Segura-Chávez Darwin, Tagle-Lostaunau Isabel, Sifuentes-Monge Juan, Aquino-Peña Francisco
Instituto Nacional de Ciencias Neurológicas, Lima, Perú.
Medwave. 2023 Apr 27;23(3). doi: 10.5867/medwave.2023.03.2663.
Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies.
A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis.
The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.
吉兰-巴雷综合征是一种自身免疫性起源的多发性神经根神经病,被认为是急性弛缓性麻痹最常见的原因。已有报道吉兰-巴雷综合征与其他非神经自身免疫性疾病的各种关联,其中一些极为罕见,例如与原发性胆汁性胆管炎相关的情况,原发性胆汁性胆管炎是一种自身免疫病因的慢性疾病,其诊断也得到临床症状、肝酶改变和抗线粒体抗体存在的支持。
一名38岁男性患者,既往无合并症病史,在两周前出现腹泻病后,出现亚急性起病的上行性无力,伴有四肢感觉异常,进展为四肢瘫和呼吸窘迫。进行了脑脊液细胞化学检查,显示蛋白细胞分离,肌电图显示与急性运动轴索性神经病相符的表现,为此他接受了0.4g/kg/天的静脉注射免疫球蛋白治疗,神经状况得到改善。自入院以来及住院期间,他的肝酶持续出现胆汁淤积型改变,此外还有轻度腹痛和全身瘙痒,为此他接受了胃肠病学评估,胃肠病学医生要求检测抗线粒体抗体,结果呈阳性。最终诊断为原发性胆汁性胆管炎。
本病例显示了吉兰-巴雷综合征和原发性胆汁性胆管炎这两种自身免疫性疾病极为罕见的关联,以至于它代表了首例报道的、与SARS-CoV-2无关的病例。