Moyamoya disease: long term follow-up including a normal pregnancy

We present a woman with moyamoya disease. The patient was born by prolonged vaginal delivery after a long pregnancy lasting 43 weeks. During her first years of life she depicted poor psychomotor development. When she was 9 years old a diagnosis of moyamoya disease was made. Annual neurologic evaluations have revealed moderate motor deficiency, slurred speech and borderline mental capacity. At 26 years of age she became pregnant and was prophylactically treated with 40 mg/day oral nicardipine. She had a healthy child after an uncomplicated 40 week pregnancy. When she was 33 years old a magnetic resonance (MR) study disclosed a zone of ischemic parenchyma. MR angiography yielded results similar to those of conventional arteriography. At present, the patient is 36 years old, lives a normal life and works in a factory.