Department of Paediatric Neurology, Great North Children's Hospital, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Pediatrics. 2013 Mar;131(3):e955-8. doi: 10.1542/peds.2012-1445. Epub 2013 Feb 11.
Autonomic instability is well recognized in Guillain-Barré syndrome (GBS), particularly in the acute inflammatory demyelinating polyneuropathy subtype. Hypertension occurs in up to two-thirds of children with GBS but is rarely the main presenting feature. We describe a teenager who presented with tachycardia, dizziness, flushing, and significant hypertension as well as ascending limb weakness and sensory disturbance with areflexia. Because the predominant initial concern was hypertension, she was referred to pediatric nephrology and appropriate investigations for hypertension were conducted. Her neurologic findings prompted a neurology referral, and a diagnosis of GBS was made. The investigations for hypertension subsequently revealed increased urinary normetadrenaline levels in a range consistent with pheochromocytoma, prompting the question of dual pathology. Both autonomic symptoms and urinary metadrenaline levels subsided with GBS resolution, and further investigations excluded the diagnosis of pheochromocytoma. Our case highlights that significant dysautonomia can occur in children with GBS, with hypertension being a prominent early feature. Recognition that urinary metadrenalines can increase to levels seen in pheochromocytoma is important in avoiding diagnostic confusion.
自主神经不稳定在吉兰-巴雷综合征(GBS)中很常见,特别是在急性炎症性脱髓鞘性多发性神经病亚型中。高血压发生在多达三分之二的 GBS 患儿中,但很少是主要表现特征。我们描述了一位青少年,其表现为心动过速、头晕、潮红以及显著的高血压,同时伴有上升支肌无力和反射消失。由于最初主要关注的是高血压,她被转介到儿科肾病科,并进行了适当的高血压检查。她的神经学发现促使进行神经科转诊,并做出了 GBS 的诊断。随后对高血压的检查显示尿去甲肾上腺素水平升高,范围与嗜铬细胞瘤一致,这引发了对双重病理的质疑。自主神经症状和尿代谢物水平随着 GBS 的缓解而消退,进一步的检查排除了嗜铬细胞瘤的诊断。我们的病例强调,儿童 GBS 中可能会出现严重的自主神经功能紊乱,高血压是早期的突出特征。认识到尿代谢物水平可以升高到嗜铬细胞瘤的水平,对于避免诊断混淆很重要。