目的探讨原发性低颅压综合征的发病机制、临床特点及诊断问题。方法回顾性分析21例原发性低颅压综合征患者的临床资料。结果所有患者均有头痛,且头痛与体位密切相关,坐位或站位时头痛加重,平卧位时头痛减轻或消失,头痛部位位于枕部8例,额部3例,顶部4例,全头痛6例;伴随症状有恶心、呕吐15例,眩晕10例,耳鸣6例,视物模糊3例,听力下降2例;体征有颈项强直10例,病理征阳性3例。经综合治疗5～15 d全部患者均痊愈出院。结论原发性低颅压综合征原因不明确,临床特征为体位性头痛,脑脊液压力<60 mm H2O可诊断,临床医师认识不够易造成误诊。 Objective To investigate the pathogenesis, clinical features and diagnosis of primary intracranial hypertension syndrome. Methods The clinical data of 21 patients with primary intracranial hypertension were retrospectively analyzed. Results All patients had headache. Headache was closely related to body position. Headache increased when sitting or standing. Headache was relieved or disappeared in supine position. Headache was located in 8 cases of occipital, 3 cases of frontal, 4 cases of top, 4 cases of headache Six cases were accompanied with symptoms of nausea and vomiting in 15 cases, vertigo in 10 cases, tinnitus in 6 cases, blurred vision in 3 cases and hearing loss in 2 cases. There were 10 cases with signs of neck stiffness and 3 cases with positive pathology. After 5 ~ 15 days of comprehensive treatment, all patients were cured. Conclusions The causes of primary low intracranial pressure syndrome are not clear. The clinical features are positional headache. The pressure of cerebrospinal fluid <60 mm H 2 O can be diagnosed. The understanding of clinicians is not enough to cause misdiagnosis.