Carlos III Health Institute, Sinesio Delgado n. 6 (pavilion 12), 28029 Madrid, Spain.
BMC Musculoskelet Disord. 2009 Dec 17;10 Suppl 1(Suppl 1):S1. doi: 10.1186/1471-2474-10-S1-S1.
Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neuropathic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarily based on spinal or peripheral electrical stimulation. It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms. Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition. It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.
Chiari 病（或畸形）通常是一种先天性疾病，其特征是颅底解剖缺陷，小脑和脑干通过枕骨大孔疝入颈椎椎管。Chiari 综合征症状的发作通常发生在第二或第三个十年（25 至 45 岁）。症状在加重和缓解期之间可能会有所不同。对于有或无症状的患者，神经影像学技术可用于诊断 Chiari Ⅰ型畸形。对于 Chiari Ⅰ型畸形/脊髓空洞症患者，最有效的治疗方法是枕骨大孔减压术，但也有非手术治疗方法来缓解神经病理性疼痛：药物和非药物。药物治疗使用作用于疼痛不同成分的药物。非药物治疗主要基于脊髓或外周电刺激。除了与信息相关的方面，重要的是要确定患者在医疗保健、社会、教育、职业和人际关系问题方面的需求，特别是在症状出现时。目前，专家们对于该疾病的病因或如何进行治疗、监测、随访和治疗该病尚无共识。参与此类疾病患者护理的医生必须全面处理患者的管理和随访，并组织包括所有能够帮助提高患者生活质量的专业人员在内的跨学科团队。
