Biousse V, Bousser M G
Neuro-Ophthalmology Unit. Emory University School of Medicine. Atlanta, Georgia 30322, USA.
Rev Neurol (Paris). 2001 Jan;157(1):21-34.
Benign intracranial hypertension (BIH) is characterized by an elevation of the intracranial pressure not associated with an intracranial process or hydrocephaly, and with normal cerebrospinal fluid (CSF) contents. The elevation of the intracranial pressure is isolated; therefore, diseases such as cerebral venous thrombosis or dural fistulas should not be considered as etiologies of BIH. The exact definition of BIH remains debated, and other terms such as "pseudotumor cerebri" or "idiopathic intracranial hypertension" are often used in the literature. Although we agree that BIH is usually not a so benign disease, we suggest that BIH is still the most appropriate term to describe this entity which should be classified as "secondary BIH" or "idiopathic BIH" depending on whether there are precipitating factors for the development of BIH or not. We also propose new diagnostic criteria emphasizing the need for investigations for the diagnostic of secondary and idiopathic BIH. The management of patients with BIH depends mainly on the presence and severity of ocular symptoms and signs on which the prognostic of the disease is based. Repeated lumbar punctures associated with acetazolamide and weight loss are usually efficient enough. However a surgical treatment (optic nerve sheath fenestration or lumboperitoneal shunt) is required when appropriate medical management does not prevent progressive alteration of vision (visual loss or visual field defect), or when the patients complains of severe, refractory headaches. Careful follow-up with repeated formal visual field testing may help preventing a devastating visual loss in these patients.
良性颅内高压(BIH)的特征是颅内压升高,且与颅内病变或脑积水无关,脑脊液(CSF)成分正常。颅内压升高是孤立的;因此,诸如脑静脉血栓形成或硬脑膜瘘等疾病不应被视为BIH的病因。BIH的确切定义仍存在争议,文献中经常使用其他术语,如“假性脑瘤”或“特发性颅内高压”。尽管我们同意BIH通常并非如此良性的疾病,但我们认为BIH仍是描述该实体的最合适术语,应根据是否存在BIH发生的诱发因素将其分类为“继发性BIH”或“特发性BIH”。我们还提出了新的诊断标准,强调对继发性和特发性BIH进行诊断时进行检查的必要性。BIH患者的治疗主要取决于眼部症状和体征的存在及严重程度,而疾病的预后正是基于这些症状和体征。联合使用乙酰唑胺并减轻体重的反复腰椎穿刺通常足够有效。然而,当适当的药物治疗无法防止视力的进行性改变(视力丧失或视野缺损),或患者抱怨有严重的难治性头痛时,则需要进行手术治疗(视神经鞘开窗术或腰大池腹腔分流术)。通过反复进行正规视野测试进行仔细随访,可能有助于预防这些患者出现严重的视力丧失。