A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis
is described. She was diagnosed after acute neurological deterioration by
MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was i
njected locally to recanalise the thrombus. The patient's condition signifi
cantly improved and she was discharged. After 2 years of followup the child
remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis sho
uld not be overlooked as a cause of acute neurological deterioration in a c
hild with Down's syndrome. MRA ap pears to be a safe and accurate alternati
ve to traditional angiography for the diagnosis of moyamoya disease. Local
fibrinolysis with r-TPA is the treatment of choice for cerebral venous thro
mbosis due to its safety and efficacy.