RUPTURED RATHKE CLEFT CYST - A POSSIBLE CAUSE OF TOLOSA-HUNT-SYNDROME

Unexpected autopsy findings are presented of a patient who died sudden ly after a 6-month history of progressive headaches, nausea, and vomit ing. A ruptured Rathke's cleft cyst (RCC) was identified within the ad enohypophysis, with a chronic inflammatory reaction in surrounding pit uitary and overlying hypothalamus. A brisk lymphoplasmacytic response was also seen in the cavernous sinuses bilaterally, identical to the p athology reported for idiopathic painful ophthalmoplegia, also called Tolosa-Hunt syndrome (THS). The pathogenesis of THS has not been eluci dated; based on the findings in this report, it is suggested that some THS cases may result from a hyperimmune response to RCC rupture with extension into one or both cavernous sinuses. Although prompt alleviat ion of symptoms with corticosteroid treatment is generally encountered with THS, recurrence of symptoms is not uncommon. A careful search fo r a ruptured RCC should be undertaken in atypical cases of THS, with p ossible consideration of surgical intervention.