[Cluster like headache in a patient with the Maffucci's syndrome]

Maffucci's syndrome is a rare congenital, nonhereditary mesodermal dysplasia characterized by soft tissue hemangiomas and multiple enchondromas. A 52 years old man was diagnosised as Maffucci's syndrome in his childhood. He complained of mild paraesthesia which gradually progressed to intolerable pain lasting one to two houres with rhinorrhea (cluster like headache). Magnetic resonance imaging showed a pituitary adenoma which invaded into right cavernous sinus. Serum hormone level was almost normal, therefore we diagnosed nonfunctional pituitary adenoma. The pharmacological preventive treatments for headache, such as loxoprofen, sumatriptan, lomerizine had no effect. On the other hand, verapamil showed moderate improvement of his headache, then the symptons was gradually getting better and finally he felt no pain. This case demonstrated occurence mechanism of cluster headache and preventive effect of verapamil associated with cavernous sinus.