Clinical Review & Education

JAMA Pediatrics Clinical Challenge

Recurrent Right-Sided Ptosis in a Child Partha S. Ghosh, MD

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Figure. A, The patient’s right-sided ptosis during the first episode at age 18 months. B, Results of brain magnetic resonance imaging showing thickening of the cisternal part of the right third cranial nerve in the perimesencephalic cistern (white arrowhead).

A young boy presented for evaluation of “droopiness” of the eyelid. He had 3 episodes of right-sided ptosis, each of which occurred at 18 months, 3 years, and 5 years of age. None of these episodes was preceded by fever. During the last 2 episodes, he experienced severe right-sided headache lasting for 6 to 7 days before development of ptosis; he also experienced double vision. There was no history of loss of consciousness, Quiz at jamapediatrics.com seizures, loss of vision or hearing, facial deviation, trouble swallowing, or weakness of the extremities in any of these episodes. There was history of migraine on the maternal side of the family. There was no family history of autoimmune disorders or cerebral aneurysms. At the time of the clinic visit, the patient was asymptomatic and results of the neurologic examination were normal. The family provided a photograph of the patient’s face at the time of the first episode (Figure, A). After the first episode, with a presumed diagnosis of acute disseminated encephalomyelitis, he received intravenous pulse methylprednisolone sodium succinate, 30 mg/kg, for 3 days and recovered completely in 3 weeks. The patient received intravenous immunoglobulin, 2 g/kg, for 2 days after the subsequent 2 episodes, with a presumed diagnosis of chronic inflammatory demyelinating neuropathy, and recovered fully. Results of brain magnetic resonance imaging performed at 18 months showed thickening of the cisternal part of the right third cranial nerve in the perimesencephalic cistern (Figure, B) with no parenchymal lesions. Results of tests on cerebrospinal fluid collected via lumbar puncture were normal.

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WHAT IS YOUR DIAGNOSIS?

A. Recurrent meningitis B. Posterior communicating artery aneurysm C. Recurrent painful ophthalmoplegic neuropathy D. Myasthenia

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Clinical Review & Education JAMA Pediatrics Clinical Challenge

Diagnosis C. Recurrent painful ophthalmoplegic neuropathy

Discussion The child in this case presented with recurrent episodes of complete right-eye ptosis. He experienced headache followed by development of ptosis and diplopia in the last 2 episodes. The history is suggestive of recurrent right third cranial nerve palsy; associated fourth and sixth cranial nerve palsies cannot be completely excluded. The differential diagnosis of recurrent third nerve palsy is broad and includes cavernous sinus pathologic conditions (eg, Tolosa-Hunt syndrome, tumors), oculomotor nerve tumors, intracranial aneurysms, intracranial infections, diabetic ophthalmoparesis, myasthenia, and recurrent painful ophthalmoplegic neuropathy.1,2 Results of neuroimaging ruled out structural lesions such as tumors and aneurysms as well as cavernous sinus pathologic conditions. The presence of only intermittent right-sided symptoms during a 3-year period with no other ocular, bulbar, or limb weakness makes myasthenia unlikely. Complete ptosis with diplopia is not consistent with Horner syndrome. Results of tests on cerebrospinal fluid collected via lumbar puncture did not suggest intracranial infection. There is a considerable debate regarding the nomenclature of this entity. The International Classification of Headache Disorders, 3rd edition (beta version),3 defines this entity as recurrent painful ophthalmoplegic neuropathy. It was referred to in the past as ophthalmoplegic migraine.3 Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia.4,5 Ophthalmoplegic migraine is characterized by repeated attacks of paresis of 1 or more ocular cranial nerves (commonly the third), at least 2 attacks presenting with unilateral headache, and ARTICLE INFORMATION Author Affiliation: Department of Neurology, Boston Children’s Hospital, Boston, Massachusetts. Corresponding Author: Partha S. Ghosh, MD, Department of Neurology, Boston Children’s Hospital, 300 Longwood Ave, BCH 3213, Boston, MA 02115 ([email protected]). Section Editor: Samir S. Shah, MD, MSCE. Conflict of Interest Disclosures: None reported. REFERENCES 1. Ishikawa H, Yoshihara M, Mizuki K, Kashima Y. A pediatric case of ophthalmoplegic migraine with recurrent oculomotor nerve palsy. Jpn J Ophthalmol. 2000;44(5):576.

ipsilateral paresis of 1 or more ocular motor nerves.3 Orbital, parasellar, or posterior fossa lesion should be excluded by appropriate investigations. The onset of this entity is almost always during childhood, but there is growing evidence that it also occurs in adults.6 Although it is thought to be common in males, it can affect both sexes.7,8 The term ophthalmoplegic migraine is rejected in the recent classification because it is thought that this syndrome is not migrainous but rather a recurrent painful neuropathy.3 Another striking feature is that the headache can precede ophthalmoparesis by about 14 days. The ophthalmoparesis usually improves after most attacks but can persist longer after subsequent attacks.9 The patient in our case experienced unilateral headache with ipsilateral ptosis and diplopia in 2 of the 3 episodes; headache preceded ophthalmoparesis by 6 to 7 days followed by complete recovery. The pathophysiologic cause of this entity is unclear without a definite vascular, infective, or systemic inflammatory process accountable for these symptoms.5 A recurrent demyelinating cranial neuropathy has been proposed as a possible hypothesis for this condition,10 but it is unknown what triggers the demyelinating event. There is no evidence to suggest a systemic inflammatory process associated with this entity.5 A family history of migraine or other types of headache is not always present4; however, our patient had a family history of migraine. Neuroimaging should be performed to diagnose or exclude other structural causes of ophthalmoplegia.4 Results of contrastenhanced brain magnetic resonance imaging often reveal focal third-nerve enhancement.3-5 There are no published treatment trials for this entity. Treatment is based on anecdotal reports, with corticosteroid administration correlating with clinical response in some patients.3,5

3. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33(9): 629-808. 4. Margari L, Legrottaglie AR, Craig F, Petruzzelli MG, Procoli U, Dicuonzo F. Ophthalmoplegic migraine: migraine or oculomotor neuropathy? Cephalalgia. 2012;32(16):1208-1215. 5. Gelfand AA, Gelfand JM, Prabakhar P, Goadsby PJ. Ophthalmoplegic “migraine” or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review. J Child Neurol. 2012;27(6): 759-766. 6. Lal V. Ophthalmoplegic migraine: past, present and future. Neurol India. 2010;58(1):15-19.

7. Roy M, Ghosh J, Deb S, Pandit N. Childhood steroid-responsive ophthalmoplegic migraine. J Pediatr Neurosci. 2011;6(1):69-71. 8. Riadh H, Mohamed G, Salah Y, Fehmi T, Fafani BH. Pediatric case of ophthalmoplegic migraine with recurrent oculomotor nerve palsy. Can J Ophthalmol. 2010;45(6):643. 9. McMillan HJ, Keene DL, Jacob P, Humphreys P. Ophthalmoplegic migraine: inflammatory neuropathy with secondary migraine? Can J Neurol Sci. 2007;34(3):349-355. 10. Bharucha DX, Campbell TB, Valencia I, Hardison HH, Kothare SV. MRI findings in pediatric ophthalmoplegic migraine: a case report and literature review. Pediatr Neurol. 2007;37(1):59-63.

2. Bisdorff AR, Wildanger G. Oculomotor nerve schwannoma mimicking ophthalmoplegic migraine. Cephalalgia. 2006;26(9):1157-1159.

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Recurrent Right-Sided Ptosis in a Child. Recurrent Painful Ophthalmoplegic Neuropathy.

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