Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There have been several reports describing that immunoadsorption therapy improves the neurologic involvement in Fisher's syndrome (FS). However, few studies have assessed the usefulness of immunoadsorption therapy in view of the removal ability of anti-GQ(1b) antibody, which may function the development of FS. We examined the ability of immunoadsorbents for the anti-GQ(1b) antibody in a patient with FS. A 28-year-old woman developed diplopia and giddiness following a cough, fever and diarrhea. On admission (day 22), neurologic examination showed bilateral moderate oculomotor paralysis and bilateral complete abducens paralysis. She had areflexia, numbness of middle and ring fingers on the left and mild ataxic gait. Her serum had IgG anti-GQ(1b) and anti-GD(1b) antibodies. We examined the absorption of anti-ganglioside antibodies onto a polyvinyl alcohol gel (PVA), a phenylalanine-linked PVA (PH-350) and a tryptophan-linked PVA (TR-350) by the batchwise adsorption method. TR-350 absorbed the autoantibodies, but the removal ability of autoantibody by PVA and PH-350 was not proved. The FS patient was treated with TR-350 (days 29, 34 and 43) and PH-350 (day 39). Anti-GQ(1b) and anti-GD(1b) antibodies were significantly removed by the TR-350, in accordance with the results of the in vivo study. There was little loss of albumin as compared with the immunoglobulins and complements. The numbness and ataxia disappeared on day 44. The diplopia disappeared on day 106. TR-350 would be better than PH-350 in the treatment of FS by immunoadsorption therapy.
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PMID:[Immunoadsorption therapy on Fisher's syndrome--removal ability of anti-ganglioside antibodies by tryptophan-linked immunoadsorbent]. 866 31

The beneficial effect of plasma exchange, plasmapheresis or immunoadsorption therapy on Fisher's syndrome, suggested previously, has not been proved since no controlled studies have been conducted. In order to assess the effect of any treatment on Fisher's syndrome, simple and reasonable grading scales for evaluating the major neurological signs are needed. We tried immunoadsorption therapy in four patients with Fisher's syndrome, whose sera had anti-GQ1b antibodies. The clinical course was observed, with assessment of the severity of the major neurological signs based on grading scores; ranging from 0 to 30 for external ophthalmoplegia, from 0 to 10 for ataxia, and from 0 to 16 for areflexia. Tryptophan- or phenylalanine-linked polyvinyl alcohol gel column (TR-350, PH-350) was used as an adsorbent. In a patient who had IgG anti-GQ1b antibody and another patient who had both IgG and IgM anti-GQ1b antibodies, we compared the effectiveness of TR-350 and PH-350 to remove the anti-GQ1b antibody during the therapy. Two patients underwent immunoadsorption therapy at the height of clinical manifestations: in one patient, the therapy was discontinued because of critical hypotension and arrhythmia; the other was given only three sessions of therapy. The other two patients received six or seven sessions during the early recovering stage. All patients recovered without major neurological sequelae. Since ataxia was improved earlier than external ophthalmoplegia, the duration of hospitalization and the time of return to social life depended upon the recovery of external ophthalmoplegia. Analysis of the time course of external ophthalmoplegia score indicated that the improving period and the 50%-recovery day came earlier in the patients who were given a sufficient number of sessions than those who received an insufficient number of sessions. The treatment with TR-350 reduced the IgG anti-GQ1b antibody titer more than that with PH-350, but reduced the IgM anti-GQ1b antibody titer similarly. Immunoadsorption therapy using TR-350 has a probable beneficial effect on Fisher's syndrome even though it is carried out after the height of illness. The evaluation method for the severity of external ophthalmoplegia that we used in the present study is useful for assessing the effect of therapy on Fisher's syndrome.
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PMID:[Immunoadsorption therapy for Fisher's syndrome: analysis of the recovery process of external ophthalmoplegia and the removal ability of anti-GQ1b antibodies]. 881 Aug 48

Immunoadsorption plasmapheresis (IAP) using a tryptophan linked gel column has been shown to effectively remove serum IgG anti-GQ1b antibody which may contribute to the pathogenesis of Miller Fisher syndrome. Two patients are reported on with Miller Fisher syndrome, who developed bilateral facial palsy during IAP using a tryptophan column, while ophthalmoplegia, ataxia, and, areflexia were improving. In these patients, the titre of anti-GQ1b antibodies was reduced. The IAP using a tryptophan column has a beneficial effect on Miller Fisher syndrome but may not inhibit the development of facial palsy. The mechanism of such a dissociated effect of IAP on Miller Fisher syndrome is discussed.
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PMID:Development of facial palsy during immunoadsorption plasmapheresis in Miller Fisher syndrome: a clinical report of two cases. 952 60

We report a novel, heteroplasmic point mutation in the mitochondrial tRNA for tryptophan at position 5532. The mutation was present in all the tissues studied and segregated with the biochemical defect, with higher levels of mutation present in cytochrome c oxidase-deficient muscle fibres. The patient manifested a neurogastrointestinal syndrome with features including failure to thrive, psychomotor retardation, ophthalmoplegia, sensorineural deafness and encephalopathy together with vomiting, diarrhoea and colitis.
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PMID:A novel point mutation in the mitochondrial tRNA(Trp) gene produces a neurogastrointestinal syndrome. 1505 99