Ophthalmoplegic “Migraine” or Recurrent Ophthalmoplegic Cranial Neuropathy

Abstract

Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia. By reviewing cases presenting to our centers in whom the phenotype has been carefully dissected, and systematically reviewing all published cases of ophthalmoplegic migraine in the magnetic resonance imaging (MRI) era, this review sets out to clearly define the syndrome and discuss possible etiologies. We found that in up to one-third of patients, the headache was not migrainous or associated with migrainous symptoms. In three-quarters of the cases involving the third nerve, there was focal nerve thickening and contrast enhancement on MRI. Observational data suggest systemic corticosteroids may be beneficial acutely. The etiology remains unclear, but may involve recurrent bouts of demyelination of the oculomotor nerve. “Ophthalmoplegic migraine” is a misnomer in that it is probably not a variant of migraine but rather a recurrent cranial neuralgia. A more appropriate name might be “ophthalmoplegic cranial neuropathy.”

Magnetic Resonance Findings in Recurrent Painful Ophthalmoplegic Neuropathy with Reversible Enhancement of Oculomotor Nerve

“…This clinical condition should not be attributed to another disorder such as tumor, infection, and aneurysm. Thrombosis must have been excluded by appropriate imaging (1,5). Recurrent painful ophthalmoplegic neuropathy is a diagnosis of exclusion.…”

“…Nevertheless, oral steroids may be of possible benefit in treating acute exacerbations based on available observational data. Because recurrent painful ophthalmoplegic neuropathy is not a variant of migraine, migrainespecific acute and preventive therapies probably do not have a role in treatment (5). Contrast-enhanced MRI is a useful adjunct in the diagnosis and monitoring response to treatment of recurrent painful ophthalmoplegic neuropathy with oculomotor nerve involvement.…”

“…The disorder usually presents in children, but may also effect young adults. The episodes of ophthalmoplegia may persist for several hours to several weeks, months or in rare cases may be constant (1)(2)(3)(4)(5)(6)(7). Because of the self-limiting and reversible nature of this disease, the pathophysiology of this condition is still unclear although there is current evidence to suggest that it is a demyelinating process (1,2,5).…”

Approach to a patient with blepharoptosis

“…It is still debated whether an uncontrolled migraine or an oculomotor neuropathy is the primary cause of OM. Some authors suggested the name of ''recurrent painful oculomotor neuropathy'' and suggested recurrent demyelination as the presumed etiology [41]. In three quarters of the Fig.…”

Management of migraine in children

“…It has recently been proposed that this is not in fact a migraine but instead a demyelinating disorder. 13 Benign paroxysmal vertigo is usually a toddler affliction, and typical attacks are characterised by suddenonset ataxia causing immobility and falls. The affected child looks frightened and pale and may vomit.…”