背景:慢性炎性脱髓鞘性多神经根神经病(CIDP)中不常见颅神经受累;对于舌下神经受累尤其如此。之前在CIDP中都没有描述过Beevor的符号或其倒置形式。
方法:一名28岁男子在18岁时出现远端优势肢体无力和麻木。诊断为CIDP,脱髓鞘的电诊断证据证实了这一点。他对静脉注射免疫球蛋白和糖皮质激素治疗反应良好,并在5年内获得缓解。然而,同样的症状在28岁时复发,持续了10个月。在检查中,除了肢体感觉障碍和肌肉无力,轻度双侧面部轻瘫,舌头萎缩和肌束震颤,还观察到倒Beevor的标志。还对CIDP和Beevor征或其倒置形式的颅神经受累进行了简短的文献综述。
结论:CI-DP患者的颅神经可能受到影响。面瘫是最常见的,而舌下神经受累是罕见的。反向Beevor征可以出现在CIDP患者中。
BACKGROUND: Cranial nerve involvement is not commonly encountered in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); this is especially true for involvement of the hypoglossal nerve. Neither Beevor\'s sign nor its inverted form has previously been described in CIDP.
METHODS: A 28-year-old man presented with distal-predominant limb weakness and numbness at the age of 18. A diagnosis of CIDP was made, which was confirmed by electrodiagnostic evidence of demyelination. He responded well to intravenous immunoglobulin and glucocorticoid treatment and achieved remission for 5 years. However, the same symptoms relapsed at the age of 28 and lasted for 10 months. On examination, in addition to limb sensory impairment and muscle weakness, mild bilateral facial paresis, tongue atrophy and fasciculations, and inverted Beevor\'s sign were also observed. A brief literature review of cranial nerve involvements in CIDP and Beevor\'s sign or its inverted form were also performed.
CONCLUSIONS: Cranial nerves may be affected in patients with CIDP. Facial palsy is most frequently present, while hypoglossal nerve involvement is rare. Inverted Beevor\'s sign can appear in CIDP patients.