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.

摘要 背景：自发性颅内低压（spontaneous intracranial hypotension, SIH）是一种继发性头痛病因，且属于易被漏诊的疾病。其临床表现多样多变，典型表现为孤立性典型体位性头痛，但患者也可能出现严重并发症，如脑静脉血栓形成（cerebral venous thrombosis, CVT）。研究目的：报告3例在三级神经内科病房收治并接受治疗的自发性颅内低压病例。研究方法：回顾3例患者的病历资料，描述其临床与手术转归。研究结果：3例患者均为女性，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出现嗜睡与复视。头颅磁共振成像（magnetic resonance imaging, MRI）表现不一，可从正常影像学表现至自发性颅内低压的典型征象，包括硬脑膜强化与小脑扁桃体下疝。脊柱磁共振成像均显示硬膜外异常积液，而计算机断层扫描（computed tomography, CT）脊髓造影仅在1例患者中明确检出脑脊液（cerebrospinal fluid, CSF）漏口。1例患者接受保守治疗，其余2例行椎板成形开放手术，术后均顺利恢复，随访期间症状完全缓解。研究结论：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。本研究重点报道了致残性严重自发性颅内低压病例及其并发脑静脉血栓形成的情况，并展示了神经外科治疗的良好预后。