1例严重抗接触素-1型眩晕症报告

Katherine Zerebiec, Magalie E. Carey, N. Kolb, Tracy Lamoy, D. Dubey, M. Hehir
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摘要

46岁,先前健康的女性通过远程医疗提出14个月进行性不对称无力,麻木,感觉异常和震颤。病史和临床检查结果提示外周病因类似于变异型CIDP的表现。电诊断测试发现只有一个运动神经脱髓鞘的迹象;其余的运动神经和感觉神经不能被唤起。MRI显示三叉神经及马尾弥漫性强化。脑脊液显示白蛋白细胞分离。该患者经甲强的松龙治疗临床怀疑的CIDP变型,更具体地说是淋巴结副病。在类固醇治疗开始两周后,脑脊液和血清中检测到接触素-1抗体,这与我们临床对自身免疫性淋巴结副病的怀疑一致,这种病对IVIG是难治性的。鉴于患者在诊断时的严重表现,添加了利妥昔单抗。自身免疫性淋巴结副病是可以治疗的,即使是在严重的情况下。缺乏临床医生的认识和延误诊断可能是有害的,在我们的病例几乎瘫痪的病人。重要的是要提高认识的疾病,它的表现和治疗管理,因为早期诊断和正确的治疗是至关重要的神经恢复。
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Case Report of a Severe Presentation of Anti-Contactin-1 Nodopathy
A 46-year-old, previously healthy woman presented via telemedicine with 14 months of progressive asymmetrical weakness, numbness, paresthesias and tremors. History and clinical exam findings suggested a peripheral etiology resembling a variant CIDP presentation. Electrodiagnostic testing identified only one motor nerve with signs of demyelination; the remainder of motor and sensory nerves were unable to be evoked. MRI showed diffuse enhancement of the trigeminal nerve and cauda equina. CSF revealed albuminocytologic dissociation. The patient was treated empirically with methylprednisolone for a clinically suspected variant of CIDP, more specifically a nodo-paranodopathy. Two weeks after initiation of steroid treatment, cerebrospinal fluid and serum yielded antibodies to contactin-1 which coincided with our clinical suspicion for an autoimmune nodo-paranodopathy, which is known to be refractory to IVIG. Rituximab was added given the patient’s severe presentation at the time of diagnosis. The autoimmune nodo-paranodopathies are treatable, even in the case of a severe presentation. Lack of clinician awareness and delay in diagnosis can be detrimental as in the case of our nearly paralyzed patient. It is important to raise awareness of the disease, its presentation and therapeutic management since early diagnosis and correct treatment is paramount for neurological recovery.
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