From the Guidelines
Moya Moya disease is a rare cerebrovascular condition characterized by progressive stenosis of the terminal portion of the internal carotid artery and the formation of an abnormal network of dilated, fragile perforators at the base of the brain [ 1 ].
Key Characteristics
- It is a stenotic-occlusive condition that leads to reduced blood flow to the brain [ 1 ]
- The disease is marked by the formation of a network of small, fragile blood vessels that can rupture and cause bleeding or stroke [ 1 ]
- The condition can be unilateral or bilateral, and proximal middle cerebral artery or anterior cerebral artery involvement is sufficient for diagnosis [ 1 ]
Treatment and Management
- The primary treatment for Moya Moya disease is surgical revascularization, which involves bypassing the blocked artery to restore blood flow to the brain [ 1 ]
- Medications such as aspirin may be prescribed to prevent blood clots and reduce the risk of stroke [ 1 ]
- In some cases, anticoagulants like warfarin or heparin may be used, but with caution due to the risk of bleeding
- Regular follow-up appointments and imaging tests, such as MRI or CT scans, are necessary to assess the progression of the disease and adjust treatment as needed [ 1 ]
From the Research
Definition and Characteristics of Moya Moya Disease
- Moya Moya disease is a chronic cerebrovascular disease characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches 2, 3, 4.
- The disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels 2, 5.
- Moya Moya disease has several unique clinical features, including two peaks of age distribution at 5 years and at about 40 years 2.
Clinical Features and Diagnosis
- Most pediatric patients have ischemic attacks, whereas adult patients can have ischemic attacks, intracranial bleeding, or both 2.
- Sudden changes in the supraclinoid internal carotid artery and middle cerebral artery can be recognized with noninvasive neurovascular imaging techniques 5.
- The diagnosis of Moya Moya disease can be established using cerebral angiography, which shows the formation of collateral vessels with the appearance of a puff of smoke (moyamoya in Japanese) 5, 4.
Treatment and Management
- Surgical revascularization, including extracranial-intracranial bypass, can help prevent further ischemic attacks and preserve quality of life 2, 5, 3, 4.
- The goal of surgical treatment is to increase cerebral blood flow and prevent recurrent stroke 5, 3.
- Long-term outcome of revascularization surgery for Moya Moya disease is favorable, but rapid increase in cerebral blood flow on the affected hemisphere could temporarily cause unfavorable phenomenon such as cerebral hyperperfusion syndrome 3.
Types and Associated Conditions
- Moya Moya disease can be unilateral or bilateral, with unilateral cases being less common and potentially representing an early form of definite (bilateral) Moya Moya disease 6.
- When Moya Moya disease is associated with other systemic or acquired diseases, it is referred to as Moya Moya syndrome 5, 4.