Qiao Lei, Wei Yanping
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Front Neurol. 2020 Nov 26;11:569432. doi: 10.3389/fneur.2020.569432. eCollection 2020.
Familial idiopathic intracranial hypertension (FIIH) is a rare condition, the etiology of which is unclear. To describe two non-obese Chinese sisters who met the criteria of FIIH and to analyze the clinical features and prognosis of FIIH. The clinical course, treatment, and prognosis of these two patients were analyzed retrospectively. Meanwhile, all the literature of familial IIH (FIIH) was reviewed. These two sisters presented with headaches and visual impairment in their mid-thirties. Magnetic resonance imaging (MRI) of the brain was unremarkable except for partial empty sella. No comorbidities or defined causes were detected. Headaches were partially relieved by dehydrated medicine, whereas the visual impairment persisted. In cases where patients present with headaches, empty sella are found on an MRI, and there is visual impairment with or without papilla edema, intracranial hypertension should be excluded. Furthermore, we should pay more attention to the relatives of those patients with increased intracranial hypertension.
家族性特发性颅内高压(FIIH)是一种罕见疾病,其病因尚不清楚。描述两名符合FIIH标准的非肥胖中国姐妹,并分析FIIH的临床特征和预后。对这两名患者的临床病程、治疗及预后进行回顾性分析。同时,对家族性IIH(FIIH)的所有文献进行综述。这两名姐妹在三十多岁时出现头痛和视力障碍。脑部磁共振成像(MRI)除部分空蝶鞍外无明显异常。未检测到合并症或明确病因。脱水药物可部分缓解头痛,但视力障碍持续存在。对于出现头痛、MRI发现空蝶鞍、伴有或不伴有视乳头水肿的视力障碍患者,应排除颅内高压。此外,我们应更加关注颅内压升高患者的亲属。