Ophthalmoplegic Migraine Presenting in infancy: A Self-Reported Case

Case report

I was born in 1976, the 4.1-kg product of an uncomplicated full-term pregnancy and spontaneous vaginal delivery to a 24-year-old prima gravida. APGARs were 9 and 9 at 1 and 5 min, respectively. My family history was significant for migraine headaches in a maternal grandmother and Bell’s palsy and migraine headaches in a maternal great aunt. My father has familial Mediterranean fever.

I was in good health until 3 months of age, when my mother, a nurse by training, noted the sudden onset of a right ptosis in the setting of 1 day of increased irritability and an incident of minor trauma to the right temporal area. The following day my paediatrician noted a complete right ptosis and some dilation of the right pupil. I was referred to an emergency room, where blood work and skull films were unremarkable. I was discharged home and my right eye began to open within 2 days with full resolution of the ptosis and anisocoria by 2 weeks (Fig. 1).

OPEN IN VIEWER

Figure 1

The author at 3 months of age with a resolving right third nerve paresis.

In July of 1978, at 21 months of age, my mother noticed the sudden onset of a complete right ptosis and mydriasis with deviation of the right eye downward and outward. After my paediatrician confirmed a right third nerve palsy he referred me to a paediatric neurologist. Computed tomography (CT) of the brain was normal and I was diagnosed with OM. The third nerve palsy completely resolved over the course of the next 4 weeks.

In July of 1980, at 3.5 years of age, I developed an episode of low-grade fever, sore throat and fussiness. One week later I developed a right third nerve palsy with complete ptosis. I was again referred to the paediatric neurologist, who noted a cranial bruit on examination and requested a head CT with and without contrast, which was once again unremarkable. The ptosis and eye deviation resolved over the course of 6 weeks but the anisocoria never completely resolved.

In March of 1982, at 5 years of age, I was noted to have slight drooping of the right eyelid one morning in the setting of low-grade fever and sore throat. By the next morning the right ptosis was complete and I was complaining of a severe headache around my right eye. The headache continued for 2 days and then began to improve with persistent, mild, intermittent headaches continuing for several more days. Three days after the beginning of the headache the right ptosis began to improve and I began complaining of binocular vertical and horizontal diplopia.

At this time I was referred to a neuro-ophthalmologist. On his examination, 15 days after the attack began, I was noted to have a visual acuity of 20/60 in the right eye and 20/25 in the left eye. My right pupil was 5 mm and 1 + reactive to light and the left was 3 mm and 3 + reactive to light. A 1-mm ptosis was noted on the right and a slight limitation of elevation, adduction and depression of the right eye.

Examination 10 weeks later revealed resolution of the right ptosis and full extraocular movements. The right pupil remained slightly larger and less reactive to light than the left.

In March of 1984, at 7 years old, I experienced another episode of severe, persistent headache and right eye ptosis that is not well documented.

In August of 1986, at 9 years old, I again complained of a severe, debilitating, right periorbital headache which persisted for several days. This is the first attack that I can remember being associated with significant photophobia and phonophobia, although this is not documented in the medical record. After 5 days, as the headache resolved, I developed a right ptosis and diplopia. By the neuro-ophthalmologist’s examination, 10 days after the beginning of the headache, I was noted to have 20/50 vision in my right eye and 20/20 vision in my left eye. The right pupil was 8 mm and 2 + reactive to light, whereas the left pupil was 6 mm and 3 + reactive to light. A mild but definite limitation of elevation, depression and adduction was noted in the right eye.

I was not seen for follow-up until 16 months later, when I was noted to have normal visual acuity in both eyes with full extraocular movements with no ptosis. The only abnormalities on examination were the persistent anisocoria and a mild, decreased accommodation in the right eye with a near vision in that eye of J3 compared with J1 + in the left eye.

In June of 1995, at 17 years old, I again developed a severe, debilitating, throbbing, generalized headache which was worse with activity and was the first attack with documented photophobia and phonophobia. As with all prior attacks, there were no associated nausea or vomiting. The headache continued for 3 days and was followed by a week of right supraorbital pain with a mild right ptosis and double vision. Examination 3 weeks after the beginning of the headache revealed normal visual acuity bilaterally with a near vision of J2 in the right eye and J1 + in the left eye. The right pupil was slightly larger than the left and there was a mild limitation of upgaze in the right eye.

Of note, between the age of 5 and 15 years I did complain of frequent, severe headaches which were not associated with any ophthalmoplegia or migraine symptoms including nausea, vomiting, photophobia or phonophobia. These headaches varied in duration from minutes to hours and also varied in location and quality.

Since that time I have been virtually headache free. A thorough ophthalmology examination at 28 years of age revealed a right pupil which is 2 mm larger than the left but equally reactive. I am right handed but have a profound left eye dominance by examination. Near vision was J1 in the right eye and J1 + in the left eye. Far vision was 16/13 bilaterally. No abnormality of extraocular movement or cranial nerves was noted. A brain magnetic resonance imaging (MRI)/magnetic resonance angiography performed soon after this examination was unremarkable.

Discussion

The diagnosis of OM cannot be made without a thorough history, physical examination and neuroimaging to exclude cavernous sinus, orbital fissure and posterior fossa lesions. In my case, CT with and without contrast was initially used. Since that time MRI with contrast has become the imaging of choice. Enlargement and inflammation of third, fourth or sixth cranial nerve as they exit the midbrain can often be seen in the setting of acute attacks of OM. Some believe that these MRI findings should be required for diagnosis of OM (6).

The diagnosis of OM is particularly challenging in the infant, as most children <3–4 years old cannot indicate the presence of headache. Unless a clear history of nausea, vomiting and irritability is present, the diagnosis of OM often cannot be made conclusively until the child can better articulate their symptoms. As in my case, the features of migraine headache may not be well described until the second decade of life and the presence of non-migraine headaches is common.

The course of OM is variable. I experienced seven attacks over the course of 18 years with attacks occurring every 1–2 years during the first 9 years of life. This frequency of attacks is relatively representative of OM, although some have more frequent attacks early in life (6). Many patients with OM in childhood have gradual resolution of the ophthalmoplegia during adolescence, although the headaches often persist (3, 7). In my case, both headaches and intermittent ophthalmoplegia have resolved.

One unique feature of my case is the apparent seasonality of the episodes of OM, with all documented episodes except the first occurring during the spring and summer. Several of these episodes were also accompanied by symptoms of a viral syndrome. Some authors have considered that a viral neuropathy may be the cause of OM in some patients (8).

Experts continue to debate the best treatment for OM. In my case, no treatment was offered because of the infrequency of the attacks of OM. Migraine prophylaxis with β-blocker or calcium channel blocker medications has been recommended by some authors in patients with recurrent attacks (9). Two cases of apparently successful prophylaxis with flunarizine have also been described (3).

Corticosteroid treatment of OM in children has also been described in 12 cases, with favourable responses documented in six of them and no adverse events. Prompt resolution of headache and ophthalmoplegia with steroid treatment seemed to be related to early administration and dose of at least 2 mg/kg per day of prednisone according to one review (6).

The long-term visual and ocular prognosis of patients with OM remains poorly defined, as few case reports include long-term follow-up. In my case, after seven attacks of OM, I have a mild anisocoria, slightly decreased near vision in the right eye and profound left eye dominance with no far vision deficit or abnormality of extraocular movements. In one series, some evidence of persistent third nerve injury was present in 6/14 patients ranging from partial paresis to complete paralysis (6).

As in my case, transient monocular visual loss in the setting of acute attacks of OM is expected, although not frequently documented. This visual loss is primarily due to a combination of loss of accommodation and glare in the setting of transient third nerve paresis with pupillary involvement. After attacks of OM resolution of my visual loss, particularly near vision loss, seemed to lag behind the return of full extraocular motion. Persistent monocular visual loss can also occur in the setting of permanent third nerve paresis (10).

I have described my own case of OM, which is one of only two such cases presenting as early as 3 months old. To the best of my knowledge, this represents the only self-reported case of OM and the long-term follow up available for this case illustrates many important features of the natural history of this disease.