Sheffield Teaching Hospitals NHS Trust, University of Sheffield, Sheffield, UK.
Acta Neurol Scand. 2010 Feb;121(2):71-82. doi: 10.1111/j.1600-0404.2009.01172.x. Epub 2009 Nov 23.
Idiopathic intracranial hypertension (IIH) is a condition which affects predominantly overweight women and is characterized by raised intracranial pressure without any identifiable pathology in the brain and with normal cerebrospinal fluid (CSF) composition. The cause of IIH is unclear and as such it remains a diagnosis of exclusion. Although the pathophysiology of IIH remains elusive, some observations have recently been added to our understanding of this, including the presence of transverse sinus stenosis on many patients and the possible role of leptin and inflammation in the disease pathogenesis. Headache is the most common symptom and papilloedema is the major clinical finding. Choices of medical treatment are limited to the use of diuretics particularly acetazolamide and encouragement of weight loss. Surgical therapies such as CSF diversion procedures and fenestration of the optic nerve may be necessary in some cases with persistent symptoms or progressive visual deterioration. While not life-threatening, IIH has a significant morbidity with up to 25% of patients developing visual impairment from optic atrophy. Visual surveillance is therefore vital. Long-term follow-up is recommended as the disease may worsen after an initial period of stability.
特发性颅内高压(IIH)主要影响超重女性,其特征是颅内压升高,而大脑无明显病变,脑脊液(CSF)成分正常。IIH 的病因尚不清楚,因此仍然是一种排除性诊断。尽管 IIH 的病理生理学仍然难以捉摸,但最近我们对该病的理解又有了一些新的认识,包括许多患者横窦狭窄的存在以及瘦素和炎症在疾病发病机制中的可能作用。头痛是最常见的症状,视盘水肿是主要的临床发现。药物治疗的选择仅限于使用利尿剂,特别是乙酰唑胺,并鼓励减肥。对于持续存在症状或视力进行性恶化的某些病例,可能需要手术治疗,如 CSF 分流术和视神经开窗术。虽然 IIH 不会危及生命,但它有很高的发病率,多达 25%的患者会因视神经萎缩而出现视力损害。因此,视觉监测至关重要。建议进行长期随访,因为在初始稳定期后,疾病可能会恶化。