[Immunoadsorption therapy for Fisher's syndrome: analysis of the recovery process of external ophthalmoplegia and the removal ability of anti-GQ1b antibodies].
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.
Chida
K
K
Department of Neurology, Kohnan Hospital, Sendai, Japan.
Watanabe
S
S
Okita
N
N
Takase
S
S
Tagawa
Y
Y
Yuki
N
N
D000328:Adult; D001259:Ataxia / Q000628:therapy*; D001323:Autoantibodies / Q000302:isolation & purification*; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007163:Immunosorbent Techniques; D008297:Male; D008875:Middle Aged; D009886:Ophthalmoplegia / Q000628:therapy*; D012021:Reflex, Abnormal; D012026:Reflex, Stretch; D013577:Syndrome; D014364:Tryptophan
D001323:Autoantibodies; D005732:Gangliosides; D014364:Tryptophan