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REVIEW ARTICLE
Year : 2021  |  Volume : 11  |  Issue : 1  |  Page : 25-38

Atypical presentations of idiopathic intracranial hypertension


1 Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA, United States; Department of Neurology, Auckland City Hospital, Auckland, New Zealand
2 Department of Ophthalmology; Department of Neurology; Department of Neurological Surgery, Emory University School of Medicine, Atlanta, GA, United States
3 Department of Ophthalmology; Department of Neurology, Emory University School of Medicine, Atlanta, GA, United States

Correspondence Address:
Dr. Benson S Chen
Neuro-Ophthalmology Unit, Emory Eye Center, 1365-B Clifton Road Ne, Atlanta, GA 30322

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjo.tjo_69_20

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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.


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