Magnetic resonance imaging findings in ophthalmoplegic migraine
A Borade, AS Prabhu, S Kumar, V Prasad, L Rajam
Department of Pediatrics, Amrita Institute of Medical Sciences, Elamakkara (P.O.), Kochi, Kerala - 682 026, India
Department of Pediatrics, Amrita Institute of Medical Sciences, Elamakkara (P.O.), Kochi, Kerala - 682 026
|How to cite this article:|
Borade A, Prabhu A S, Kumar S, Prasad V, Rajam L. Magnetic resonance imaging findings in ophthalmoplegic migraine.J Postgrad Med 2009;55:137-138
|How to cite this URL:|
Borade A, Prabhu A S, Kumar S, Prasad V, Rajam L. Magnetic resonance imaging findings in ophthalmoplegic migraine. J Postgrad Med [serial online] 2009 [cited 2020 Jan 24 ];55:137-138
Available from: http://www.jpgmonline.com/text.asp?2009/55/2/137/52848
Ophthalmoplegic migraine (OPM) is a rare variant of migraine seen most commonly in children and presents with headache and palsy of third, fourth or sixth cranial nerve. Episodic ophthalmoplegia associated with vomiting and irritability is the prominent presenting manifestations. Headache is an inconsistent feature.  It is associated with magnetic resonance imaging (MRI) findings, which can be confused with TB or malignancy. 
We have under our care a 6-year-old female child who presented with complaints of recurrent episodes of ptosis of the right eye and diplopia in all directions for the last 14 months. Each episode started as a bilateral throbbing headache associated with photophobia and intolerance to loud sounds. Vomiting followed, after which she developed ptosis on the right side. Over the next 2-3 days, she complained of diplopia in all directions that coincided with relief of the headache. Her symptoms improved slowly over the next 3-4 weeks. The asymptomatic interval between episodes was six and eight months. She presented to us shortly after onset of the third episode. There was no family history of migraine or tuberculosis. Neurological evaluation showed mild ptosis with adductor restriction of the right eye [Figure 1] but a normal papillary reaction bilaterally. Computed tomography brain which had been performed during the 1 st episode was normal. Her baseline blood investigations showed a normal total leucocyte count with a normal erythrocyte sedimentation rate An magnetic resonance imaging MRI of brain revealed a thickened and enhancing right oculmotor nerve in the suprasellar cistern region [Figure 2a],[Figure 2b],[Figure 2c]. Cerebrospinal fluid (CSF) was negative for malignant cells and oligoclonal bands. CSF for adenosine deaminase was within normal range. Chest X-ray was normal and Mantoux test was negative. Screening for a generalized demyelinating disorder in form of nerve conduction velocity, somatosensory evoked potential, and visual evoked potential was negative. A diagnosis of OPM was made and she was started on oral propranolol.
The term ophthalmoplegic migraine was first coined by Charcot in 1890. This condition was included as a migraine variant in the first Headache Classification of the International Headache Society in 1988.  The episodes of headache with ophthalmoplegia should accompany the absence of any demonstrable intracranial lesion other than MRI changes within the affected nerve.  Headaches are accompanied with or followed within 4 days of onset by paresis of one or more of the third, fourth or sixth cranial nerves.  Rarely, the pupil is spared. The ophthalmoplegia usually resolves completely over several weeks. However, residual ptosis and ophthalmoplegia may persist after repeated attacks. 
In fact, a number of sinister conditions including vascular aneurysms, carotid dissection, carotid-cavernous fistula, infarcts due to vasculitis, and neoplasms like primary intracranial tumors and metastatic disease can lead to a similar presentation. Other differentials that could be considered are orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome, and mycobacterial infections.  The diagnosis of OPM used to be one of the exclusion. With advancements in technology, MRI has now proven useful for diagnosis.
The unique radiological findings and clinical features point towards the pathology being one of idiopathic inflammatory neuropathy and has prompted the International Headache Classification to reclassify OPM from a subtype of migraine to the category of neuralgia. , The headache in OPM could possibly be related to secondary activation of the trigeminovascular system. 
Optimal treatment of OPM is still unclear. Calcium channel blocking drugs such as verapamil or beta blocking drugs such as propranolol have been tried in case of frequent attacks. However, efficacy remains unproven. Steroids have been used with mixed results. Our patient was started on propranalol prophylaxis.
Increased awareness among physicians regarding MRI findings of this disorder may promote early diagnosis and prevent undue investigations.
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