Moyamoya
Disease
It is caused by blocked arteries at base of brain.
The word "moyamoya" implies
"smoke puff". It describes appearance of tiny vessels narrow and
become blocked, which can result in stroke.
Blood supply of brain
The cartiod artery is large whose pulse can be felt
on both sides of neck. It starts at aorta and divides near larynx into external
and internal carotid arteries. The external artery supply blood to face and
scalp. Internal arteries supplies blood to front and side of brain.
What is moyamoya disease?
It is a chronic and progressive narrowing of
internal carotid arteries at base of brain which divides in middle and anterior
cerebral arteries. The walls of arteries become thick, which narrows the inside
diameter of vessel. The narrowing result in complete block and stroke.
To reduce the narrow arteries, the brain create a
collateral blood vessels in attempt to deliver rich-oxygen blood to the
deprived areas of brain. These small vessels, when seen in angiogram looks
hazy. In this condition, the blood
vessels are more weaker than normal blood vessels. The small collaterals can
break and bleed, which can cause brain hemorrhage. The disease can affect front
and side areas of brain.
The progression of the disease follow typical course
and are as follows:
· Stage
1- Internal carotid arteries get narrowed.
· Stage
2- Development of any moyamoya vessels at the base of brain.
· Stage
3-Internal cartoid artery narrowing and moyamoya vessels gets intensified.
· Stage
4- Increased collateral vessels from scalp and minimizing moyamoya vessels.
· Stage
5- Narrowing of internal carotid artery and reductionof moyamoya vessels.
· Stage
6- The disappearance of moyamoya vessels, internal carotid artery is completely
blocked.
Once the process of blockage begins, it continues.
There is no medicine to reverse the effect. If had a major stoke or bleeding
occurred, the patient will have permanent loss of function.
Symptoms
The symptoms include:
· Mini
stroke.
· Ischemic
stroke.
Causes
The real cause of moyamoya disease is not known. The
inheritance can be a cause of the disease.
Diagnosis
If you are having symptoms like stroke, then you
will be referred to neurosurgeon. The surgeon will check about the possibility
of some symptoms, any medical problems, current medications ,family history.
Physical exam, diagnostic tests like mri, ct scan, angiogram, etc. will also be
performed. To confirm the diagnosis of moyamoya disease, an angiogram is
required.
Surgery
The surgery is recommended for moyamoya patient with
progressive TIAS or stroke. There are various kinds of surgeries available.
Cerebral
bypass procedure: It is a method of revascularization. It
involves connecting a blood vessel from the outside of brain to inside vessel of brain, so that the reroute
of blood flow around an artery which is blocked, damaged or narrowed. Most
common bypass procedure is STA-
MCA(Superficial Temporal Artery to Middle
Cerebral Artery),this may help in instant improvement in the blood flow. If
suppose, the superficial temporal artery is not available then another artery
can be used.
EDAS(Encephalo Duro Arterio Synangiosis) procedure:
It is a process of revascularization in which the superficial temporal artery
is kept in contact with the surface of brain. A hole is made in skulljust below
the artery. The artery is then stitched to the surface of brain and the bone
replaced. After sometime, small arterial vessels form to the brain.
EMS(Encephalo Myo Synangiosis)
procedure: It is a process in which the temporal muscle which is on side of the
head is dissected, through an opening of skull and is placed onto the surface
of brain. Then new vessels form between blood muscles and brain.
EDAMS (Encephalo Duro Arterio Myo Synangiosis)
procedure: It is a combination of the technique of EDAS and EMS.