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JAMA Pediatrics Clinical Challenge
April 2017

A 15-Year-Old Girl With Unexplained Intracranial Hypertension

Author Affiliations
  • 1Department of Ophthalmology and Visual Sciences, Yale School of Medicine, New Haven, Connecticut
  • 2Department of Neurology, Yale School of Medicine, New Haven, Connecticut
JAMA Pediatr. 2017;171(4):389-390. doi:10.1001/jamapediatrics.2016.4129

A white, nonobese, 15-year-old girl presented with 1 month of frontal and occipital throbbing headaches, tinnitus, and “whooshing” in the ears. She had 1 week of nonbilious, nonbloody emesis, and “graying out” of her vision only when standing up or sitting down. Examination noted normal visual acuities and swelling of both optic discs (Figure, A). Magnetic resonance imaging and magnetic resonance venography of the head showed no intracranial mass or venous sinus thrombosis. Lumbar puncture showed an opening pressure of 550 mm of water and normal cerebrospinal fluid (CSF) composition. A diagnosis of idiopathic intracranial hypertension was made, and she was treated with acetazolamide. Headaches initially improved but worsened during the following weeks. Three months after initial presentation, she reported a 9.07-kg weight loss (height 1.57 m; body mass index dropped from 21.3 to 16.8 [calculated as weight in kilograms divided by height in meters squared]), postural lightheadedness, worsening fatigue, muscle weakness, salt cravings, loss of axillary hair, and missed periods for 2 months. Physical examination was remarkable for ill appearance, blood pressure of 85/42 mm Hg, and hyperpigmentation of the knuckles, distal fingers, and tongue (Figure, B and C). Repeated ophthalmic examination showed normal visual acuities and worsening optic disc swelling.