Moyamoya Disease MRI

A 31 yo known case of recurrent episodes of ishemia under evaluation.MRI study brain Axial FLAIR image of brain with non contrast 3 D TOF MR Angiography shows:
Bilateral cortical border zone infarcts.
Severe stenosis involving intra cranial portions of both the ICAs. MCAs not visualised along with its cortical branches. Distal portion of PCAs not visualised along with its cortical branches. Multiple vessels from proximal portion of PCAs are the thalamo striate perforators giving is so called Puff of Smoke appearance contributing to basal cerebral anastomotic or collateral network.

Moyamoya disease 

A bilateral steno occlusive disease of the intra cranial internal carotid artery.
Moyamoya is a Japanese word for a "puff" or "cloud of smoke" , and it has been used to refer to an extensive basal cerebral network of small anastomotic vessels at the base of the brain around and distal to the circle of Willis secondary to segmental stenosis or occlusion of the terminal parts of both internal carotid arteries.  The basal vascular network is contributed by lenticulostriate, chorioidal, thalamoperforating, premammilary and thalamogeniculate arteries, as well by to unnamed branches arising directly from the circle of Willis and meningeal arteries via ECA.
Pseudo aneurysms and micro aneurysm are well reported along these collaterals and circle of Willis. Pathogenesis of MD is not well understood. Various theories of inflammatory and immunologic mechanisms remain unproven. Very high concentration of basic fibroblast growth factor (bFGF) with high angiogenic activity in Csf samples of patients typical imaging findings of Moyamoya disease. There are strong evidences to support hereditary and familial factors especially among the Japanese.

Clinical manifestations of MD includes secondary to ischemia and or bleed. Ischemic symptoms common in children. Infarcts are more common along cortical border zones. Intracranial bleed in the form of subarachnoid bleed,  intra parenchymal or intraventricular bleed common in adults. Causes of intracranial haemorrhage in MD are rupture of dilated fragile collaterals or rupture of aneurysms along the circle of Willis and basal cerebral network of collaterals.


Reference: MOYAMOYA DISEASE: CLINICAL AND ANGIOGRAPHIC FEATURES Dragan Stojanov ,  Petar Bošnjaković,  Zoran Milenković, Nebojša Stojanović CLINICAL FEATURES OF MOYAMOYA DISEASE yong seung Hwang,


Other cases of Moya moya disease:
Moyamoya disease DSA 
Intraventricular-hemorrhage-in-moyamoya

Intraventricular hemorrhage in Moyamoya

A  30 years old male brought to casually with a CT showing intra ventricular bleed for further evaluation and management.

MRI  Axial T1w images of brain shows an intra ventricular bleed in left lateral ventricle (yellow arrow), T1 bright meth Hb – a sub acute stage blood degradation product.

3 D TOF MR Angiography of Brain shows non visualization of intracranial portion of both the ICA. Both the MCA show poor flow related signal with marked sparsity of cortical branches of MCA on either side. Lateral and oblique view showing collaterals from PCA giving puff of smoke appearance (Red arrow). 

Diagnosis: Intraventricular hemorrhage in Moyamoya disease.

Moyamoya disease is a bilateral steno occlusive disease of the intra cranial internal carotid artery. Moyamoya is a Japanese word for a "puff" or "cloud of smoke" , and it has been used to refer to an extensive basal cerebral network of small anastomotic vessels at the base of the brain around and distal to the circle of Willis secondary to segmental stenosis or occlusion of the terminal parts of both internal carotid arteries.  The basal vascular network is contributed by lenticulostriate, chorioidal, thalamoperforating, premammilary and thalamogeniculate arteries, as well by to unnamed branches arising directly from the circle of Willis.
Pseudo aneurysms and micro aneurysm are well reported along these collaterals and circle of Willis. Pathogenesis of MD is not well understood.
Various theories of inflammatory and immunologic mechanisms remain unproven. Very high concentration of basic fibroblast growth factor (bFGF) with high angiogenic activity in Csf samples of patients typical imaging findings of Moyamoya disease.
There are strong evidences to support hereditary and familial factors especially among the Japanese.

Clinical manifestation of MD include ischemic symptoms common in children where as intracranial bleed in the form of subarachnoid bleed,  intra parenchymal or intraventricular bleed common in adults.

Causes of intracranial haemorrhage in MD are rupture of dilated fragile collaterals or rupture of aneurysms along the circle of Willis and basal cerebral network of collaterals.

Reference: MOYAMOYA DISEASE: CLINICAL AND ANGIOGRAPHIC FEATURES Dragan Stojanov ,  Petar Bošnjaković,  Zoran Milenković, Nebojša Stojanović CLINICAL FEATURES OF MOYAMOYA DISEASE yong seung Hwang,

Moyamoya Disease DSA

Cerebral Angiogram : Left Internal Carotid Artery injection (AP view) show stenosis involving cavernous portion with prominent collateral vessels giving puff of smoke appearance of Moyamoya disease, a rare idiopathic disorder characterized by progressive narrowing of the distal internal carotid arteries and their branches.
It is typically seen in children, although rarely reported in adults.
As the carotid artery is compromised, there is progressive enlargement of the collateral circulation, especially among the lenticulostriate vessels. This results in a characteristic angiographic picture of a blush or "puff of smoke" in the area of the lenticulostriate vessels.
Typically presents with recurrent infarcts where as in some cases, the fragile collaterals can rupture resulting in intra cranial bleed.