Commentary

Neuro-Ophthalmology: No Longer Diagnose and Adios


 

Deborah I. Friedman, MD, MPH

Dr. Friedman is a Professor in the Departments of Neurology and Ophthalmology at the University of Texas Southwestern Medical Center in Dallas.

Neuro-ophthalmology is a small subspecialty that had its origins in ophthalmology but has always included neurologists. National neuro-ophthalmology meetings in North America began with the Rocky Mountain Neuro-Ophthalmology Course in 1975, which led to the formation of the Rocky Mountain Neuro-Ophthalmology Society in 1980. By 1986, it had 240 members and changed its name to the North American Neuro-Ophthalmology Society (NANOS). Already existing was the 1.5-day annual Frank Walsh Society meeting with its clinical-pathological correlation format, which originated at Johns Hopkins Medical School. The two societies merged in 1992. Currently, NANOS has grown to more than 650 members and is the premier organization for neuro-ophthalmology worldwide. The society’s journal, the Journal of Neuro-Ophthalmology, commenced publication in 1994, one year after the inaugural issue of Neurology Reviews.

Deborah I. Friedman, MD, MPH

Overcoming an early reputation for “admiring” disease, neuro-ophthalmology has evolved considerably over the past 25 years, with advances in neuroimaging, genomics, proteomics, office-based technologies, and immunology. The first multicenter, randomized trial in neuro-ophthalmology—the Optic Neuritis Treatment Trial (ONTT),1 published 25 years ago—showed that intravenous methylprednisolone 250 mg QID for three days followed by an 11-day prednisone taper was superior to either oral prednisone (1 mg/kg for 14 days) or oral placebo (14 days), speeding the recovery of vision with slightly better vision at six months. There was a higher rate of relapse in the group assigned to receive oral prednisone. The ONTT was a pivotal event for neuro-ophthalmology, heralding a paradigm shift from observational reporting to randomized clinical trials.

Three years later, the Ischemic Optic Neuropathy Decompression Trial (IONDT)2 showed that optic nerve sheath fenestration surgery, performed within 14 days of symptom onset for acute non-arteritic ischemic optic neuropathy or within 30 days in participants with a progressive course, did not improve visual acuity or visual field compared to careful follow-up and medical management at six months. Enrollees randomized to undergo surgery had a lower rate of improvement and were more likely to lose three or more lines of vision than the control group.

With this momentum, the Neuro-Ophthalmology Research Disease Investigators Consortium was formed to create infrastructure for future clinical trials of neuro-ophthalmic disorders. The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT)3 studied patients with idiopathic intracranial hypertension and mild visual field loss. IIHTT confirmed that acetazolamide (500 mg BID, increasing to a maximum dose of 4 grams daily) was superior to placebo treatment when combined with a supervised weight loss program, resulting in improved visual field loss, papilledema grade, weight reduction, lumbar puncture opening pressure, and quality of life. Ongoing research efforts are investigating treatments for Leber hereditary optic neuropathy, non-arteritic ischemic optic neuropathy, thyroid eye disease, rehabilitation for poststroke homonymous hemianopia and giant cell arteritis in adults, and idiopathic intracranial hypertension and optic nerve gliomas in children.

Attendance at the NANOS annual meeting continues to grow, emphasizing scientific as well as clinical neuro-ophthalmic topics. Vibrant and active neuro-ophthalmology societies in other parts of the world (eg, Europe, Japan, Australia, British Isles) contribute to the camaraderie, collegiality, collaboration, and discovery that advance our field.

Happy 25th anniversary to Neurology Reviews! The next 25 years will likely transform the field of neuro-ophthalmology as we better understand the molecular and genetic basis of neuro-ophthalmic disorders. No longer “diagnose and adios,” we anticipate significant therapeutic advancements—and perhaps cures—for individuals with severe, sight-impairing, neuro-ophthalmic conditions.

References

1. Beck RW, Clearly PA, Anderson MM Jr, et al. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med. 1992;326(9):581-588.

2. The Ischemic Optic Neuropathy Decompression Trial Research Group. Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. JAMA. 1995;273(8):625-632.

3. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss. JAMA. 2014;311(16):1641-1651.

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