We described a case with cranial form of Guillain-Barre syndrome (GBS), who was highly responsive to the pulse therapy such as the intravenous administration of methylprednisolone in high dose. A 57-year-old female was admitted because of diplopia and blepharoptosis. She has had a mild symptom of upper respiratory infection about 3 weeks before admission. Several days after admission, total ophthalmoplegia and bilateral peripheral facial nerve palsy developed rapidly. Deep tendon reflexes were lost, however, neither palsy nor ataxia were observed. The albumino-cytological dissociation in cerebrospinal fluid (CSF) was observed and anti-myelin-antibody was positive in serum and CSF. Her symptoms were resistant to conventional corticosteroid therapy consisting of oral administration of prednisolone (60 mg/day). We, therefore, applied the pulse therapy such as the methylprednisolone in high dose (1000mg/day for 3 days) in this case. Most of her symptoms, (total ophthalmoplegia, facial nerve palsy and areflexia) were recovered remarkably. At present, she is in good condition without any neurological sequelae. A cranial form of GBS, especially the type of total ophthalmoplegia without any palsy in extremities, is very rare in the literature. As the effect of steroid therapy for GBS is not established yet, much work will be necessary to evaluate the effect of pulse therapy for GBS.
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