Recurrent painful ophthalmoplegic neuropathy

Deborah I Friedman MD MPH (Dr. Friedman of the University of Texas Southwestern Medical Center has no relevant financial relationships to disclose.)
Stephen D Silberstein MD, editor. (Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Alder Biopharmaceuticals, Allergan, Amgen, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura, electroCore Medical, Lilly USA, Supernus Pharmacerticals, Teva, and Trigemina for consulting and/or advisory panel membership.)
Originally released August 22, 1995; last updated October 23, 2016; expires October 23, 2019

This article includes discussion of recurrent painful ophthalmoplegic neuropathy, ophthalmoplegic migraine, and RPON. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Recurrent painful ophthalmoplegic neuropathy, formerly termed “ophthalmoplegic migraine,” is a fascinating and controversial entity. The International Classification of Headache Disorders (ICHD-2) 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.

Key points

 

• The International Classification of Headache Disorders version 3-beta classifies recurrent painful ophthalmoplegic neuropathy as a cranial neuralgia.

 

• Although a clinical diagnosis, recurrent painful ophthalmoplegic neuropathy may show enhancement of the involved cranial nerve on post-gadolinium magnetic resonance imaging.

 

• The third cranial nerve is the most frequently involved nerve in recurrent painful ophthalmoplegic neuropathy.

 

• Most of the cases start in childhood or early adulthood.

 

• Episodes of recurrent painful ophthalmoplegic neuropathy appear spontaneously and then resolve without treatment.

 

• Ocular motor nerve schwannomas may produce identical symptoms and resolve spontaneously.

 

• The etiology for recurrent painful ophthalmoplegic neuropathy is controversial and likely multifactorial, and although a number of treatments have been proposed (eg, steroids, anti-migraine agents), none have proven to be a consistently effective therapy.

Historical note and terminology

Recurrent ocular motor palsies associated with a headache have been referred to as ophthalmoplegic migraine. The oculomotor (third) nerve is the one most commonly affected, but trochlear (fourth) or abducens (sixth) nerve palsies may occur. The original 1860 report was by Gubler (Gubler 1860; Charcot 1890), but the term "ophthalmoplegic migraine" was first used by Charcot in 1890. Gubler's patient subsequently died, and autopsy showed a fibrous mass in which the third nerve was embedded. The patient had no history of migraine, but had been afflicted with syphilis 10 years earlier. Gowers believed that diplopia was a rare symptom of migraine, and Liveing only briefly alluded to diplopia in his monograph (Liveing 1873; Gowers 1888). Prior to Charcot's use of the expression "ophthalmoplegic migraine," other synonymous terms included "recurring ocular palsy," "periodic oculomotor paresis," and "recurring third nerve palsy." The advent of newer neuroimaging studies (eg, CT, MRI, and angiography) has made the diagnosis more secure, and the term "ophthalmoplegic migraine" has been replaced by “recurrent painful ophthalmoplegic neuropathy,” a definable, albeit rare, clinical condition.

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