A case of adult-onset ophthalmoplegic migraine
Keywords: Adult, diplopia, ophthalmoplegic migraine
According to International Classification Of Headache Disorders (ICHD-3) Ophthalmoplegic migraine (OM) is classified as cranial neuralgia and this is now termed as recurrent painful ophthalmoplegic neuropathy (RPON). OM is an uncommon disorder characterized by recurrent attacks of ophthalmoplegia and severe migraine type of headache. It was first recognized by Charcot in 1890. The onset of OM is generally in childhood and occurs rarely in adults. However, OM may be reported for the first time in adult life. Incidence of OM is rare in the general population, more in young adults. The incidence in general population is 0.7 per 1,000,000. Different pathogenesis of OM is postulated currently including, ischemia, compressive and demyelinating causes.
A 40-year-old man came to neurology OPD with the complaint of severe left-sided headache. He had a history of occasional headache for long duration which was less severe and self-limiting for which he did not consult any doctor due to this self-limiting headache and he was not taking any prophylactic medication. In the first visit, he was diagnosed as migraine without aura according to ICHD3 and treatment with prophylaxis with propranolol and flunarizine (40 + 10 mg) daily and naproxen and domperidone s.o.s started. After 2 days, he came with left sided-drooping of upper eyelid and double vision (left-sided ophthalmoplegia). He denied any history of fluctuation of ptosis in day and night. The pain was pressure like and sometimes pulsating sensation with no radiation.
The headache was usually associated with nausea and photophobia, and it aggravated by physical activity and would last for 2 to 3 days and remitted spontaneously. He had a history of migraine without aura attacks in a frequency of 12–16 times per year for more than 10 years.
He had no history of vision loss, weakness, or numbness of limb during headache. He had no history of vomiting, loss of consciousness, or abnormal body movement. There was no relevant family history.
On examination, there was left third nerve palsy with pupil involved. He was unable to lower and adduct his left eye [Figure 1]. There was diplopia. His left pupil was larger than his right, but pupillary reaction was slow to light and accommodation. Higher mental function was intact. There was no other cranial nerve involvement.
Fundoscopic examination was normal. Motor and sensory functions were normal in all limbs. Coordination and gait were normal. He had no meningeal signs.
Complete blood counts, erythrocyte sedimentation rate, blood sugar, liver function, and renal function were within normal limits. Serology for HIV and VDRL was negative. CSF analysis was normal. TSH was normal. CT of the brain (plain and with contrast) and MRI of the brain with contrast were normal [Figure 2].
OM is now termed as RPON. This is a rare disorder with features of migrainous type of headache followed by ophthalmoplegia. Onset is generally in childhood and adult-onset is very rare. Here, we are reporting a case of adult-onset OM. He had migraine type of headache for long duration. He came to neurology OPD for severe left-sided headache. After 2 days, he came with left-sided ophthalmoplegia, and provisional diagnosis OM made. Eye movements and headache became normal after treatment with tapering dose of oral steroids and migraine prophylaxis [Figure 3]. The patient was in follow-up for 1 year with propranolol and flunarizine (40 + 10 mg). Frequency and severity of headache became less but occasional headache was present. In follow-up, there was no relapse of ocular symptoms and ophthalmoplegia.
Different pathogenesis of OM is postulated currently of which ischemia, compressive and demyelinating causes are main.
In the past, it was postulated that during a severe migraine attack, the carotid artery of ipsilateral side becomes edematous and it compresses the oculomotor nerve inside the cavernous sinus. In ischemic theory, it was postulated that the ostia of the vasa nervosa that supplies the oculomotor nerve inside the cavernous sinus become narrow. The course and recovery of our patient is likely to fit in this postulation. Later it became possible to do magnetic resonance imaging of brain with MR angiogragraphy of the brain, which turned to be normal. It might be due to the resolution of acute ischemia of vasa nervosa supplying oculomotor nerve [Figure 2].
However, Gelfand and his colleagues found no evidence to suggest a systemic inflammatory process associated with OM in his analysis.
There is also other possible cause of OM is viral infection. This was postulated by Mark and colleagues. Although there is no significant data that can show an association between viral infection and OM, there is a single report which claims an association between OM and a positive cytomegalovirus IgG, but not IgM. However, due to the absence of the evidence of acute infection, this report shows doubtful significance.
Lance and Zigami reported five patients of OM, in which a child developed recurrent attacks of OM after vaccination on three occasions. Therefore, combining these observations and presence of nerve edema of oculomotor nerve, the authors suggested that a recurrent demyelinating cranial neuropathy may be the cause of this syndrome, in which it was supposed that inflammatory process affecting the oculomotor nerve might have irritated trigeminal sensory fibers and triggered migraine headache.
Familial cases of recurrent attacks of Bell's palsy and ophthalmoplegia that was preceded by headache have been reported. There is also several case reports of OM in which patient had personal or family history of Bell's palsy.
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[Figure 1], [Figure 2], [Figure 3]