自发性低颅压综合征14例临床分析并文献复习

Spontaneous intracranial hypotension:case report and literature review

目的分析自发性低颅压综合征的临床多样性,并讨论其可能的发病机制及治疗方法。方法回顾性分析14例自发性低颅压综合征患者的临床表现及影像学资料并文献复习。结果 14例患者均出现体位性头痛,伴恶心、呕吐8例,强迫性体位7例;体位改变造成的颅神经功能障碍明显,其中头晕11例、听力下降和耳鸣7例,并有复视3例,视物模糊、面部感觉异常以及真性球麻痹各1例;其他体位相关的神经功能障碍包括小脑性共济失调4例、意识障碍2例、肌张力增高和巴氏征阳性2例、性格改变1例及小便潴留1例。颅脑MRI可见硬脑膜强化10例、硬膜下积液10例、脑下沉10例、脑疝6例、大脑大静脉与直窦夹角缩小2例以及颅内静脉窦血栓形成1例等。脑脊液检查仅有1例患者蛋白轻度升高。结论自发性低颅压综合征临床表现多种多样,体位性因素为其显著特点。自发性脑脊液漏可能为其主要原因。保守疗法、血贴疗法、手术治疗等方式是处理自发性低颅压综合征的有效手段。

Objective To investigate the clinical characteristics of spontaneous intracranial hypotension （SIH）and explore the underlying mechanism and therapeutic strategies.Methods The clinical and radiographic features of 14 SIH cases were retrospectively analyzed.The published literatures related to SIH were reviewed.Results All patients showed orthostatic headache （14 /14）,which was complicated with nausea/vomiting in 8 cases （8 /14）,and with com-pulsive posture in 7 cases （7 /14）.Orthostatic cranial nervous impairments included dizziness （11 /14）,hearing loss and tinnitus （7 /14）,diplopia （3 /14）,blurred vision （1 /14）,facial paresthesia （1 /14）and true bulbar paralysis （1 /14）.Other orthostatic nervous impairments were cerebellar ataxia （4 /14）,conscious disturbance （2 /14）,hyper-myotonia （2 /14）,positive pathological reflex （1 /14）,personality changes （1 /14）and urine retention （1 /14）.Mag-netic resonance imaging （MRI）showed diffuse meningeal enhancement （10 /14）,subdural effusion（10 /14）,brain sagging （10 /14）,cerebral hernia （6 /14）,smaller angle between Galen vain and straight sinus （2 /14）and cerebral venous sinus thrombosis （1 /14）.Conclusion The clinical and radiographic manifestations of SIH are varied,and orthostatic feature is the most important factor leading to the diagnose.As the potential cause of SIH is spontaneous cerebrospinal fluid leak,conservative therapy,blood patch and surgery are effective management.