Moyamoya is a rare disease where the narrowing of the carotid arteries takes place leading to formation of collateral blood vessels in the brain. These vessels have a hazy appearance on angiogram and hence the name moyamoya is given to them, which means puff of smoke in Japanese. It is characterized by stenosis or occlusion of the vessels supplying oxygenated blood to the brain. These vessels that are formed to compensate the blood supply are weaker and rupture and leak inside the brain leading to ischemic or hemorrhagic strokes.
Recovery Period For Moyamoya
The recovery of the patients depends on multiple factors such as the age of the patient, progression of the disease, extent of vascular blockage and severity of disability the stroke produces. In some cases the disease progresses slowly with few incidents of TIAs or strokes, while in others there can be rapid decline in the health of the patient. If the condition of the patient is manageable then the person can undergo surgical procedures to develop collateral circulation. The outcome after surgery is excellent and chances of recovery are quite good. In patients who are not given treatment there will be progression of the disease and worsening of the symptoms and health of the patient in almost 50-65% of the cases.
The occurrence of stroke in these people is also found to be higher even when no symptoms are present. A person can develop neurological deficits if the disease is not properly managed or left untreated and the disability produced as a result of strokes can be permanent. In these patients the rehabilitation programs are helpful where speech, physical and occupational therapy is given to help them cope with the disability and limitations that have occurred as result of the disease.
The exact cause of moyamoya is not known, but it is believed to be genetically predisposed in at least 10% of the cases. They are also found to be associated with several diseases like Down’s syndrome, neurofibromatosis and sickle cell disease. It most commonly occurs in children in the age group of 10 to 15 years and adults aged between 30 to 50 years. Symptoms presented by the patients are headache, involuntary movements, seizures, developmental delay, and loss of speech, stroke that might result in loss of function of both the limbs or paralysis of one side of the body (also known as transient ischemic attacks). Adults are more prone to suffer hemorrhagic strokes as compared to children due to development of hypertension in adulthood.
It is important to identify the disease in early stages and get treatment as soon as possible. Any symptoms suggesting ischemic or hemorrhagic pathology of brain should be immediately brought to the attention of the physician. Based on the history and certain tests the condition is diagnosed. A neurosurgeon will advise CT and MRI angiogram to confirm the diagnosis of moyamoya where the vessels can be clearly seen in the brain. The medical treatment is started on the basis of the symptoms experienced by the patient.
The use of aspirin is of utmost importance to prevent blood clots from developing that might clog the already narrowed vessels in the brain. Anti-seizure drugs should be administered in patients who are having seizures. Ca blockers are given to reduce symptoms of headache and transient ischemic attacks. They should be given under the supervision of a trained specialist as they can lower blood pressure and increase the risk of development of strokes.
There is no medicine that can reverse or prevent the narrowing or stenosis of the blood vessels so the disease of moyamoya will continue to progress even with treatment. The main goal is to prevent the occurrence of stroke that can lead to irreversible health complications in the patient. Surgical treatment can help prevent the occurrence of ischemic and hemorrhagic strokes by revascularization of the affected brain. The procedures used are encephalomyosynangiosis, EDAS, pial synangiosis, etc. where they help in inducing growth of new vessels in the brain.