MOYAMOYA DISEASE - CLINICAL PROGRESSION AND LONG-TERM OUTCOME: A CASE REPORT

Abstract

Moyamoya disease (MMD) is a chronic cerebrovascular disease, characterized by stenotic- obstructive changes at the terminal portions of the internal carotid artery (ICA) and formation of an abnormal vascular network at the cerebral base. The clinical presentation of MMD may vary from TIA and ischemic or hemorrhagic strokes to seizures, headaches and cognitive impairment. Typically strokes in the MCA and ACA are prevalent at the initial stages, while PCA vascular incidents increase with disease progression and are considered to be a poor prognostic factor. We present a case of a 21-year-old man admitted at the Neurology Department with Jacksonian seizures in the right arm followed by right-sided weakness and sensory-motor aphasia. Over the following decades he suffered subsequent transitory episodes of visual impairment, one ischemic stroke with right-sided weakness and a left thalamic hemorrhage. CT scans, angiographies and MRI showed multiple ischemic lesions. A catheter-based DCA showed multiple stenotic regions in the ICA, MCA and ACA and occlusion of the left PCA in P1 and well-developed collateral network of the right PCA thus confirming the diagnosis of MMD. Moyamoya should always be considered as a possible cause for vascular events in young adults and especially in children. Therapeutic measures are focused on secondary prophylaxis of ischemic events with antiplatelets as there is no specific treatment available to stop the progression of the disease. Current guidelines suggest surgical methods as the only effective prevention of further disabling ischemic and hemorrhagic strokes.