The high incidence of elevated immunoglobulin (Ig)G anti-GQ1b antibody in sera from patients with acute phase Miller-Fisher syndrome (MFS) was first reported by Chiba et al. 1 and later confirmed by two other groups. 3,4 Chiba et al. 2 pointed out that anti-GQ1b IgG antibody is associated specifically with ophthalmoplegia in both MFS and Guillain-Barre ´syndrome (GBS). Ophthalmoplegia without the ataxia, the areflexia, or both has been designated atypical MFS 2 or acute ophthalmoparesis. 5 The detailed clinical profile of acute isolated ophthalmoplegia, however, has yet to be clarified. We report a patient who presented ophthalmoplegia without ataxia or areflexia and who had increased anti-GQ1b IgG antibody activity in the acute phase.
A 41-year-old woman had rhinorrhea and sore throat, and 2 days later suddenly experienced diplopia, dizziness, and increased tonus of the neck. She consulted our department 5 days after the onset of neurological complications. On neurological examination, abduction of the eyes was mildly impaired. There was gaze-evoked nystagmoid movement on the left side. Pupil size and reaction were normal. Deep tendon reflexes were slightly increased, without extensor plantar responses. Other cranial nerve functions, sensation in the extremities, and coordination were all intact. Laboratory findings were unremarkable. Lumbar puncture showed a fluid pressure of 100/70 mmH 2 O for the initial/final sampling, a cell count of 1/ mm 3 , and total protein of 20 mg/dL. Three days after onset a serum sample showed increased anti-GQ1b IgG antibody activity (titer, 1:640). No antibodies against other glycolipids (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1b, GA1, and galactocerebroside) were detected. Brain magnetic resonance imaging showed no abnormal findings. She was treated with a low dose of prednisolone (30 mg/ day) for 14 days, which was tapered off for the next 4 weeks. Six days after onset, abduction of the eyes was severely impaired; adduction and the upward movement of the right eye also were limited; and there was horizontal gaze nystagmus on both sides. She was aware of transient tinnitus that was more pronounced in the right ear. Three months after onset, all these symptoms and signs had lessened, except for the subjective diplopia in her horizontal gaze and the increased deep tendon reflexes.
The patient initially presented with bilateral abducens palsy followed by oculomotor palsy without areflexia or ataxia. Anti-GQ1b antibody activity was increased during the acute phase. This case directly demonstrates the close relationship between ophthalmoplegia and increased anti-GQ1b antibody.
Acute ophthalmoplegia is caused by various etiologies; cerebrovascular diseases, aneurysm of the internal carotid artery, tumors, infections, diabetes mellitus, multiple sclerosis, and myasthenia gravis. Anti-GQ1b antibody, however, is negative in all those diseases. 2 This antibody assay therefore is very useful for distinguishing the acute oph-thalmoplegia caused by the pathogenic mechanisms underlying MFS and GBS from that produced by other causes. Like the patients described by Chiba et al. 2 and Yuki, 5 our patient first presented with impaired abduction of the eyes. Bilateral abducens palsy followed by oculomotor nerve involvement probably is the most characteristic sign of this isolated ophthalmoplegia.