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Images in Neurology |

Fulminant Idiopathic Intracranial Hypertension

Aasef G. Shaikh, MD, PhD1; James H. Bates, MD1,2; Scott W. Yeates, MD2; Bashar Katirji, MD1; Michael W. Devereaux, MD1
[+] Author Affiliations
1Department of Neurology, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, Ohio
2Department of Ophthalmology, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, Ohio
JAMA Neurol. 2013;70(7):937-938. doi:10.1001/jamaneurol.2013.1951.
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Fulminant idiopathic intracranial hypertension is extremely rare but invariably is associated with permanent blindness unless there is prompt intervention. Hypothetically, a rapid increase in pressure in the perineural space around the optic nerve can lead to ischemic optic neuropathy and blindness.1 An 18-year-old overweight woman with rapidly progressive visual loss over 4 days (leading to no light perception), nonreactive pupils, binocular abduction deficit suggestive of sixth nerve palsies, bilateral optic-disc edema (Frisén stage 5),2 and cerebrospinal fluid opening pressure at 600 mm H2O received a diagnosis of fulminant idiopathic intracranial hypertension.1 The results of cerebrospinal fluid laboratory studies, magnetic resonance imaging of the brain, and vascular imaging were normal. Our Figure, A and B, shows magnetic resonance imaging scans of the patient’s orbits at the time of presentation.

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Figure.
Magnetic Resonance Imaging and Fundus Photographs of an 18-Year-Old Woman With Fulminant Idiopathic Intracranial Hypertension

Magnetic resonance imaging (MRI) scans of the orbits (A-D) and fundus photographs of the eyes (E and F) of an 18-year-old woman with fulminant idiopathic intracranial hypertension. Before treatment (day 1), T2-weighted MRI (A) demonstrated a distended perioptic nerve sheath (white arrows), protrusion of the papillae (black arrows), and a “strangled” optic nerve proximal to the optic disc in both eyes, although it was worse in the left eye (white arrowhead). Optic disc edema (arrows) and retinal layer edema (arrowheads) were prominent on a gadolinium-enhanced MRI scan (B). After the placement of an emergent, controlled lumbar drain and treatment with intravenous steroids and furosemide (day 5), T2-weighted (C [arrows]) and gadolinium-enhanced (D) MRI scans show the near-complete resolution of the distended perioptic nerve sheath, papillary protrusion, and impingement of the optic nerve. This finding clinically correlated with improved visual acuity and papilledema. These findings support the hypothesis that increased perineural-space pressure leads to compression-induced ischemic optic neuropathy, axoplasmic stasis, and subsequently progressive visual loss. Prompt decompression of the cerebrospinal fluid pressure and timely relief of the perineural pressure could reverse ischemic optic neuropathy and prevent permanent blindness. Fundus photographs (E and F) show Frisén stage 2 papilledema with moderate disc pallor on day 9.2

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