脳脊髄液漏出症の疾患概念と診療方針の現状

Spinal Cerebrospinal Fluid Leak: Current Concepts and Management Policies

Point

・脳脊髄液漏出症は特発性低髄液圧症候群のみならず，難治性むち打ち損傷関連障害（WAD），小児若年者の起立性調節障害（OD）から体位性頻脈症候群（POTS）などを含む幅広い病態である．

・診断では，脊髄MRIによるスクリーニング，硬膜外持続注入による症状改善の確認，CT脊髄造影検査による診断確定の順に進むのが効率的である．

・従来知られていた髄液漏出部位に加えて，椎骨動脈硬膜貫通部からの漏出の可能性を念頭に診断・治療にあたる必要がある．

　In this article we provide an update on the recent developments in understanding the diagnosis and treatment of spinal cerebrospinal fluid（CSF）leaks. Patients with spinal CSF leak show an increasingly broad spectrum of clinical presentations including intractable whiplash-associated disorder（WAD）and orthostatic dysregulation in childhood, in addition to classical spontaneous intracranial hypotension（SIH）. A simple understanding of the condition defined by the presence of low CSF pressure is no longer sufficient or accurate. Fat-suppressed T2-weighted magnetic resonance images and continuous epidural fluid infusion as a diagnostic therapy ensure accurate diagnosis. Accurate localization of the leak followed by targeted epidural blood patch（EBP）significantly improves the outcome of patients with spinal CSF leak. In addition to the known sites of CSF leakage, a newly identified CSF leak site, at the craniovertebral junction dural entry point of the vertebral artery（VA）, is described.