Children with neuromuscular diseases present a wide range of clinical manifestations and clinical implications for the anaesthesiologist. Neuromuscular diseases in children affect muscle strength by either directly weakening the muscle fibrils or indirectly by a degenerative nerve supply and weak neuromuscular junction. Of the more than 200 neuromuscular disorders known, the vast majority are g...

Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy...

Focal adhesion kinase is a recently characterized tyrosine kinase that is concentrated at focal contacts in cultured cells. It is thought to play an important role in the regulation of the integrin-based signal transduction mechanism involved in the assembly of this membrane specialization. In this study, we examined the immunocytochemical distribution of focal adhesion kinase in Xenopus skelet...

introduction:yasthenia gravis is an autoimmune disorder resulting from a decreased number of active acetylcholine receptors at the neuromuscular junction. thymectomy is one of its current treatments. due to sensitivity of myasthenic patients to non-depolarizing muscle relaxants and also the interaction of this medication with anti-cholinestrase drugs, determining the dosage of non-depolarizing ...

As mammals age, their neuromuscular junctions (NMJs) gradually change their form, acquiring an increasingly fragmented appearance consisting of numerous isolated regions of synaptic differentiation. It has been suggested that this remodelling is associated with impairment of neuromuscular transmission, and that this contributes to age-related muscle weakness in mammals, including humans. The un...

This paper reports on a microfluidic device with high spatiotemporal control used as an in vitro model of neuromuscular junction (NMJ) developmental biology. The device is assembled using three poly(dimethylsiloxane) (PDMS) layers and consists of micro-trenches for long-term culture of myotubes and an array of 32 identical focal stimulation jets to stimulate the myotubes. The device operates in...

The concentration of acetylcholine (ACh) in the synaptic cleft at the neuromuscular junction (NMJ) remains impossible to quantify using direct measurements based upon spatio-temporal resolution. We present a model of the concentration of ACh in the synaptic cleft. Our model considers the release mechanism and the clearance process, represented by the hydrolysis of acetylcholinesterase (AChE) an...

As a tetramer, acetylcholinesterase (AChE) is anchored to the basal lamina of the neuromuscular junction and to the membrane of neuronal synapses. We have previously shown that collagen Q (ColQ) anchors AChE at the neuromuscular junction. We have now cloned the gene PRiMA (proline-rich membrane anchor) encoding the AChE anchor in mammalian brain. We show that PRiMA is able to organize AChE into...

INTRODUCTION The aim of this study was to re-evaluate jitter values of healthy subjects in whom pairs of single-fiber-like potentials were recorded from voluntary activated periocular muscles using a disposable 37-mm concentric needle electrode (CNE) with 2-kHz low-cut filtering. METHODS We reviewed the recordings of 129 subjects (85 women; 44 men; mean age, 43.8±15.3 years). The m. frontalis...

It is known that ring fibers are often observed in some human muscle diseases, especially in myotonic dystrophy (HEIDENHAIN, 1918; PERRY et al., 1956; SCHOTLAND et al., 1966), and in normal ocular muscles (WOHLFART, 1932; MORI, 1953; RUBINSTEIN, 1960; BETHLEM and VAN WIJNGAARDEN, 1963; INKE and SITKA, 1965 and others). Although there have been a few reports dealing with the fine structure of th...