Chiari畸形类型I（CM-I）是后颅窝和后脑的一组畸形，包括脑桥、小脑和延髓。文献中只有一次报道过CM-I患者的间歇性瘙痒。本研究是一项横断面研究，为期12个月，在一家三级保健儿科医院进行，涉及1岁至18岁的CM-I患儿，这些患儿出现了间歇性瘙痒。三名CM-I患者表现出严重的间歇性瘙痒。患者3因癫痫开始接受卡马西平治疗，结果，他的瘙痒突然减轻了。据报道，神经病理性瘙痒与蛛网膜下腔积液、海绵状脑病、自身免疫性疾病如多发性硬化、接受透析的终末期肾病患者以及肿瘤患者有关。抗组胺药和抗过敏药对这些患者无效，说明瘙痒有一个中枢机制。目前，尚未批准专门用于治疗神经病理性瘙痒的药物。对于神经病理性瘙痒，常用的治疗方法包括抗癫痫药物、三环抗抑郁药物、Gabapentinoid、Ketamine和口服H3镇痛药物，包括Butorphanol和Difelikefalin。需要更好结构的前瞻性研究来分析神经病理性瘙痒在CM中的流行率和评估残疾程度，并可增进人们对此领域的理解。版权所有 © 2022 Elsevier Inc. All rights reserved.

Chiari malformation type I (CM-I) is a group of deformities in the posterior fossa and hindbrain, including the pons, cerebellum, and medulla oblongata. Paroxysmal pruritus in CM-I has been reported only once before in the literature. This study was a cross-sectional study over 12 months at a tertiary care pediatric hospital involving children aged one to 18 years with CM-I presenting with paroxysmal itching. Three patients with CM-I presented with severe episodes of paroxysmal itching. Patient 3 was started on carbamazepine therapy for seizures, and incidentally, his itching subsided. The pruritus of neuropathic etiology has been reported to be associated with syringomyelia, spongiform encephalopathies, autoimmune disorders like multiple sclerosis, patients with end-stage renal failure on dialysis, and neoplasms. Antihistamines and antiallergics are ineffective in treating these patients, reiterating a central mechanism for pruritus. At present, no drugs have been approved for the treatment of neuropathic pruritus specifically. The commonly used treatments for neuropathic itch are antiseizure medications, tricyclic antidepressants, gabapentinoids, ketamine, and oral kappa opioids, including butorphanol and difelikefalin. Better structured prospective studies are needed to analyze the prevalence and scales to assess disability caused due to neuropathic itch in CM and may enhance understanding in this area.Copyright © 2022 Elsevier Inc. All rights reserved.