The Website moyamoya.net
Moyamoya disease is a cerebrovascular disease where the main arteries feeding the brain are occluded on both sides. This results initially in transient episodes of “ischemia” where due to decrease in blood flow the brain suffers from attacks of loss of function presenting in the form of weakness of a limb, sensory disturbances, epileptic seizures or simply headaches. If this continues the brain can suffer a “stroke” with permanent loss of function and invalidity. In children this disease can be fatal or leave a child handicapped, mentally retarded, unable to speak and bound to a wheel chair.
The disease was first described in Japan in 1956 and is therefore mainly well known in Asia, although in recent years patients have also been diagnosed and treated in US A and Europe. Treatment with prevention of stroke and all its consequences is possible through an EC-IC bypass procedure which allows for extra blood to reach the brain areas suffering from lack of adequate blood supply.
Over the past few years I have been involved in the management of Moyamoya children from all across Europe. The lack of awareness of the existence of this disease in Europe and the lack of understanding, diagnosing and treating these children, not only among those suffering from the disease but also the medical professionals forced me to initiate a “Moyamoya Awareness Project”. This project was first and foremost initiated for the children, their parents and their caretakers to help understand it and to take away any anxiety related to its diagnosis and surgical treatment.
The initial step was to produce two childrens books. “Sven” to explain the disease in simple illustrations for children upto 8 years of age and the second “Fatmas fantastic Journey” a comic for older children, telling a fantasy story, a journey of 4 Moyamoya children within the human brain. This process of increasing awareness also involves the making of a universal homepage allowing for a single international platform of information and exchange for all MM patients and caretakers across the globe.
This moyamoya.net website should be a source of information, explaining the patients/parents the disease, its presenting symptoms and the consequences if not treated. It should be an easy guide for parents of moyamoya children helping them to answer questions like “what do I do now that I know my child has Moyamoya?”, “Where do I go for treatment?”. It should provide for assistance for patients and families in coping and understanding this disease and its management, provide an updated list of specialists and centres of care across the world, provide addresses of self help groups for further information and self-participation. It should also allow for linking websites from USA, Europe and Japan to provide online access of Q/A sessions between patients and specialists locally and internationally to provide the necessary information, update and assistance.
All this pooled and linked information will be “new” and “first time round” .The Moyamoya angiopathy is always referred to as a rare disease. In our experience this is defintiely not because of the lack of its existence in the non Asian population but mainly because of the lack of awareness of this disease entity as such and especially in children. “Stroke in children” is an extremely important yet very often ignored topic amongst the medical professionals. The need to “write more” about this an giopathy is hence obvious. Explaining its presentation in adults and children, the possibilities of diagnosis as well as the surgical management need to be outlined, protocoled and published so that care takers all over the world can follow guidelines in their day to day patient management.
Nadia Khan MD
Children's University Hospital Zurich