Moyamoya disease (MMD)
Bilateral progressive steno-occlusive changes of
supraclinoid ICA segments involving carotid fork
region; Unilateral involvements (overall 20%) are
also seen
“Puff of Smoke”; (1) Classical basal collaterals
recruiting leptomeningeal vessels and deep
parenchymal vessels of striatum (2) Collateral
vessels are formed during prenatal period
Pathophysiology; Fibrocellular intimal thickenings
with wavy internal elastic lamina and thinning of
media
Moyamoya disease (MMD)
Etiology; Unknown
Some Genetic Predispositions; 10% of familial
incidence, hereditary and multifactorial, autosomal
dominant familial MMD with reduced penetrance
Genetically Susceptible Loci; 3p, 6p, 17q, and band
8q23
Angiographic Classification of MMD; Suzuki & Takaku
since 1969, but often fail to represent the disease
progression
Controversies about its etiologies as
Moyamoya disease (MMD)
Moyamoya disease (MMD)
Moyamoya disease (MMD)
(ORM) annexations with
(primitive ventral
ophthalmic artery) and
(primitive olfactory artery):
Visualization of Embryonic Remnant Vascular Configurations
Moyamoya disease (MMD)
dwindling SOD of Stapedial Artery anastomosis with
Ophthalmic Artery
which passes around the
dorsal side of the optic nerve.
-
Progression of Unilateral MMD: A
Clinical Series. Kelly ME et al.
Presence of
are important
prognostic factors of progression
“True definition of Unilateral MMD is
Unclear”
Anatomic configuration of the cerebral vessels of Unilateral
MMD :
Immediate Surgery for Moyamoya Syndrome?
Roach ES.
March 2002 to April 2004
Angiographic Analysis: Independently Reviewed by Two
Experienced Neuroradiologists (C.J.I & W.Y.C.)
Based upon the Significant Contributions by Dorcas Hager
Padget (1906-1973)
All 20 patient showed Asymmetric Contralateral Carotid
Fork Abnormalities with Typical MMD Collaterals
POlfA with or without ORM (ophthalmic rete mirabile):
8/19, 42.1%
Ophthalmic Ethmoidal Collaterals: 2/19, 10.5%
Callosal Artery Collaterals: 3/19, 15.8%