Introduction: We present 12 patients with moyamoya disease and their evolution from an early age to adulthood in some cases.
Material and methods: The patients (nine females and three males) were first studied before 10 years of age because of neurological disease. Bilateral carotid and vertebrobasilar arteriographies were performed during initial evaluation. In the patients who were followed-up during several or many years (in one, during 33 years and, in two, during 25 years), neuroimaging studies were done by magnetic resonance angiography (MRA). One patient also had neurofibromatosis type 1 (NF1).
Results: All patients showed bilateral occlusion of the supraclinoid portion of the internal carotid arteries. The basilar artery was markedly narrowed in one patient. One patient with very early onset of symptoms also showed collateral vascularization through the Bernasconi- Cassinari artery. Cerebral multifocal cortical and subcortical lesions were seen in the patients who had very long follow-up, although these already had appeared in the first MR studies. One patient showed arterial moyamoya 31 images and fibromuscular dysplasia. She was the only patient treated surgically during childhood with death after a short time. Another patient had a pregnancy and vaginal delivery at 26 years of age without complications. Images of moyamoya did not disappear in any of the patients in this series. Seizures were controlled with antiepileptic medication in all patients except in one who had NF1 and severe cerebral lesions. Although it is difficult to demonstrate the efficacy of calcium-antagonist medication (nicardipine), we found that it improved the neurological symptoms in most of our patients.
Conclusions: Long-term follow-up of the patients in this series showed some neurological problems, although most of them did well, including those cases with early onset of symptoms. All adult patients in this series were working, although some of them in low qualified jobs. Collateral vascularization was very marked in all patients except in one who also showed fibromuscular dysplasia. Moyamoya images did not disappear in any patient. Ischemic cerebral lesions seem to be underlying the cause of the neurological sequelae. Seizures responded well to antiepileptic medication.