Browsing by Author "Linton, D M"

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    Myasthenia gravis at Groote Schuur Hospital Author: an audit (1970-1990)
    (1990) Linton, D M; Philcox, Derek
    I have reviewed all of the available international literature on myasthenia gravis (MG) up to the end of 1989, and I have established a databank of this literature for future retrieval and publications (see Annexure 1). Myasthenia gravis is a fascinating neuromuscular disease, the specific precipitating cause of which remains obscure after a century of clinical experience and five decades of scientific research (Simpson, 1983) The disease is specifically characterized by the development of clinically demonstrable weakness in voluntary muscle groups (Rowland, 1982). This weakness is accentuated by repetitive muscular activity and improved by resting the muscles (Simpson, 1981; Drachman, 1978). It is this feature of the disease, namely, the demonstrable fatiguability of striated muscle which tends to improve after periods of muscular inactivity and responds to the initial administration of acetylcholinesterase inhibitors, which distinguishes MG from other neurological and muscular disorders (Harvard, 1973). The fundamental abnormality in the most common form of MG (adult generalized) is an immunologically mediated premature degradation of acetylcholine receptors in the neuromuscular junction (Drachman, 1978; Walton, 1981). A model of classical MG has been constructed by immunologists with the thymus featuring as the leading actor and it is on this model that current medical and surgical therapy is based (Harvard, Scadding, 1983; Engel, 1 984; Simpson, 1987; Genkins et al, 1 987). There is an abundance of clinical, pathological and immunological evidence confirming the heterogenicity of myasthenia gravis (Efthimiou, 1985; Compston et al, 1980). Many questions regarding MG remain unanswered and controversy abounds, particularly relating to the technique and timing of thymectomy and the choice of therapy in different manifestations of the disease in older patients, in children and in patients with associated diseases such as thyrotoxicosis, systemic lupus erythematosis and diabetes.