Spontaneous intracranial hypotension: from cerebral venous thrombosis to neurosurgical treatment: a case series experience from a Brazilian tertiary health care center

Abstract Background Spontaneous intracranial hypotension (SIH) is a secondary cause of headache and an underdiagnosed disease. The clinical presentation can be highly variable. It typically presents with isolated classic orthostatic headache complaints, but patients can develop significant complications such as cerebral venous thrombosis (CVT). Objective To report 3 cases of SIH diagnosis admitted and treated in a tertiary-level neurology ward. Methods Review of the medical files of three patients and description of clinical and surgical outcomes. Results Three female patients with SIH with a mean age of 25.6 ± 10.0 years old. The patients had orthostatic headache, and one of them presented with somnolence and diplopia because of a CVT. Brain magnetic resonance imaging (MRI) ranges from normal findings to classic findings of SIH as pachymeningeal enhancement and downward displacement of the cerebellar tonsils. Spine MRI showed abnormal epidural fluid collections in all cases, and computed tomography (CT) myelography could determine an identifiable cerebrospinal fluid (CSF) leak in only one patient. One patient received a conservative approach, and the other two were submitted to open surgery with lamino-plasty. Both of them had uneventful recovery and remission in surgery follow-up. Conclusion The diagnosis and management of SIH are still a challenge in neurology practice. We highlight in the present study severe cases of incapacitating SIH, complication with CVT, and good outcomes with neurosurgical treatment.

背景：自发性颅内低压（Spontaneous Intracranial Hypotension, SIH）是引发头痛的继发性病因，亦是一种诊断不足的疾病。其临床表现具有高度异质性，典型表现为孤立性体位性头痛，但患者可并发脑静脉血栓形成（Cerebral Venous Thrombosis, CVT）等严重并发症。
目的：报告3例在三级医院神经内科病房收治并接受治疗的自发性颅内低压病例。
方法：回顾3例患者的病历资料，对其临床与手术结局进行描述。
结果：本研究纳入3例女性自发性颅内低压患者，平均年龄为25.6±10.0岁。所有患者均出现体位性头痛，其中1例因并发脑静脉血栓形成而出现嗜睡与复视。脑部磁共振成像（Magnetic Resonance Imaging, MRI）表现各异，可表现为正常，亦可出现自发性颅内低压的典型征象：硬脑膜强化与小脑扁桃体下疝。脊柱磁共振成像（spine MRI）显示所有患者均存在异常硬膜外积液，而计算机断层脊髓造影（Computed Tomography Myelography, CT脊髓造影）仅在1例患者中明确定位了脑脊液漏（cerebrospinal fluid leak, CSF）位点。1例患者接受保守治疗，其余2例接受椎板成形开放手术治疗，二者术后随访均顺利康复，症状完全缓解。
结论：自发性颅内低压的诊断与治疗仍是神经内科临床实践中的一大挑战。本研究着重报告了致残性自发性颅内低压并发脑静脉血栓形成的重症病例，以及神经外科治疗取得的良好预后。