Numata Yurika, Uematsu Mitsugu, Hino-Fukuyo Naomi, Kakisaka Yosuke, Kobayashi Tomoko, Hirose Mieko, Haginoya Kazuhiro, Tsuchiya Shigeru
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Miyagi.
No To Hattatsu. 2011 Nov;43(6):482-5.
We report a case of a 3-year-old boy with acute muscle weakness that initially affected neck and all four limbs but later vanished from the lower limbs. Pharyngeal palsy was not observed during the course. All deep tendon reflexes were absent. Peripheral nerve conduction studies showed a demyelination pattern in each limb. The patient received intravenous high-dose corticosteroid hormone, followed by two immunoglobulin therapies. His muscle strength gradually improved after treatment and was almost completely restored four months later. We ultimately diagnosed the condition as the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, in consideration of the patient's muscle weakness of the neck and four limbs, the greater degree of weakness of the upper limbs versus the lower limbs. His clinical presentation was atypical for the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, as he presented no pharyngeal muscle weakness or anti GT1a antibodies, typical manifestations of the condition.
我们报告一例3岁男孩，患有急性肌无力，最初影响颈部及四肢，但随后下肢肌无力消失。病程中未观察到咽麻痹。所有深腱反射均消失。周围神经传导研究显示每个肢体均有脱髓鞘模式。患者接受了静脉高剂量皮质类固醇激素治疗，随后进行了两次免疫球蛋白治疗。治疗后其肌力逐渐改善，四个月后几乎完全恢复。考虑到患者颈部和四肢肌无力，上肢肌无力程度大于下肢，我们最终将该疾病诊断为吉兰-巴雷综合征的咽-颈-臂变异型。他的临床表现对于吉兰-巴雷综合征的咽-颈-臂变异型来说并不典型，因为他没有咽肌无力或抗GT1a抗体，而这些是该疾病的典型表现。
