Moyamoya Disease (MMD)
Moyamoya disease (MMD) is a progressive occluding intracranial carotid artery disease, which can be either unilateral or bilateral. Here, the anterior circulation is commonly involved. In the center core, there is development of a profuse thin wall capillary known as moyamoya vessels. MMD is among the most common causes of neurological morbidity and disability in children. In children, it usually presents with ischemia and infarct, whereas in adults, it presents with hemorrhage. A CT scan of the head, an MRI of the brain, CTA and MRA are the necessary investigation methods, whereas a cerebral DSA is the gold standard. Perfusion images are important for follow up. Treatment includes cerebral revascularization by a direct or indirect bypass, or in combination. In this chapter, the etiopathogenesis, clinical features, investigation methods and management of MMD will be discussed.
