ADULT-ONSET PRESENTATION OF DANDY-WALKER VARIANT IN SIBLINGS

BACKGROUND The Dandy-Walker syndrome and Dandy-Walker variant usually present as isolated cases of hydrocephalus in pediatric patients. METH ODS AND RESULTS This paper consists of a case report of the adult onse t of symptoms in two sisters having Dandy-Walker variant. Such an occu rrence has never before been reported in the medical literature. Both patients presented with headaches and progressive neurologic deficit. On computed tomography (CT scan) of the head, both were found to have hydrocephalus, with hypoplasia of the inferior vermis. Both patients w ere treated successfully with ventriculoperitoneal shunting. A third s ister, with a similar history, elected not to undergo CT scanning or s urgical treatment. CONCLUSION Variants of the Dandy-Walker syndrome ma y occasionally present clinically in the adult age group. Such an occu rrence in siblings is consistent with an underlying genetic etiology.