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Myasthenia gravis and related disorders
  1. B Lecky

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    Edited by H J Kaminski. The Humana Press, Totowa, 2002, US$125.00, pp 396. ISBN 1 58829 058 1

    This is a scientific and clinical review of myasthenia gravis (MG) with chapters on Lambert-Eaton syndrome, congenital myasthenic syndromes, acquired neuromyotonia, and toxin-induced neuromuscular junction disorders. Neuromuscular junction physiology, acetylcholine receptor structure, and immunology are well reviewed in the first three chapters. The clinical chapters on ocular and generalised myasthenia are uniformly strong, although I have some disagreements. The edrophonium (Tensilon) test can have complications and my preference is to give atropine beforehand. In my experience, few patients with ocular MG (OMG) tolerate ptosis crutches or using adhesive tape. Using steroids “as a last resort” is somewhat extreme: many OMG patients respond well, without side effects, and severe chronic untreated ophthalmoparesis results in fixed deficit which may require strabismus surgery or botulinum toxin treatment. In generalised MG, the use of pyridostigmine, steroids, and azothioprine is discussed. What if these fail? Ciclosporine (bad side effect profile) and mycophenolate are mentioned, but methotrexate is not.

    Most readers will not be familiar with the complexity of thymic anatomy and the range of thymectomy procedures. Thymectomy has never been evaluated in a prospective controlled trial but hopefully this will be starting soon subject to funding (Jaretzki has slightly jumped the gun here). This review is excellent.

    The chapter on acquired neuromyotonia is handicapped by the lack of clear definition, clinically or neurophysiologically, of neuromyotonia and cramp-fasciculation syndrome. Congenital myasthenia can be very difficult to diagnose and the chapter has an internal contradiction, referring to disorders presenting in infancy and early childhood but then describing adult onset presentations (which are often misdiagnosed). Living muscle microelectrode studies are available in very few centres and clearer clinical guidance would have been helpful. The chapter on toxic neuromuscular disorders (things that bite you or things you should not have eaten) is worth reading.

    This book is strongly recommended to clinicians and researchers dealing with neuromuscular disorders (and to thoracic surgeons performing thymectomy).

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