Recurrent Ophthalmoplegia Presenting Different Clinical Features in a Patient with Anti-GQ1b Antibody Syndrome

Dear Editor, It has been proposed that Miller-Fisher syndrome (MFS), Guillain-Barré syndrome (GBS), Bickerstaff’s brainstem encephalitis, and acute ophthalmoplegia without ataxia are considered to make up a continuous spectrum of illness: anti-GQ1b antibody syndrome [1-3]. We present a case of recurrent anti-GQ1b antibody syndrome that demonstrates why this syndrome should be regarded as a spectrum disease. A 37-year-old man experienced sore throat and mild fever. Two weeks after the onset of his symptoms, he was admitted to the Department of Neurology at the Asan Medical Center for acute binocular horizontal diplopia. With the onset of diplopia, he also reported clumsiness in his hands, giddiness, unsteady gait, limb weakness, and dysphagia. When he was referred for an ophthalmologic work-up, esotropia of 15 prism diopters was observed in the primary position on the alternate prism cover test. On ocular motility examination, -2 limitation of abduction and -1 limitation of adduction with gaze-evoked nystagmus were noted in both eyes. The patient’s vertical gaze was intact. He had normally reactive pupils to both light and near stimulus. He was fully conscious and oriented. However, he could not walk without support due to gait ataxia. The patient also had dysmetria and finger-nose ataxia. His deep tendon reflexes were noted as normo-hyperactive. Blood laboratory tests, cerebrospinal fluid examination, and brain and orbital magnetic resonance images were revealed to be normal. In the serologic analysis of antibodies against ganglioside complexes (anti-GD1b, anti-GM1, anti-GQ1b, and anti-GT1b antibodies) using GanglioCombi ELISA (Bühlmann Laboratories, Schönenbuch, Switzerland), anti-GQ1b IgG antibody was positive (100%), and anti-GQ1b IgM antibody was borderline positive (43.87%) (borderline range, 30% to 50%). After treatment with intravenous immunoglobulin, the patient’s neurological symptoms began to improve. Two months later, the abnormality in ocular motility had completely disappeared. Eighteen months after the initial episode, the same patient presented with recurrent binocular diplopia. With the onset of diplopia, he reported mild dizziness, but no clumsiness, giddiness, unsteady gait, limb weakness, or dysphagia. On the alternate prism cover test, esotropia of 8 prism diopters was observed in the primary position. Ocular motility examination revealed a -1 limitation of abduction and a -1 limitation of adduction with gaze-evoked nystagmus in both eyes (Fig. 1). Although the degree of ophthalmoplegia was less severe than that of the first episode, the oculomotor findings suggesting bilateral sixth cranial nerve nucleus and medial longitudinal fasciculus abnormality were quite similar to those from the first episode. The same laboratory and imaging work-ups as performed in the initial episode were repeated. Brain magnetic resonance images Korean J Ophthalmol 2016;30(4):314-315 http://dx.doi.org/10.3341/kjo.2016.30.4.314