e-book Neuromuscular Junction Disorders: Diagnosis and Treatment

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Resistant cases may benefit from long term immunosuppression with prednisolone and azathioprine, though not all patients respond to this. It goes without saying that any underlying tumour should be treated appropriately, though like MG this is unlikely to result in neurological remission. Autoimmune disorders of the NMJ, although uncommon, are a fascinating group of diseases, both clinically and scientifically. Although the pathophysiology of these conditions is now well understood, management is still largely based on anecdote and personal preference, and there is a clear need for well designed randomised trials to clarify the optimal treatment for these diseases.

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Table 1 Comparison of the key features of the different autoimmune conditions that affect the neuromuscular junction. Figure 1 Diagrammatic representation of neuromuscular transmission. Figure 2 Treatment algorithm for myasthenia gravis MG. Making the diagnosis Clinical features The first symptom noticed by the patient is difficultly walking, generally because of proximal leg weakness but also occasionally because of ataxia.

Congenital myasthenic syndromes. Adv Neurol ; 88 : — OpenUrl PubMed.


Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med ; 7 : —8. Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry ; 65 : —6. A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Myasthenia gravis study group. Neurology ; 50 : — A clinical therapeutic trial of cyclosporine in myasthenia gravis.


Ann N Y Acad Sci ; : — Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia gravis clinical study group. Ann Neurol ; 41 : — Practice parameter: thymectomy for autoimmune myasthenia gravis an evidence-based review : report of the quality standards subcommittee of the American Academy of Neurology.

Neurology ; 55 : 7 — Neurological paraneoplastic syndromes in patients with small cell lung cancer.

A prospective survey of patients. J Neurol Neurosurg Psychiatry ; 54 : —7. Newsom-Davis J. A treatment algorithm for Lambert-Eaton myasthenic syndrome. Hart IK. Autoimmune neuromyotonia. Channelopathies of the nervous system. Oxford: Butterworth-Heinemann, — Read the full text or download the PDF:. All patients require careful nursing in an intensive care setting with close co-operation among anaesthetists, intensivists, and neurologists.

Adequate analgesia is essential, especially after thymectomy, and thoracic epidural analgesia has been used successfully. However, many patients have a decreased requirement in the first 48 h. Spontaneous recovery tends to occur within 6 weeks although anticholinesterase therapy and occasionally mechanical ventilation of the lungs may be needed.

This may be associated with penicillamine therapy; it usually resolves when the drug is withdrawn. Congenital myasthenic syndromes CMS are a rare, heterogeneous group of genetic diseases that can affect all components of the NMJ. Often the clinical features are of generalized, often life-threatening weakness from birth. Postsynaptic CMS are caused by genetic defects in the AChR subunits and are generally divided into slow- and fast-channel syndromes. It often presents in early adolescence with facial, limb, and respiratory muscle weakness but ocular muscles are unaffected. Quinidine, an open-channel blocker, is the treatment of choice.

Owing to their rarity, little anaesthetic literature exists about anaesthesia and the CMS. Sedation of a 3-yr-old child with CMS with isoflurane was uneventful 28 and successful spinal anaesthesia for Caesarean section has also been reported.

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Volume Article Contents. Structure and function of the NMJ. Diseases affecting the presynaptic NMJ complex Table 1.


Neuromuscular junction disease

Neuromuscular junction in health and disease N. Oxford Academic. Google Scholar. Cite Citation. Fig 1. Open in new tab Download slide. Fig 2. Open in new tab. Incubation period 4—14 days. Rare and complex group of diseases. Slow channel syndromes are because of a defect producing prolonged opening of AChR channels.

Results in varying degrees of myopathy. Thoracic epidural anaesthesia and analgesia with bupivacaine for transsternal thymectomy for myasthenia gravis. Search ADS. Lambert—Eaton myasthenic syndrome: pre-anaesthetic treatment with intravenous immunoglobulins.

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Google Preview. Predicting the need for postoperative mechanical ventilation in myasthenia gravis. Resistance to succinylcholine in myasthenia gravis: a dose—response study. Congenital myasthenic syndromes: multiple molecular targets at the neuromuscular junction. Paucity and disorganisation of presynaptic membrane active zones in the Lambert—Eaton myasthenic syndrome.

Autoantibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Difference in sensitivity to vecuronium between patients with ocular and generalized myasthenia gravis. Sevoflurane as a sole anaesthetic agent for thymectomy in myasthenia gravis. Perioperative management of a patient with congenital myasthenic syndrome for elective Caesarean section. Le Gars. Nicotinic acetylcholine receptors of muscles and nerves: comparison of their structures, functional roles and vulnerability to pathology.

Halothane-induced variability in the neuromuscular transmission of patients with myasthenia gravis. The spectrum of mutations causing endplate acetylcholinesterase deficiency.