Cranial polyneuropathy with elevated serum antiganglioside antibody

Abstract

We report a 6-year-old girl with cranial polyneuropathy with elevated serum levels of antiganglioside antibodies. She manifested herpetic vesicles around the right upper eyelid and mouth without antecedent infection. She developed facial asymmetry and double vision 5 days after the first apperance of the vesicles. Neurological examination on admission disclosed palsies of the bilateral sixth and twelfth cranial nerves and right third and seventh cranial nerves, but limb muscle weakness, ataxia, and areflexia were not observed. Cerebrospinal fluid examination and MRI of the brain showed no abnormalities. Serum antibodies to gangliosides GQ1b and GT1b, but not GM1, and those to Campylobacter jejuni were significantly increased on admission and on the hospital day 14. These observations suggest that the present case is a variant form of Miller Fisher syndrome or Bickerstaff's brainstem encephalitis subsequent to asymptomatic C. jejuni infection. We treated her with intravenous administration of high-dose methylprednisolone and acyclovir, but almost no effect was observed. All cranial nerve palsies, however, had resolved completely approximately 4 months later. This may be the first pediatric case in which cranial polyneuropathy and antiganglioside antibodies were associated.