Moyamoya is a rare and progressive disease of vasculature of brain that can lead to stroke or hemorrhage of blood vessels. The occlusion or stenosis of circle of willis can lead to reduced blood flow and oxygen supply to the brain posterior to occlusion/stenosis. To compensate this reduced blood supply, collateral vessels around the occlusion develop, which give a hazy puff of smoke appearance in angiogram. Moyamoya is the Japanese translation of ‘puff of smoke’ as it was first described there. These collaterals are very fine and fragile and their rupture can cause hemorrhage. The reduced blood supply can lead to ischemia causing transient ischemic attack or stroke in moyamoya disease.
How To Cope With Moyamoya?
The backbone of moyamoya treatment is surgical intervention. Surgery can provide revascularization to the ischemic area. Surgery has very good outcome and drastically reduces the probability of stroke by improving the blood flow the area distal to occlusion/stenosis, which provides symptomatic relief. The main complication related to surgery could be hyperperfusion, postoperative cerebral infarction, vascular bypass occlusion, bypass anastomotic aneurysm among others.
Postoperative care is needed and lifelong daily aspirin or other anticoagulant (Coumadin) is prescribed to prevent future strokes and better coping. Patients may feel headaches that might or might not be related to moyamoya. Patients are advised to keep hydrated and drink lots of water as this will increase blood volume and increase blood flow and maintain circulation.
Patients with moyamoya require minimal lifestyle modification and restriction in diet or activity; they can live their lives comparable to individuals without moyamoya. Smoking and alcohol consumption should be avoided. Caffeinated drinks should be avoided and people should follow a healthy diet. However, in females birth control pills should be avoided as they carry a higher risk of blood coagulation. Individuals should also be wary of hypertension, as it has a potential to cause intracerebral hemorrhage. Blood pressure should be within the range of 110-130/70-90.
As the disease is more common in children, there is general anxiety in parents about the health of their child and recurrent stroke. They may even have speech problem, developmental problem and language problem. These children can undergo rehabilitation, occupational and/or speech-language therapy, according to their requirements.
Family members should be aware of recurrence of stroke or progression of cognitive problems, if so, then neurologist and stroke specialist should be immediately consulted.
Long-term follow up with specialist is required for blood flow, angiogram, MRI, and neuropsychological testing at various intervals post surgery. Long-term follow up is required to ensure that blood flow is adequate without development of any complications. People with unilateral moyamoya should follow annually for CT/MRI angiography to study the progression of moyamoya on contralateral side. People with bilateral surgeries do not require routine testing, unless symptomatic.
Although, people with moyamoya post-surgery can live their lives like any other individual keeping in mind certain things and following the instructions of their doctors, immediate follow up should be undertaken, if patient suffers from any complication or symptoms suggestive of stroke.
The etiology of moyamoya is still unclear. However, it has been associated with other diseases, such as Grave’s disease, hypertension, Down’s syndrome, neurofibromatosis type 1, atherosclerosis, sickle cell anemia, tuberculosis and Turner’s syndrome. It affects females more than males and is known to have a genetic predilection. Moyamoya can affect infants as young as 6 months and commonly affects children less than 10 years. In adults it affects people in their 30 and 40s.
Since the disease is progressive without treatment the symptoms will worsen over time. It mostly presents with symptoms of stroke or transient ischemic attacks, including weakness of face and arms along with paresthesias, seizures, speech problem and difficulty understanding language, visual problems, balancing problem along with headaches.
It is important to diagnose the condition as early as possible to receive treatment in the earliest phase when the symptoms are evolving instead after a complete stroke, which has poorer prognosis.