Schwartzmann Yoel, Moscovici Samuel, Ekstein Dana, Jubran Hamza, Ben-Hur Tamir, Kruger Joshua M
Department of Neurology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
The Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.
Eur J Neurol. 2025 Jun;32(6):e70139. doi: 10.1111/ene.70139.
Fulminant idiopathic intracranial hypertension (fIIH) is a severe condition which causes rapid visual loss. Conventional fIIH management involves surgical interventions, but their effectiveness and safety remain uncertain. The use of temporary continuous CSF drainage (CD) for fIIH has not been fully explored in the adult population. We present the results of CD treatment in severe IIH cases.
IIH Patients treated with CD were compared to control groups of extreme CSF opening pressure (> 45 cm HO) and random IIH patients (> 25 cm HO).
Eighteen female patients underwent CD treatment. Indications for CD treatment were acute visual deterioration or development of a VI nerve palsy and severe papilledema (despite acetazolamide treatment). All patients stabilized after CD treatment. Four patients had acute complications that fully resolved. Visual acuity (VA) in fIIH patients was significantly reduced prior to treatment (mean 6/15 ± 6/19 vs. 6/7.5 ± 6/30, p < 0.01). CD treatment resulted in marked long-term improvement in VA (mean 6/7.5 ± 6/19 vs. 6/7 ± 6/38, p = 0.12 and 6/7 ± 6/48, p = 0.05). Compared to the extreme CSF opening pressure group, female sex, obesity, and TVO's were more common in the CD group. Compared to the general IIH group, female sex, obesity, endocrinopathy, and CSF opening pressure were higher in the CD group.
IIH patients with very high CSF opening pressure and the presence of obesity and endocrinopathy may be at higher risk to develop fIIH. CD treatment is an extremely effective and safe treatment option for severe cases of IIH.
暴发性特发性颅内高压（fIIH）是一种导致快速视力丧失的严重病症。传统的fIIH治疗方法包括手术干预，但其有效性和安全性仍不确定。在成人患者中，临时持续脑脊液引流（CD）治疗fIIH的应用尚未得到充分研究。我们展示了严重IIH病例的CD治疗结果。
将接受CD治疗的IIH患者与脑脊液初压极高（>45 cm H₂O）的对照组和随机选取的IIH患者（>25 cm H₂O）进行比较。
18名女性患者接受了CD治疗。CD治疗的指征为急性视力恶化或出现动眼神经麻痹以及严重视乳头水肿（尽管已使用乙酰唑胺治疗）。所有患者在CD治疗后病情稳定。4例患者出现急性并发症，但均完全缓解。fIIH患者治疗前视力（VA）显著降低（平均6/15 ± 6/19 vs. 6/7.5 ± 6/30，p < 0.01）。CD治疗使VA得到显著长期改善（平均6/7.5 ± 6/19 vs. 6/7 ± 6/38，p = 0.12和6/7 ± 6/48，p = 0.05）。与脑脊液初压极高组相比，CD组女性、肥胖和静脉窦狭窄更为常见。与一般IIH组相比，CD组女性、肥胖、内分泌病和脑脊液初压更高。
脑脊液初压极高且存在肥胖和内分泌病的IIH患者发生fIIH的风险可能更高。CD治疗是严重IIH病例极其有效且安全的治疗选择。
