Chen Benson S, Newman Nancy J, Biousse Valérie
Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA, United States.
Department of Neurology, Auckland City Hospital, Auckland, New Zealand.
Taiwan J Ophthalmol. 2020 Dec 2;11(1):25-38. doi: 10.4103/tjo.tjo_69_20. eCollection 2021 Jan-Mar.
Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.
特发性颅内高压（IIH）是一种病因不明的疾病，可导致孤立性颅内压升高。IIH的典型症状和体征包括头痛、视乳头水肿、因第六神经麻痹和散开功能不全导致的复视以及搏动性耳鸣。非典型表现包括：（1）高度不对称甚至单侧视乳头水肿，以及无视乳头水肿的IIH；（2）因第三神经麻痹、第四神经麻痹、核间性眼肌麻痹、弥漫性眼肌麻痹和斜视导致的眼球运动障碍；（3）嗅觉功能障碍；（4）三叉神经功能障碍；（5）面神经功能障碍；（6）听力丧失和前庭功能障碍；（7）下颅神经功能障碍，包括悬雍垂偏斜、斜颈和舌肌无力；（8）自发性颅底脑脊液漏；以及（9）癫痫发作。尽管非典型发现应引起警惕并促使进一步调查其他病因，但临床医生应熟悉这些不寻常的表现。
