Moyamoya Disease Presenting as Acute Encephalitis Syndrome

Abstract

Background: Moyamoya disease (MMD) is a chronic, progressive occlusion of the circle of Willis arteries usually affects bilateral carotid arteries than unilateral that leads to the development of characteristic collateral vessels seen on imaging, particularly cerebral angiography. Once the occlusion process begins, tends to continue despite any known medical management unless treated with revascularization surgery Report of the case: A 28 year female gravida four in 34th week of gestation presented with features suggestive of acute meningoencephalitis and CSF examination suggested 75% polymorphs of 300 total leukocytes. After 7 days of optimum management, complaints of headache aggravated so patient was subjected to MRI Brain which reported as early subacute intraparenchymal haemorrhage left frontal lobe in periventricular region with mild surrounding perifocal edema and intraventricular extension. On further MR angiogram, patient was diagnosed as MMD. Patient was asymptomatic in her previous 3 pregnancies and presently underwent planned preterm high risk caesarean section after a week. However condition of patient deteriorated over 2 weeks post delivery, did not remain fit for any neurosurgical intervention and unfortunately succumbed to death. Conclusion: Antenatal patient presenting as acute meningoencephalitis with persistent headache refractory to medical therapy, should be subjected to CT/MR brain imaging for rare possibility of MMD and early neurosurgical interventions in view of its grave prognosis.