Study of sensitivity to curare in certain neurological disorders using a regional technique.

Brown, J C; Charlton, J. E.

doi:10.1136/jnnp.38.1.34

Cited by 27 publications

(7 citation statements)

References 20 publications

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“…There was no significant difference in the dose‐response relationship obtained by mechanomyography between the three MD1 patients with mild to moderate disease and the normal controls. This result is also in keeping with observations made earlier [8–11].…”

Section: Discussionsupporting

confidence: 93%

Comparison of mechanomyography and acceleromyography for the assessment of rocuronium induced neuromuscular block in myotonic dystrophy type 1

Vanlinthout

Booij

Egmond³

et al. 2010

Anaesthesia

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SummaryWe measured acceleromyography and mechanomyography simultaneously with monitoring of rocuronium-induced neuromuscular block in four patients with myotonic dystrophy type 1. Furthermore, we compared neuromuscular block measures from these patients with those from normal controls from previous studies. In myotonic dystrophy type 1 patients, the dose-response curve obtained with acceleromyography was steeper and right-shifted compared with that obtained using mechanomyography. However, the effective doses to produce 95% neuromuscular block determined with both acceleromyography and mechanomyography were similar to each other and to values found in normal patients. In the three myotonic dystrophy type 1 patients with mild to moderate disease, times to recovery from block were similar to those observed in normal controls. In both patients and normal controls, neuromuscular block recovered faster with acceleromyography. However, in one patient with severe muscle wasting, recovery of neuromuscular block was prolonged. We conclude that mechanomyography and acceleromyography cannot be used interchangeably to monitor neuromuscular block in myotonic dystrophy type 1 patients.

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Section: Discussionsupporting

confidence: 93%

Comparison of mechanomyography and acceleromyography for the assessment of rocuronium induced neuromuscular block in myotonic dystrophy type 1

Vanlinthout

Booij

Egmond³

et al. 2010

Anaesthesia

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“…However, guanidine, which overcomes the defect in the latter condition, had no effect upon the MAP fade in myotonia. Furthermore, though edrophonium affected the degree of fade in two patients, sensitivity to curare is normal in myotonic disorders (Lambert and Beckett, 1950;Brown and Charlton, 1975), so there is no evidence for a defect of neuromuscular transmission. Since there is no fade in the amplitude of the action potential evoked in the ulnar nerve during repetitive stimulation, there is no evidence that defective nerve conduction could be responsible either.…”

Section: Discussionmentioning

confidence: 96%

Muscle weakness after rest in myotonic disorders: an electrophysiological study

1974

Journal of Neurology, Neurosurgery & Psychiatry

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SYNOPSIS Changes in amplitude of the evoked muscle action potential (MAP) have been observed in four patients with myotonia congenita and two with dystrophia myotonica. A fade in the response occurred in every case with stimulus frequencies of 10 per second or less, provided that the muscle was in a rested state and that long enough stimulus trains were used. Intramuscular stimulation and recording techniques show that the myotonic muscle fibre is the site of this defect. The MAP fade is thought to represent the transient weakness from which such patients may suffer, particularly after rest. Since neither this weakness nor the fade was related to the severity of the myotonia, nor were they significantly influenced by cooling or hydantoins, they may well be due to a separate defect in the myotonic muscle from that which causes the hyperexcitability of the fibre membrane.

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“…These functional changes are associated by fragmentation of the postsynaptic ACh receptor (AChR) cluster area [5,6]. DMD patients may have similar NMJ abnormalities, indicated by an increased sensitivity to the AChR blocker d-tubocurarine and the prolonged duration of its effect, as compared to healthy controls [7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

Low dystrophin levels are insufficient to normalize the neuromuscular synaptic abnormalities of mdx mice

Pijl

Putten

Niks

et al. 2018

Neuromuscular Disorders

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Dystrophin is a sub-sarcolemmal component of skeletal muscle fibres and is enriched at the postsynaptic membrane of the neuromuscular junction (NMJ). In the mdx mouse, dystrophin absence not only causes muscle damage but also mild synaptic dysfunctions and clear morphological aberrations at NMJs. In particular, reduction of postsynaptic sensitivity for the neurotransmitter acetylcholine and extra exhaustion of presynaptic acetylcholine release during intense synaptic activity exists. Current experimental therapeutic approaches in Duchenne muscular dystrophy aim to restore dystrophin expression. An important question is what dystrophin levels are needed to improve muscle function. Recent experimental and clinical studies suggested that levels as low as a few percent of normal can be beneficial. Similarly, it is of interest to know how dystrophin levels relate to NMJ function and morphology. We investigated NMJs of a series of mdx-Xist mice, which expressed dystrophin between ~2% and 19% of normal. Most functional and morphological NMJ parameters of these mice remained comparable to mdx. On the other hand, mdx mice (expressing ~50% dystrophin) showed normal NMJ features. Thus, the minimal dystrophin level required for normal NMJ function and morphology lies between 19% and 50% of normal when expression of dystrophin is not uniform.

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Study of sensitivity to curare in certain neurological disorders using a regional technique.

Cited by 27 publications

References 20 publications

Comparison of mechanomyography and acceleromyography for the assessment of rocuronium induced neuromuscular block in myotonic dystrophy type 1

Comparison of mechanomyography and acceleromyography for the assessment of rocuronium induced neuromuscular block in myotonic dystrophy type 1

Muscle weakness after rest in myotonic disorders: an electrophysiological study

Low dystrophin levels are insufficient to normalize the neuromuscular synaptic abnormalities of mdx mice

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