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| Appearance of Moyamoya disease (MRI) |
Moyamoya disease, coined by Suzuki and Takaku in 1969, is a cerebrovasculopathy of
unexplained etiology. The main and terminal veins of the internal carotid
artery undergo progressive vein occlusion. The internal carotid artery plays an
essential role in supplying blood to Cerebral areas of the brain.
Cerebrovasculopathy, also known as cerebral vasculitis (angiitis), involves the inflammation of the walls of the blood vessels in the brain and sporadically the spinal cord. The affect is destined to all kind of vessels like capillaries, arterioles, venules, arteries, and veins. The cerebral vasculitis interrupts the blood flow in the vessels as the oxygenated blood to the brain slows down and increases the chance of blood clot to form. Consequently, the brain experiences diverse neurological symptoms, phenomena of a mass lesion, or multiple sclerosis. To compensate the shortage of blood flow, new collateral vessels portrays the “puff of smoke”, which is the appearance of the Moyamoya disease.
Cerebrovasculopathy, also known as cerebral vasculitis (angiitis), involves the inflammation of the walls of the blood vessels in the brain and sporadically the spinal cord. The affect is destined to all kind of vessels like capillaries, arterioles, venules, arteries, and veins. The cerebral vasculitis interrupts the blood flow in the vessels as the oxygenated blood to the brain slows down and increases the chance of blood clot to form. Consequently, the brain experiences diverse neurological symptoms, phenomena of a mass lesion, or multiple sclerosis. To compensate the shortage of blood flow, new collateral vessels portrays the “puff of smoke”, which is the appearance of the Moyamoya disease.
Mass lesion
Brain lesion is an area
in the brain where tissue has been damaged through injury or disease.
Multiple sclerosis
Sclerosis is a disease
in which the insulating covers of nerve cells in the brain are damaged.
Moyamoya disease is a
progressive condition, meaning that symptoms worsen over time.
-
Stroke
-
Transient
ischemic attack (TIA)
-
Seizures
-
Headaches
There are four
components to diagnose Moyamoya disease:
-
Detailed
neurological and genetic evaluation to differentiate the specific state or form
of the disease. (Primary or secondary)
-
Brain imaging
allows the acute setting to examine such evidence of stroke or ischemic brain
injury.
-
Vessel-specific
imaging: CT, MR angiography, and cerebral angiography.
-
Special
neuroimaging modalities, CT, MR perfusion scans, Diamox SPECT scans, and PET
scans, are often requisite to assess and quantify the degree of hypoperfusion –
the lack of blood supply of the brain.
The treatment of the Moyamoya disease demand for great deal of effort from various fields such as neurosurgery, neuroradiology, genetics, neuropsychologists, physical, occupational and speech therapists. The main purpose of the treatment is to prevent blood clotting in the thickened vessel walls. This is done by the use of anti-platelet therapies or specific revascularization procedures.
Bibliography
KSJ
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