Recurrent painful ophthalmoplegic neuropathy

Deborah I Friedman MD MPH (

Dr. Friedman received honorariums from Alder BioPharmaceuticals, Allergan, Amgen/Novartis, Biohaven Pharmaceuticals, electroCore, Revance, Supernus, Teva, and Zosano  as an advisory board member and/or speaker; honorariums and grant support from Autonomic Technologies, Eli Lily, and Merck as clinical trial investigator; and honorariums from Promius as a consultant.

)
Stephen D Silberstein MD, editor. (

Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Allergan, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura,  INSYS Therapeutics, Lilly USA, Supernus Pharmacerticals,  Thernica and Trigemina for consulting. He is also the  principal investigator for a clinical trials conducted by Alder Biopharmaceuticals, Amgen, electroCore Medical, Lily USA and Teva.

)
Originally released August 22, 1995; last updated June 20, 2019; expires June 20, 2022

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-3) no longer considers the disorder a type of migraine and 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 were previously 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 Charcot first used the term "ophthalmoplegic migraine" in 1890. Gubler's patient subsequently died, and autopsy showed a fibrous mass embedding the third nerve. The patient had no history of migraine but was 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."

Prior to the modern imaging era, authors suggested that ophthalmoplegic migraine represented a congenital anomaly whereby the third nerve or, less commonly, the sixth nerve is perforated by an arteriole at the base of the brain (Imes et al 1984) or, perhaps, harbors an occult cavernous hemangioma or other occult vascular malformation. Walsh and O'Doherty cited 200 cases from the literature and postulated that edema of the wall of the carotid within the confines of the cavernous sinus causes pressure on the third nerve, thereby producing an oculomotor nerve palsy involving the pupil (Walsh and O'Doherty 1960; O'Day et al 1980). In addition to the cavernous sinus, the other potential location for vascular compression is the locus where the third nerve passes between the posterior cerebral and superior cerebellar arteries (Ehlers 1928; Morimoto et al 1985).

However, newer neuroimaging techniques (eg, CT, MRI, and angiography) secured the diagnosis, and the term "ophthalmoplegic migraine" was replaced by “recurrent painful ophthalmoplegic neuropathy,” a definable, albeit rare, clinical condition.

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