Recurrent Painful Ophthalmoplegic Neuropathy: Report of the Two New Pediatric Cases

OBJECTIVE: Recurrent painful ophthalmologic neuropathy (RPON) is characterized by attacks of one or more ocular cranial nerve palsies with ipsilateral headache. While steroid therapy has been reported to be beneficial for attacks, no clear consensus on prophylactic treatments exists. We present two cases emphasizing the diagnostic significance of loss of enhancement during symptom-free period and valproate as a beneficial option in prophylaxis.

A 4-year-old girl presented with right frontal headache, vomiting and photophobia. Neurological examination revealed ptosis and oculomotor nerve paresis in right eye. Brain MRI demonstrated a 5.5 mm nodular enhancement in 3rd cranial nerve in the right premesencephalic area. The enhancement regressed after 6-month symptom-free period. While propranolol, topiramate and flunarizine were inefficacious in prophylaxis, the patient responded to valproate prophylaxis and benefited from steroids during the attacks.

A 7-year-old girl presented with a right frontal headache and double vision. Neurological examination revealed inward gaze restriction and ptosis in the ipsilateral eye to the headache. Brain MRI revealed a 4.5 mm, enhancing, nodular lesion in the 3rd cranial nerve lodge in the right perimesencephalic area. Her symptoms regressed with dexamethasone and she received prophylactic propranolol. Neuroimaging findings disappeared after a 3-month symptom-free period. After valproate was added because of a relapse, no further attack was seen.

CONCLUSION: RPON is an uncommon disease with an unknown etiology. In ü brain MRI with contrast during the symptom-free period, regression of the enhancement or complete resolution of the lesion are guiding diagnostic features. Valproate may have beneficial effects on RPON treatment.
Keywords: ophtalmoplegia, cranial nerves, neuropathy, headache, valproate