Recurrent painful ophthalmoplegic neuropathy – case presentation

Abstract

Recurrent painful ophthalmoplegic neuropathy (previously named ophthalmoplegic migraine, idiopathic inflammatory neuropathy manifesting as cranial neuropathy) may begin in childhood and adolescence. According to the third edition of the International Classification of Headache Disorders in 2018, the disease is characterized by recurrent episodes (at least two) of unilateral headache and ipsilateral paresis affecting one, two or three of the oculomotor nerves (usually the third cranial nerve). The diagnosis should be made after exclusion of orbital, parasellar and cerebellar lesions and when the symptoms can’t be better explained by another diagnosis from the classification. A typical finding in MRI is the presence of gadolinium enhancement or thickening of one of the oculomotor nerves.
We present the case of a boy with a normal premorbid development and a family history of a mother who suffers from headache. The first episode of a headache happened when the boy was 7 years old. Initially the episode started with a pain behind the right eye, accompanied by nausea and vomiting that was followed two days later by a third cranial nerve paresis. The second episode was when 11 years of age and started with a pulsating headache, accompanied by photoand phonophobia, nausea and vomiting. Seven days later the headache continued and a paresis of the right oculomotor nerve was presented. Between the two episodes the boy continued to experience migraine headache. The patient was with normal hematologic and biochemical tests, the EMG didn’t show myasthenic reaction, the CT was normal, the ophthalmologic examination showed lesion of the right oculomotor nerve.
The MRI showed enlargement and enhancement of the right third cranial nerve in the area where the nerve exits the brainstem and in its intracisternal segment which can be explained by an aseptic proximal inflammation and edema. The boy was treated with corticosteroids and a complete resolution of the symptoms was observed after 2 weeks.
The presented case fulfills the criteria for recurrentpainfulophthalmoplegicneuropathy according to the International Classification of Headache Disorders. Discussion remains for the role of the headache, whose characteristics fulfill the diagnostic criteria for a migraine without aura.