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Moyamoya disease



Moyamoya disease Causes


Though there is no clinical evidence about the causes of the disease, it is believed to be hereditary. In 1997 Fukui reported a family history in 10% of patients with Moyamoya disease. Genetic studies also revealed that susceptibility loci have been found on 3p, 6p, 17q, and band 8q23.


Moyamoya disease Definition


In most parts of the world, except Japan, Moyamoya is considered an extremely rare disease. Characterized by progressive intracranial vascular stenoses of the circle of Willis, the condition may result to ischemic events as well as hemorrhagic events. It may also lead to irreversible blockage of the carotid arteries to the brain as they enter into the skull.


Moyamoya disease Diagnosis


CT, MRI, or an angiogram are initially suggested for the diagnosis of the disease. The angiographic image of the disease reveals a “puff of smoke,” which is moyamoya in Japanese, thus the name. Contrast T1-weighted images however are better than FLAIR images for depicting the leptomeningeal ivy sign in moyamoya disease. For further diagnosis and follow up MRI and MRA should be performed. The decreased blood and oxygen supply to areas of the brain can be demonstrated through single photon emission computerized tomography or SPECT, a procedure in nuclear medicine.


Moyamoya disease Symptoms and Signs


Moyamoya tends to affect children and adults in the third to fourth decades of life. Strokes and seizures may be experienced by children. Bleeding and strikes can also occur among adults. The disease's clinical features are cerebral ischaemia, recurrent transient ischaemic attacks, also known as TIAs or mini strokes, sensimotor paralysis, convulsions, and/or migraine-like headaches. Moyamoya is also common among women than in men.


Moyamoya disease Treatment


The most favored operations developed to treat the disease are the in-direct procedures encephaloduroarteriosynangiosis (EDAS), encephalomyosynangiosis (EMS), and multiple burr holes and the direct procedure STA-MCA. Although its efficacy related to hemorrhagic disease is still uncertain, direct superficial temporal artery (STA) to middle cerebral artery (MCA) bypass is considered the treatment of choice. Said operations carry the concept of a blood and oxygen starved brain reaching out to grasp and develop new and more efficient means of bringing food to the brain and bypassing the areas of blockage.


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