of motor axons in patients with amyotrophic lateral sclerosis (ALS) have revealed a changing pattern of abnormal membrane properties with disease progression, but the heterogeneity of the changes has made it difficult to relate them to pathophysiology. The SOD1(G93A) mouse model of ALS displays more synchronous motoneuron(More)
Non-invasive excitability tests have been developed to appraise axonal membrane properties in peripheral nerves and are contributing to our understanding of neuropathies and neuronopathies. These techniques have been adapted to in vivo and in vitro rat models, but little data are available on mice, although mice provide more transgenic models of(More)
OBJECTIVE To compare various techniques of stimulation and methods of analysis to estimate absolute (ARP) and relative (RRP) refractory periods in motor nerve trunks of humans. METHODS Double collision (DC) technique and two types of paired pulse (PP) technique, with test stimulation of supramaximal (PP(supra)) or submaximal (PP(sub)) intensity, were(More)
Intravenous immunoglobulin (IVIg) infusions may provide clinical benefits in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). The short delay in the clinical response to IVIg therapy is not consistent with a process of remyelination or axonal regeneration. We assessed whether or not the efficacy of IVIg(More)
The main purpose of this study was to characterise neuromuscular fatigue induced by 30 contractions of the knee extensor muscles evoked by electromyostimulation (EMS). Twelve healthy subjects were tested before and after a typical EMS session (frequency: 75 Hz, on-off ratio: 6.25 s on-20 s off) used for quadriceps femoris muscle strengthening. Surface(More)
Immediately after action potential occurrence, owing to transient sodium channel inactivation, axon excitability is reduced for a short period of time, including the absolute refractory period, a first period of total inexcitability, followed by the relative refractory period. There are basically two different stimulation protocols to estimate axonal(More)
OBJECTIVE To study peripheral motor nerve excitability in patients with multiple sclerosis (MS). METHODS Twenty MS patients with normal nerve conduction parameters and no predisposing factors for peripheral neuropathy were included. Compound muscle action potentials were recorded from the abductor digiti minimi muscle to paired-pulse stimulation of the(More)
Congenital peripheral nerve hyperexcitability (PNH) is usually associated with impaired function of voltage-gated K(+) channels (VGKCs) in neuromyotonia and demyelination in peripheral neuropathies. Schwartz-Jampel syndrome (SJS) is a form of PNH that is due to hypomorphic mutations of perlecan, the major proteoglycan of basement membranes. Schwann cell(More)
BACKGROUND Fatigue frequently occurs in myotonic dystrophy type 1 (DM1), but its pathophysiology remains unclear. This study assessed central and peripheral components of exercise-related fatigability in patients with DM1, compared to controls. METHODS Examinations were performed before and after a contraction of the abductor digiti minimi (ADM) muscle(More)
Hyperkalemia is an important cause of membrane depolarization in renal failure. A recent theoretical model of axonal excitability explains the effects of potassium on threshold electrotonus, but predicts changes in superexcitability in the opposite direction to those observed. To resolve this contradiction we assessed the relationship between serum(More)