Al-Shifa Journal of Ophthalmology (ASJO)
|Aims and Scope||
Ophthalmoplegic Migraine; A case report and review of literature
Afghani, MBBS, MCPS, MS (Pak), DCEH(London), FIACLE(Australia)
highlight the presentation and management of a case of ophthalmoplegic
migraine and mini review of the subject.
Design: Case report.
12 years old boy presented with 4-months history of severe episodic
ipsilateral left sided headache along with pain in and around the eye,
usually starting in the evening and lasting for 1-2 hours, improving
mostly with vomiting followed by sleep. Five months after the start of
these episodes, he started having mild swelling and moderate drooping of
upper lid on the same side. This was associated with limited ocular
movement especially in elevation and abduction. Interestingly the pain in
and around the eye and vomiting was less marked when ocular motility
disturbances started. The
child was brought to oculoplastic clinic when child began to have ptosis.
Clinically the eye examination was normal except for left upper lid
ptosis, and limited abduction and elevation. The systemic examination was
also normal. Radiological studies and serological examination was within
normal limits. The child was diagnosed as a case of ophthalmoplegic
migraine and put on 30 mg of steroids tapered over a period of three
weeks. The child responded well and all symptoms and signs disappeared.
Two weeks after the steroids were stopped, there was recurrence of the
same. The child was again given the same dose and course of treatment.
The child is alright and symptom free for the last one year.
received: October 2, 2004
Left ptosis at presentation Limited elevation on left side
incidence of childhood migraine in general has been estimated at between
2% and 5.7%.1,2.
Ophthalmoplegic migraine is a rare condition usually beginning in infancy
or childhood and characterized by paralysis of ocular cranial nerves in
association with vascular headache. The diagnosis is one of exclusion.
Most patients should have high resolution magnetic resonance imaging (MRI)
or magnetic resonance angiography (MRA) to eliminate the possibility of
aneurysm, tumor, or granulomatous inflammation
of the cases of acute third nerve palsy and headache formerly referred to
as "ophthalmoplegic migraine" show MRI enhancement of the nerve3,4.
It appears now that this entity is most like Bell's palsy in that it is an
isolated "idiopathic" probably post-viral inflammation of a
cranial nerve, the III. The headaches may
also be associated with other ocular motor nerve palsies as well5,6.
Usually the ophthalmoplegia is transient; however, it can become permanent
especially after repeated attacks. In this case, isolated, recurrent
unilateral eyelid ptosis with some degree of reduced ocular motility was
the sole manifestation of ophthalmoplegic migraine.
presented specific criteria for the diagnosis of the syndrome:
Ophthalmoplegia including one or more nerves and possibly alternating
sides with attacks. Extraocular muscle paralysis may occur with the first
attack of headache or, rarely, precede it. However, the paralysis usually
appears subsequent to an established migraine pattern.
Exclusion of other causes
et al 8 found an annual incidence of 0.7 per million
inhabitants for ophthalmoplegic migraine.
In a review of 5,000 migraine patients, Friedman et. al9
found 8 patients with ophthalmoplegic migraine, thus attesting to its
rarity (0.16%). All 8 patients (5 male and 3 female) had periodic migraine
headaches and unilateral ophthalmoplegia. Three patients had persistent
ophthalmoplegia after years, and 1 had a definite family history of
migraine. Six patients had arteriograms during the attacks; all results
were normal. The details of this review are listed below:
of attacks: 4 patients with 20+ attacks; 2 patients with 5 to 10 attacks;
2 patients with less than 5 attacks; and 1 patient with a single attack.
Age at onset: 2, 2, 3, 3, 5, 8, 17, and 30 years. Clinical findings:
Oculomotor paresis in all; pupillary involvement in 7. Pain: Always on the
same side as ophthalmoplegia. Paresis: Occurred 3 to 5 days after onset of
headache in 6 patients. Recovery: 1 to 4 weeks. Arteriogram: Normal in
attack in 6 of 6. Therapy: Limited success.
the differential diagnosis, consideration should be given to aneurysm,
tumor, diabetes, and sphenoid sinus mucocele. The age at onset, negative
glucose tolerance test, and radiologic studies will usually rule out the
listed possibilities. Other clinical entities confused with
ophthalmoplegic migraine have included myasthenia gravis and the Tolosa-Hunt
syndrome. The former condition is ruled out if the pupil is involved (and
actually should not be considered in the presence of pain) and with
response to edrophonium chloride (Tensilon); the latter possibility should
be considered if pain persists. On rare occasions only limited involvement
of the third nerve occurs.
migraine is a diagnosis of exclusion, and noninvasive imaging tests such
as magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA)
should be performed in all cases to exclude the possibility of aneurysm6.
Aneurysmal third nerve
palsies are extremely rare in children under age 14 years15. However,
in third nerve palsy involving pupillomotor function, serious
consideration should be given to angiography. The usual cause will be a
posterior communicating artery aneurysm, which is best excluded by
conventional angiography. However, newer techniques such as MRA or spiral
contrast-enhanced computed tomographic scanning may soon provide
sufficient resolution to exclude aneurysm as a cause6.
Ideally, prophylactic therapy would prevent the occurrence of repeated episodes and prevent the development of permanent eye muscle palsies, but reports suggest that therapy has met with only limited success9. A trial with calcium channel blocking drugs such as verapamil or beta blocking drugs such as propranolol or even methysergide may be warranted if the attacks are frequent.
a case reported by Japanese observers21 the frequency and symptoms of migraine were remarkably
reduced by topical administration of 0.25% timolol maleate twice daily to
both eyes. Others have found
cyproheptadine hydrochloride quite useful in
preventing recurrent attacks22.
Based on a recent suggestion that prostaglandins' release (especially
PGE1 and PGF2alpha) play a key role in the development of the migrainous
attack. Rabey et al 23
found a therapeutic trial with flufenamic acid (a prostaglandin inhibitor
of the fenamates group) to be quite successful.
Given the fact that this entity is probably similar to Bell's palsy one now may consider initial treatment with steroids and perhaps antiviral agents such as famcyclovir.
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Enhancement of oculomotor nerve: a diagnostic criterion for
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Hallet M, Cogan DG: Episodic unilateral mydriasis in otherwise normal
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Sugiyama N, Hamano S,
Tanaka M, Mochizuki M, Nara T; MRI findings and effectiveness of
cyproheptadine in two patients with ophthalmoplegic migraine.