Combined plasma exchange and immunosuppression in myasthenia gravis.
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Biomedical subjects
Publications and source records attributed to P O Behan.
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Twenty-one patients with myasthenia gravis underwent a course of plasma exchange combined with immunosuppressive therapy. In fifteen there was dramatic clinical improvement which has been maintained for periods up to 19 months. Nine of these patients now take no anticholinesterase drugs. Six patients had a recurrence 3--9 months after the first course but in the three given a second course remissions were again obtained.
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The neuropathological findings in six patients who developed neurological signs after the onset of "septic shock" caused by Gram-negative septicaemia are described. The changes in the brains were characteristic of acute haemorrhagic leucoencephalitis, and there was evidence, particularly in the kidneys, of disseminated intravascular coagulation with tubular necrosis and, in some, appearances indistinguishable from membrano-proliferative glomerulonephritis. It is agreed that acute haemorrhagic leucoencephalitis is another manifestation of a generalised Shwartzman reaction, and it is suggested that activation of complement is the final common pathway that produces tissue damage in the brain and kidney.
Complement components were measured and immune complexes were sought in 75 patients with myasthenia gravis. Thirty-four per cent had decreased concentrations of complement component C4, and 29% had circulating immune complexes. The greatest immunological abnormalities were found in patients with mild disease which supports recent immunoelectronmicroscopic findings.
Low serum IgA levels were found in patients taking phenytoin, together with evidence of depressed T cell function. There was no correlation between the dose or the serum level. A correlation was found, however, with HL-A status, patients with a low IgA showing increased frequency of HL-A2. It is suggested that epileptic patients with HL-A2 status are likely to develop IgA deficiency when given phenytoin.
The possibility that acute disseminated encephalomyelitis (ADEM) and epidemic myalgic encephalomyelitis ('epidemic neuromyasthenia') may share a common pathogenesis is examined and many factors common to the two diseases are described. It is suggested that further study of ADEM may help our understanding of epidemic myalgic encephalomyelitis.
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The distribution of histocompatibility antigens was studied in 44 patients with motor neurone disease (MND). An unusually high incidence of HLA antigens A2 and A28 was found, compared to a control population.
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We describe here a distinct syndrome of chorea without dementia, occurring in three generations of a family and inherited as a mendelian dominant.
Patients with and without adverse reactions on practolol therapy showed altered immune responses. There was cutaneous anergy to Candida albicans and streptokinase/streptodornase antigens and depression of lymphocyte function in vitro. Anticomplementary activity and a wide range of autoantibodies were found in patients who had received practolol.
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