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Ataxic form of Guillain-Barré syndrome associated with anti-GD1b IgG antibody
  1. Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321–0293, Japan
  1. Dr N Yuki yuki{at}

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Richter1 proposed an ataxic variant of Guillain-Barré syndrome, in which patients have severe ataxia of the cerebellar type at the onset of Guillain-Barré syndrome but no ophthalmoplegia or severe loss of proprioceptive sense. Patients with ataxic Guillain-Barré syndrome have distal paraesthesias, areflexia, and raised CSF protein concentrations. Kusunoki et al 2 reported that of 149 patients who had anti-GQ1b IgG antibodies without profound weakness, five had acute self limited ataxia without ophthalmoplegia. The nosology of these patients, however, was not discussed. Of our 340 consecutive patients who had anti-GQ1b IgG, six had no external ophthalmoplegia and one had minimal external ophthalmoplegia. The clinical features of these seven anti-GQ1b-positive patients were consistent with an “ataxic form of Guillain-Barré syndrome” (Yuki et al, unpublished observations). Tentative diagnoses made by the primary physicians were Guillain-Barré syndrome (n=3), atypical Miller Fisher syndrome (n=3), and acute cerebellar ataxia (n=1). Arakiet al,3 however, reported on a patient with Guillain-Barré syndrome who had prominent cerebellar signs. That patient had high monospecific anti-GD1b IgG antibody titre in the acute phase of the illness, but did not have anti-GQ1b IgG. These findings led us to examine whether some patients in whom acute cerebellar ataxia has been diagnosed have anti-GD1b IgG antibodies.

Serum samples were obtained from 39 patients for whom acute cerebellar ataxia or acute cerebellitis had tentatively been diagnosed. One patient who had associated anti-GQ1b IgG was excluded because the tentative diagnosis was acute cerebellar ataxia and the final one ataxic Guillain-Barré syndrome. Serum IgM or IgG antibodies to GM1, GM2, GD1a, GD1b, GT1b, GQ1b, or GQ1bα were measured by an enzyme linked immunosorbent assay as described elsewhere.4GQ1bα is a possible target molecule for serum antibodies from patients with sensory ataxic neuropathy.4 Serum was considered positive when the antibody titre was 500 or more. One of the 39 patients had a high anti-GD1b IgG titre of 16 000 but carried no antibodies to the other six gangliosides. His clinical presentation is that described later. The other 38 patients had no antibodies to those gangliosides. Further study showed that serum from the patient who had anti-GD1b IgG did not react with asialo-GM1, fucosyl-GM1, GM1b, GalNAc-GM1b, GalNAc-GD1a, GD3, GT1α, GT1aα, or sulfated glucuronyl paragloboside.

A 55 year old man had a cough and nasal discharge that disappeared after a few days. After resolution of this illness, he noted paraesthesias in his fingers and toes (day 1) which worsened, and he developed an unsteady gait on day 3. He was apyrexial and fully conscious. Blepharoptosis was absent. Ocular movement was not limited, but smooth pursuit was saccadic. His pupils were normal, and light reflexes prompt. Neither facial nor oropharyngeal palsy was present. Limb weakness was insignificant. Deep tendon reflexes were absent. Babinski's sign was negative. Finger to nose and heel to knee tests were dysmetric and uncoordinated. Romberg's sign was negative, but standing in a tandem position was unsteady. Tandem gait was impossible, and assistance was needed to walk. Paraesthesias of the glove and stocking type were present. There was no impairment of pinprick, touch, position sense, or vibratory sensation. Autonomic nervous function was normal except for hyperhidrosis in the palmar and planter surfaces. On days 3, 4, 6, 8, and 10, he underwent immunoadsorption treatment. The neurological signs rapidly disappeared. Motor and sensory nerve conduction values were normal on days 7 and 21. Protein in CSF was 32 mg/dl on day 3, and 60 mg/dl on day 10 with normal cellularity. On day 24 he was discharged without clinical signs, but still with mild paraesthesias in the right fingers. No external ophthalmoplegia was found during the course of the illness.

Acute cerebellar ataxia had been tentatively considered, but the ataxic form of Guillain-Barré syndrome, as proposed by Richter,1 could be diagnosed. This is important because some patients in whom acute cerebellar ataxia has been diagnosed may have ataxic Guillain-Barré syndrome, and they could benefit from undergoing established treatment for the syndrome—namely, plasmapheresis or intravenous administration of immunoglobulins. Serum samples from patients for whom acute cerebellar ataxia or acute cerebellitis is diagnosed should be tested for anti-GD1b and anti-GQ1b IgG antibodies in order not to overlook cases of ataxic Guillain-Barré syndrome.

Monospecific anti-GD1b IgG antibodies have been detected in a patient with Guillain-Barré syndrome who had prominent sensory ataxia and in a patient who had acute distal paraesthesias and areflexia after upper respiratory tract infection.5 6 Whether the presence of monospecific anti-GD1b IgG is correlated with a particular clinical condition, therefore, is uncertain. By contrast, a patient with cerebellar ataxia and chronic polyneuropathy has been reported to have IgM M-protein to GD1b, GM1, and asialo-GM1.7 Both the monoclonal IgM with anti-GD1b activity and murine monospecific anti-GD1b monoclonal antibody bind to the human cerebellar granular layer. The binding of anti-GD1b IgG to the cerebellar granular layer or spinocerebellar 1a fibres in the peripheral nerves may have produced the patient with cerebellar ataxia reported by Arakiet al 3 and our present patient.