In this article, Dr. Deborah Friedman of the University of Texas Southwestern Medical Center discusses the fascinating and controversial entity of recurrent painful ophthalmoplegic neuropathy, formerly termed “ophthalmoplegic migraine.” The International Classification of Headache Disorders (ICHD-3 beta+) no longer considers the disorder a type of migraine but has reclassified it as a cranial neuralgia.
Although many cases are typical of a cranial neuralgia, with enhancement of the involved cranial nerve on MRI and improvement using corticosteroids, other cases are more suggestive of a migraine variant. The third nerve is the nerve most frequently involved, and most cases appear in childhood or early adulthood. The episodes of ophthalmoplegia appear spontaneously and then resolve. As the etiology for recurrent painful ophthalmoplegic neuropathy is likely multifactorial and remains uncertain, the condition may more appropriately be considered a syndrome rather than a distinct diagnosis.
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