Moyamoya disease is a rare cause of stroke, particularly affecting children. The main blood vessels supplying blood to the brain slowly get blocked over a period of time. The block occurs typically at the base of the brain at the ends of the main blood vessels supplying the brain. This causes the smaller blood vessels around that area to form alternative channels of blood supply to the brain. However, these alternate channels may not meet the blood supply-demand and the brain suffers strokes, which may be minor or major. Minor strokes cause arm or leg weakness, loss of speech, and seizures, which recover completely after a short duration. Major strokes cause the same symptoms as minor strokes but may be permanent; partial recovery may occur. Rarely, the tiny blood vessels that form the alternate channels may rupture causing bleeding into the brain.

You should consult a child specialist (Paediatrician) or a Neurologist.

If you are suspected to have Moyamoya disease, the following investigations will be advised:

Magnetic Resonance Imaging (MRI): This is a safe imaging technique of the brain which is used to identify strokes. A dye may be injected to study the blood vessels of the brain (MR Angiogram). Inaddition, a perfusion MRI is advised to study the quantity of blood supplied to the brain.

Computed Tomography Angiogram (CTA):This imaging technique uses X-rays to study the blood vessels of the brain after a dye is injected into a vein.

Digital Subtraction Angiogram:This imaging technique uses X-rays to accurately image the blood vessels. A dye is injected through a catheter (tube) inserted into an artery in the groin. The patient is admitted to the hospital for this investigation.

If you are suspected to have Moyamoya disease, the following investigations will be advised:

General measures: It is recommended to consume plenty of oral fluids to keep the blood flowing. The patient is advised not to exert or engage in games and sports that result in deep and rapid breathing. Parents are advised to keep affected children calm and avoid excessive crying. Spicy foods that result in deep breathing may also be avoided.

Medications:The child-specialist or the Neurologist will prescribe blood thinners such as Aspirin. This dissolves any small clots that form at the site of block in the blood vessel. Your doctor may diagnose a high blood pressure and recommend medications. However, a higher blood pressure may be protective against stroke by maintaining blood supply to the brain. Rigorous control of blood pressure is not recommended until after surgery.

Surgery:

Two types of surgeries are performed for Moyomoyo disease:

Direct re-vascularisationsurgery:A small blood vessel in the skin of the scalp is connected to a blood vessel in the brain. This provides an immediate alternate blood flow pathway to the brain. This procedure is difficult in children who have tiny scalp blood vessels.

Indirect re-vascularisation surgery:
In this surgery, there is no direct connection between bloodvessels. Instead, the tissues surrounding the blood vessels may be stitched to the brain surface. The surgical procedure is named depending on the tissue involved, as Encephalo (brain)-Duro (dura mater)-Arterio (blood vessel and surrounding cuff of tissue) -Myo (temporalis muscle)- Pericranio (pericranium) Synangiosis (joining of blood vessels). Thus, the various surgical options of indirect revascularisation include EDAMPS, EDAMS, EDAS, EMS etc. Multiple holes may be placed on the skull bone and sleeves of scalp tissue inserted to allow for blood vessels to grow into the brain. These tissues provide sources for new blood vessel formation and growth into the brain. The process is slower and may take upto 6months for blood flow to develop into the brain.

Surgery prevents the development of stroke and bleeding into the brain.

Therapy: Rehabilitation therapies including physiotherapy, speech therapy, occupational and cognitive therapy will be advised.

The Department of Neurosurgery and the Comprehensive Stroke Care Centre run clinics on Monday, and Wednesday. An appointment can be registered by contacting us at Phone: 0471 – 2524363 or you can E-mail us at strokesct@sctimst.ac.in

When you visit the clinic, all medical reports and scan images are expected to be presented. A diary of events recorded by you will help us understand your medical condition better.