밀러 피셔 증후군에서 보이는 지연성 안면마비의 임상양상과 전기생리학적 소견
Clinical and Electrophysiological Characteristics of Delayed Facial Palsy in Miller-Fisher Syndrome
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Background: Miller-Fisher syndrome (MFS) is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia,and is considered a variant form of Guillain-Barre syndrome. Although some cases of delayed-onset facial palsy in MFS have been reported, the characteristics of this facial palsy are poorly described in the literature. Methods: Between 2007and 2010, six patients with MFS were seen at our hospital. Delayed facial palsy, defined as a facial palsy that developed while the other symptoms of MFS began to improve following intravenous immunoglobulin treatment, was confirmed in four patients. The clinical and electrophysiological characteristics of delayed facial palsy in MFS, as observed in these patients,are described here. Results: Four patients with delayed-onset facial palsy were included. Delayed facial palsy developed 8-16days after initial symptom onset (5-9 days after treatment). Unilateral facial palsy occurred in three patients and asymmetric facial diplegia in one patient. The House-Brackmann score of facial palsy was grade III in one patient, IV in two patients,and V in one patient. None of the patients complained of posterior auricular pain. Facial nerve conduction studies revealed normal amplitude in all four patients. The blink reflex showed abnormal prolongation in two patients and the absence of action potential formation in two patients. Facial palsy resolved completely in all four patients within 3 months. Conclusions:Delayed facial palsy is a frequent symptom in MFS and resolves completely without additional treatment. Thus, standard treatment and patient reassurance are sufficient in most cases.
Abstract
Background: Miller-Fisher syndrome (MFS) is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia,and is considered a variant form of Guillain-Barre syndrome. Although some cases of delayed-onset facial palsy in MFS have been reported, the characteristics of this facial palsy are poorly described in the literature. Methods: Between 2007and 2010, six patients with MFS were seen at our hospital. Delayed facial palsy, defined as a facial palsy that developed while the other symptoms of MFS began to improve following intravenous immunoglobulin treatment, was confirmed in four patients. The clinical and electrophysiological characteristics of delayed facial palsy in MFS, as observed in these patients,are described here. Results: Four patients with delayed-onset facial palsy were included. Delayed facial palsy developed 8-16days after initial symptom onset (5-9 days after treatment). Unilateral facial palsy occurred in three patients and asymmetric facial diplegia in one patient. The House-Brackmann score of facial palsy was grade III in one patient, IV in two patients,and V in one patient. None of the patients complained of posterior auricular pain. Facial nerve conduction studies revealed normal amplitude in all four patients. The blink reflex showed abnormal prolongation in two patients and the absence of action potential formation in two patients. Facial palsy resolved completely in all four patients within 3 months. Conclusions:Delayed facial palsy is a frequent symptom in MFS and resolves completely without additional treatment. Thus, standard treatment and patient reassurance are sufficient in most cases.
참고문헌 (11)
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