Moyamoya Center

Diagnosis and treatment of Moyamoya disease

Early diagnosis can not only prevent a whole range of disabilities resulting from repeated strokes but also significantly improve the chances of recovery. It is noticeable that in children under five the disease is generally well advanced at the time of first diagnosis. Symptoms range from headaches, transient ischemic events, loss of motor or sensory function of an arm or leg, speech disturbances, visual problems, movement disorders, changes in cognition and behaviour as well as epileptic attacks. As the disease progresses multiple strokes can occur resulting in progressive brain damage.


Image-generating processes to diagnos Moyamoya

Exact diagnosis and preliminary examinations

Examinations begin with an MRI/MRA test (Magnetic Resonance Imaging and Magnetic Resonance Angiography). Examination of the brain and of the six main blood vessels of the brain confirm the suspicion of Moyamoya. This is followed by an H215O-PET scan (Positron Emission Tomography) to assess the blood flow and perfusion reserves.

In order to be able to plan future surgical interventions, additional neurological, genetic and child behaviour and development investigations are also necessary. Depending on the extent of the angiopathy in the brain and/or presence of additional systemic diseases (Moyamoya Syndrome), further targeted examinations may be required.

 

Require revascularisation operations

Surgical intervention and hospital stay

Once the neuroradiological tests (MRI, angiography, PET) results have been evaluated, the surgical revascularisation can be planned. Depending on which areas of the brain lack adequate blood flow/perfusion, surgery can be performed in a staged fashion in all these areas. Most children require surgery. The blood flow is reinstated as a result of a direct or indirect revascularisation operation. Surgery is the only means of stroke prevention in Moyamoya children. The earlier the diagnosis is made and the earlier the disease is treated, the better is the prognosis and the child can subsequently lead a normal life again without any mental and physical impairment.

Depending on the number of surgeries required, the duration of the hospital stay varies between seven and fourteen days.

 

Check-ups and follow-up examinations

Check-ups and follow-up examinations

The first postoperative follow-up is carried out at six months to one year after surgery. Thereafter regular check-ups are carried out on an annual to three-yearly basis until puberty.