Recurrent multiple cranial nerve palsies: a distinctive syndrome of cranial polyneuropathy

This report of 14 Thai patients describes the clinical features of a symptom-complex we have termed recurrent multiple cranial nerve palsies. While the disorder is common in South Eastern Asia, patients suffering similar symptoms have been seen in Europe and America. Characteristically, a long prodromal headache precedes the abrupt onset of multiple cranial nerve palsies affecting predominantly, although not exclusively, the oculomotor and facial nerves. Symptoms are self-limited and steroid therapy hastens recovery in most cases. Recurrence after many months or years is not uncommon and different cranial nerves may then be affected. Wider neurological involvement seems not to occur. Although the erythrocyte sedimentation rate is frequently elevated there is no other evidence of co-existing systemic disease. The single necropsy study thus far reported indicates that the pathogenesis of the syndrome is granulomatous inflammation of the meninges (dura), adjacent to, and involving the perineurium of cranial nerves. It is uncertain whether this inflammation is a response to a single agent, as yet unidentified, or a focal and selective hypersensitivity reaction of connective tissue to a number of different factors. As a clinical entity the syndrome is distinctive, international, and deserving of wide recognition.