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Guillain-Barré syndrome is defined as an acute, monophasic polyneuropathy. It recurs in 2%-5% of patients.1 Both the initial and recurrent episodes are preceded by an infection. Antibodies against several gangliosides are often present in the acute phase in serum samples from patients with Guillain-Barré syndrome.2 There have been no previous reports, however, on anti-ganglioside antibodies in patients with recurrent disease. We report finding anti-ganglioside antibodies in a patient with recurrent Guillain-Barré syndrome during the third and fourth episodes.
Ten days after a bout of upper respiratory tract infection, a 26 year old man noted paraesthesias in his fingers and toes, and rapidly developed limb weakness. He had bulbar palsy and areflexic tetraparesis on day 1, and underwent three sessions of plasmapheresis on days 3, 4, and 5. Disability was maximal on day 6. Bulbar weakness disappeared on day 8, and he could walk without support on day 14. He had had similar episodes of acute, monophasic paralytic disease at the ages of 14 and 19. Six years later, he experienced a fourth episode. Three days after an upper respiratory tract infection, he rapidly developed dysarthria, digital paraesthesias, and leg weakness. Neurological examination on day 2 showed bilateral external ophthalmoplegia, facial diplegia, and areflexic tetraparesis. He required mechanical ventilation from days 2 to 19, and underwent five sessions of plasmapheresis on days 2, 3, 4, 6, and 9, then intravenous immunoglobulin therapy from days 11 to 15. His disability began to lessen on day 11 and had disappeared by day 34.
An enzyme linked immunosorbent assay3 showed IgG antibody titres to GM1 (<500), GM1b (128 000), GD1a (128 000), GalNAc-GD1a (<500), GT1a (64 000), GD1b (4000), and GQ1b (32 000) on day 3 during the third episode. No IgM anti-ganglioside antibodies were detected. On day 113, during the recovery phase, the anti-ganglioside IgG titres were <500. Serum on day 3 of the fourth episode had IgG antibodies titres to GM1 (<500), GM1b (256 000), GD1a (128 000), GalNAc-GD1a (<500), GT1a (64 000), GD1b (1:4000), and GQ1b (1:64000). Thin layer chromatography with immunostaining4 confirmed that the IgGs in the acute, progressive phase of both attacks reacted with the same gangliosides (data not shown).
Differentiation between Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP) relies on the time needed to reach maximum disability. Our patient had four episodes of typical Guillain-Barré syndrome, not of CIDP. Neurological function in each episode reached a nadir within 2 weeks of onset and returned to normal within 5 weeks. The distinction between Guillain-Barré syndrome and CIDP, however, is blurred in the early onset phase in some patients. Protein concentrations in CSF in CIDP tend to remain raised during remission and at the onset of recurrence; whereas CSF protein concentrations are normal within 1 week of the onset of Guillain-Barré syndrome, which may provide a way of distinguishing it from CIDP.1 Only low anti-ganglioside antibody titres, which were unlikely to have a role in the pathogenesis, were found in some patients with CIDP.3 The presence of high anti-ganglioside antibody titres at the onset of a recurrence may be of use in confirming the diagnosis of recurrent Guillain-Barré syndrome.
In recurrent Guillain-Barré syndrome, the nature of the antecedent illness usually tends to differ from episode to episode.1Our patient, however, had similar upper respiratory symptoms before each onset of neurological symptoms. Antecedent agents such asCampylobacter jejuni and cytomegalovirus have ganglioside epitopes,4 5 and therefore may produce the anti-ganglioside antibodies in Guillain-Barré syndrome.
This research was supported in part by grants in aid from the Uehara Memorial Foundation, Ciba-Geigy Foundation (Japan) for the Promotion of Science, Nakabayashi Trust for ALS Research, the Ryoichi Naito Foundation for Medical Research, and a Research Grant for Neuroimmunological Diseases from the Ministry of Health and Welfare of Japan.
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