A child with anti-GQ1b syndrome presenting with complete ophthalmoplegia and unilateral facial palsy.

2014 
Introduction : The classical phenotype of Miller Fisher syndrome is characterized by ophthalmoplegia, ataxia and areflexia. However, less extensive forms have been described. We report a child with positive anti-GQ1b antibodies with unusual clinical findings. Methods : The clinical records of a child with positive anti-GQ1b antibodies were reviewed and reported. Results : a 14-years-old boy presented with headache, double vision and vomiting for 7 days. There was no history of fever, gait disturbances, altered sensorium or difficulty in swallowing. He had a viral respiratory infection 15 days prior to onset of symptoms. Examination revealed complete opththalmoplegia, right lower-motor-neuron facial palsy, no limb weakness or cerebellar signs and normal fundus. CSF examination was normal with negative oligoclonal bands. MRI brain was normal. Electrophysiological studies done in second week of illness showed normal limb nerve conduction studies, low CMAP amplitude of right facial nerve, abnormal blink reflex and negative repetitive nerve stimulation test. The neostigmine challenge test was negative. Lyme disease serology was negative. Anti-GQ1b antibodies were positive with negative Anti-GM1, -GM2, -GM3, -GD1a, -GD1b, -GT1b antibodies. The child was managed conservatively. The child showed complete recovery after 3 months. Conclusions: The patients with positive anti-GQ1b antibodies who do not demonstrate the full complement of the Miller Fisher syndrome triad have been reported previously. However, unilateral facial palsy has not been reported previously. This report further expands the phenotypic spectrum of anti-GQ1b syndrome.
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