Typical Idiopathic Intracranial Hypertension: Optic Nerve Appearance and Brain MRI Findings

A 24-year old African American woman was referred for bilateral optic disc edema that was incidentally noted on a routine eye examination. She had excellent visual function and dilated examination showed bilateral optic disc edema with peripapillary wrinkles in the right eye and pseudodrusen in the left eye (Figure 1). Optical coherence tomography of the optic nerve demonstrated the peripapillary wrinkles in the inner retina (Figure 2). Humphrey 24-2 SITA-Fast visual fields were normal (Figure 3). MRI and MRV of the with contrast were performed (Figures 4 and 5) and showed a partially empty sella (Figure 6), caudal displacement of the cerebellar tonsils (Figure 7), flattening of the posterior globes (Figure 8), distention of the CSF spaces around the optic nerves (Figure 9), and narrowing of the distal transverse sinuses (Figure 10), consistent with intracranial hypertension. The patient had a lumbar puncture, which confirmed an elevated opening pressure of 39 cm of water with normal cerebrospinal fluid contents leading to the diagnosis of idiopathic intracranial hypertension. Treatment with acetazolamide and weight loss was initiated and she was seen in follow-up at 3 months, 6 months, and 1 year after her initial visit. Her optic disc edema gradually resolved (Figure 11) and she continued to maintain excellent visual function (Figure 12). [[Number of Figures and legend for each: 12 figures included. Figure 1. Optic disc photos of both eyes demonstrating bilateral optic disc edema with peripapillary wrinkles in the right eye and pseudodrusen in the left eye. Figure 2. Spectralis optic coherence tomography of the optic nerve shows peripapillary wrinkles on the surface of the inner retina of the right eye. Figure 3. Humphrey 24-2 SITA-Fast visual fields. Figure 4. Sagittal T1, axial T2 FLAIR, and coronal T2 MRI images. Figure 5. MRV of the head with contrast. Figure 6. Sagittal T1 MRI shows a partially empty sella. A normal appearing sella is shown for reference. Figure 7. Sagittal T1 MRI shows caudal displacement of the cerebellar tonsils. A normal appearing sagittal T1 MRI is shown for comparison. Figure 8. Axial FLAIR MRI shows flattening of the posterior globes. Normally appearing globes are included for comparison. Figure 9. Coronal T2 MRI shows distention of the CSF spaces around the optic nerves. Normal appearing optic nerves and surrounding CSF spaces are shown for comparison. Figure 10. MRV of the brain shows narrowing of the distal transverse sinuses. Figure 11. Optic disc photos at follow-up visits show resolution of the optic disc edema. Figure 12. Optic disc photos are shown with accompanying pattern deviation from the 24-2 SITA-Fast Humphrey visual fields. She maintained excellent visual function at each follow-up visit. ]]