背景:格林-巴利综合征(GBS)是由自身免疫引起的急性炎性周围神经病变。神经节苷脂和硫酸盐是周围神经的重要组成部分。抗硫酸脂抗体介导的补体与GBS的急性感觉运动性周围神经病变有关,其特点是疼痛和感觉异常。
方法:这个孩子是一个7岁的女孩,有头痛和腹痛,其次是肢体麻木和疼痛。头颅成像显示心室扩张,周围神经功能传导检查显示多神经根病,脑脊液测试显示细胞计数正常,但蛋白质水平升高,所有这些都导致了GBS的诊断。静脉注射免疫球蛋白治疗后(400mg/kg×5天),症状没有改善,肌肉力量逐渐恶化,伴有阵发性面部潮红,心率波动,多汗症,脑脊液蛋白逐渐增加(高达3780.1mg/L)。根据这些发现结合血清抗硫酸脂IgM阳性,考虑了抗硫酸盐抗体相关的GBS,低剂量泼尼松龙(1mg/kg/d)治疗可改善症状。
结论:抗硫酸盐抗体相关GBS与小纤维性周围神经病变相关。主要表现是疼痛,感觉异常和自主神经功能障碍。除了脊髓神经根吸收功能障碍引起的脑脊液蛋白增加,自主神经功能障碍可能与疼痛有关。当免疫球蛋白的治疗效果不理想时,可以考虑低剂量和短疗程的皮质类固醇,预后良好.
BACKGROUND: Guillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral nerves. Anti-sulfatide antibody-mediated complement is associated with acute sensorimotor peripheral neuropathy in GBS, which is characterized by pain and paresthesias.
METHODS: The child was a 7-year-old girl with headache and abdominal pain, followed by limb numbness and pain. Cranial imaging showed ventricular dilatation, peripheral nerve function conduction examination showed polyradiculopathy, and cerebrospinal fluid tests showed normal cell counts but elevated protein levels, all of which led to the diagnosis of GBS. After treatment with intravenous immunoglobulin (400 mg/kg × 5 days), the symptoms did not improve, and muscle strength progressively worsened, accompanied by paroxysmal complexion flushing, heart rate fluctuation, hyperhidrosis, and a progressive increase in cerebrospinal fluid protein (up to 3780.1 mg/L). On the basis of these findings combined with serum anti-sulfatide IgM positivity, anti-sulfatide antibody-related GBS was considered, and treatment with low-dose prednisolone (1 mg/kg/d) led to symptom improvement.
CONCLUSIONS: Anti-sulfatide antibody-associated GBS is associated with small fiber peripheral neuropathy. The main manifestations are pain, paresthesias and autonomic dysfunction. In addition to the dysfunction of spinal nerve root absorption caused by increased cerebrospinal fluid protein, autonomic dysfunction may be involved in pain. When the therapeutic effect of immunoglobulin is not satisfactory, a low dose and short course of corticosteroids can be considered, and the prognosis is good.