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Neuro-ophthalmology is an academically-oriented subspecialty that merges the fields of neurology and ophthalmology, often dealing with complex systemic diseases that have manifestations in the visual system. Neuro-ophthalmologists initially complete a residency in either neurology or ophthalmology, then do a fellowship in the complementary field. Since diagnostic studies can be normal in patients with significant neuro-ophthalmic disease, a detailed medical history and physical exam is essential and neuro-ophthalmologists often spend significantly more time with the patient than specialists in other disciplines.
Common pathology referred to a neuro-ophthalmologist includes afferent visual system disorders (e.g. optic neuritis, optic neuropathy, papilledema, brain tumors or strokes) and efferent visual system disorders (e.g. anisocoria, diplopia, ophthalmoplegia, ptosis, blepharospasm and hemifacial spasm). The largest international society of neuro-ophthalmologists is the North American Neuro-Ophthalmological Society (NANOS), which organizes an annual meeting and publishes the Journal of Neuro-Ophthalmology.
Neuro-ophthalmology focuses on diseases of the nervous system that affect vision, control of eye movements, or pupillary reflexes. Neuro-ophthalmologists often see patients with complex multi-system disease and “zebras” are not uncommon. Neuro-ophthalmologists are often faculty at large university-based medical centers, typically in the ophthalmology department but may also be housed in other departments or be in private practice. Patients often have co-existing disease in other fields (rheumatology, endocrinology, oncology, cardiology, etc.), thus the neuro-ophthalmologist is usually a liaison between the ophthalmology department and other departments in the medical center.
A neuro-ophthalmologist’s office is filled with patients who have been misdiagnosed or incorrectly diagnosed and drag literally pounds of diagnostic studies, which often reiterate that neuroimaging is normal, incorrectly performed, or incorrectly interpreted in many neuro-ophthalmologic disorders. Neuro-ophthalmologists are often active teachers in their academic institution, and the first four winners of the prestigious Straatsma American Academy of Ophthalmology teaching awards were neuro-ophthalmologists. Most neuro-ophthalmologists are passionate about their discipline and report high job satisfaction, stating that they think the field continues to be both fascinating and challenging.
Neuro-ophthalmology is mostly non-procedural, however, neuro-ophthalmologists may be trained to perform eye muscle surgery to treat adult strabismus, optic nerve fenestration for idiopathic intracranial hypertension, and botulinum injections for blepharospasm or hemifacial spasm.
Frank B. Walsh was one a pioneer of neuro-ophthalmology, helping to popularize and develop the field. Walsh was born in Oxbow, Saskatchewan in 1895 and earned a degree for University of Manitoba in 1921. He joined the Wilmer Ophthalmological Institute at Johns Hopkins University and began organizing Saturday morning neuro-ophthalmology conferences. Walsh compiled the first neuro-ophthalmology textbook, which was published in 1947 and has been updated over the years by generations of his students.
Ophthalmologists have been decreasing the time spent with a patient due to economic pressures, the use of nonphysicians, and increasing reliance on laboratory tests. Neuro-ophthalmology has been affected moreso than other specialties due to the complexity of the patients and the time required to do a neuro-ophthalmic history and physical exam. Additionally, the current medical reimbursement system rewards quantity of service (performing assembly line procedures) rather than quality of service (making a correct diagnosis, patient education, and counseling), and seeing complex patients is not adequately recognized.
Improved functional neuroimaging is paving the way for better understanding, assessment, and management of many neurologic and neuro-ophthalmologic conditions. As our understanding of neuroscience evolves, neuro-ophthalmologists are becoming increasingly better at treatment, rather than only diagnosis, and novel therapies are emerging to treat traditionally vision-devastating disease. For example, clinical trials began in February 2014 to use gene therapy to treat Leber hereditary optic neuropathy, which is one of the first uses of gene therapy in the central nervous system. Progress has also been made in understanding retinal ganglion cell regeneration and in re-establishing synaptic connections from the optic nerve to the brain, more than in other regions of the central nervous system. One of the goals of the National Institutes of Health is to use the visual system as a window to understand neural plasticity and regenerative medicine in the central nervous system, an area of neuroscience that has a promising future and is intimately intertwined with neuro-ophthalmology.
The weakening financial environment for academic neuro-ophthalmologists must be addressed so that there is the clinical infrastructure to treat patients, assess and implement emerging technologies and treatments, and train the next generation of neuro-ophthalmologists. Data is needed to quantify the problem (the revenue provided to other departments, the amount of money wasted on unnecessary tests, visits, and procedures before seeing a neuro-ophthalmologist, the average time a patient spends with the neuro-ophthalmologist, etc), and given the direction of ophthalmic and neurologic research, it is imperative to continue to have a vibrant academic neuro-ophthalmologic community for the future.