摘要颅颈交界区畸形是一类位于枕骨大孔及上颈椎的先天性畸形，包括骨性和神经的畸形病变，可压迫小脑和后组颅神经，导致运动、感觉障碍。颅颈交界区畸形的临床症状评估，对把握手术指征和评价手术效果非常重要，目前临床工作中常用的有日本骨科协会评分、疼痛视觉模拟评分、健康调查简表等。但这些临床评分往往侧重于描述某一类型临床症状，而不针对颅颈交界区疾病。颅颈交界区畸形症状复杂，往往需要综合使用不同评价量表才能提高效度。芝加哥Chiari畸形预后量表、宣武医院脊髓空洞结局量表是目前已发表的两个专用于颅颈交界区疾病的量表，但仍需在更大范围内开展临床研究以证明其有效性。本文结合既往文献结果，介绍了既往用于颅颈交界区骨性和神经畸形的临床评价方法，并讨论其应用特点和局限。

abstractsCraniovertebral junction malformation is a congenital malformation located in the foramen magnum and upper cervical spine, including bone and nerve malformation, resulting in motor and sensory disorders, cerebellar and lower cranial nerves, etc. The evaluation methods of clinical symptoms and efficacy of craniovertebral junction malformation are important for the surgical indications and effects, mainly including the evaluation of clinical symptoms and the quality of life. At present, the commonly used methods in clinical work and literature are the Japanese orthopaedic association scores, visual analogue scales, 36-item short-form health survey, etc. Most of these clinical evaluations are not aimed at craniovertebral junction diseases but focus on the description of a certain type of clinical symptoms. Chicago Chiari outcome scale and syringomyelia outcome scale of Xuanwu hospital are dedicated to Craniovertebral junction malformation, but more clinical studies are needed to prove their effectiveness. Based on the literature reports, this article reviewed the previous clinical evaluation methods of craniovertebral junction malformation and discusses their applications and limitations.