About Moyamoya Disease
In 1950's, Japanese leading neurosurgeons
at that time began to notice the new clinical
entity of moyamoya disease. Since its etiology
was unknown, it was named in various ways,
such as moyamoya disease, chiri-chiri disease,
etc. Moyamoya disease is now officially named
in Japan as "the spontaneous occlusion
of the circle of Willis."
The terminal portions of the intracranial
internal carotid arteries progressively become
stenotic (narrowing) or occlusive. Due to
reduced blood flow to the brain, tiny collateral
vessels at the base of the brain enlarge
to become as collateral pathways. These vessels
are called "moyamoya vessels" because
the angiographic appearance of these vessels
resemble the "cloud" or "puff"
of the cigarette smoke, which is described
as "moya-moya" in Japanese language
(Suzuki J, Takaku A, 1969).
About the name of moyamoya disease
Moyamoya Vessels (Abnormally proliferating
arteries?)
In 1960's, moyamoya disease became
the main
interest for the neurosurgeons and
neurologists
because this disease was prevalent
solely
in Japan and/or Japanese ethnicity.
In 1994,
the number of the annual patients having
moyamoya disease was estimated about
3,900.
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Symptoms of moyamoya disease include brain
ischaemia and hemorrhage. There are two peaks
in age distribution: below 10 and at 30-40
years old, with the highest peak in the first
decade and lower peak in the adults. Under
the age of 10, brain ischemia (transient
ischemic attack and cerebral infarction)
is common whilst brain hemorrhage is more
common at the age of 30-40 years old.
Age Distribution of Moyamoya Disease
Incidence of moyamoya disease among
females
is 1.8 times more than that among males.
Although there have been many reports
on
moyamoya disease all over the world
including
Asia, Europe, Australian, and the United
States, it is most common among Japanese.
About Korean Moyamoya Disease
Worldwide Distribution of Moyamoya
Disease
Its etiology is still unknown, although some
investigators regard it as cerebral arteritis.
Moyamoya disease was initially though to
be congenital. It had been believed in 1980's
that antecedent infection causes the disease
(acquired theory). However, about 10% of
familial incidence of the disease and high
incidence among Japanese indicate congenital
factors contributing to the disease (congenital
theory). It is believed now that some moyamoya
disease have multiple genetic factors.
Brain ischemia includes transient ischemic
attacks (TIAs) and cerebral infarction. The
former means that all symptoms disappear
within 24 hours, but commonly within 15-30
minutes. When whistling, puffing hot soup,
playing a flute, cerebral arteries become
spastic leading to cerebral ischaemia. Altered
consciousness, weakness of the extremities,
weakness of one side of the body, speech
disturbance (aphasia), involuntary movements,
headache, convulsion, sensory abnormality,
visual disturbance or mental alteration may
be observed.
When brain hemorrhage occurs, according
to
the location and size of the hemorrhage,
the patients develop altered consciousness,
weakness of arms and legs, sensory
disturbance,
convulsion (seizure), or mental alteration.
If hemorrhage is large enough, it is
occasionally
fatal.
When you first heard of the name of
"moyamoya
disease"
General information of moyamoya disease
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