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CASE REPORT Table of Contents   
Year : 2004  |  Volume : 14  |  Issue : 4  |  Page : 427-429
Images : Moya moya disease

Department of Radio diagnosis & Imaging, B. J. Medical College, Civil Hospital, Ahmedabad., India

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Keywords: Moya Moya Disease, Puff of Smoke, MRI

How to cite this article:
Jain A S, Goswami V G, Joshi M A, Patel J M, Shah A, Gupta A. Images : Moya moya disease. Indian J Radiol Imaging 2004;14:427-9

How to cite this URL:
Jain A S, Goswami V G, Joshi M A, Patel J M, Shah A, Gupta A. Images : Moya moya disease. Indian J Radiol Imaging [serial online] 2004 [cited 2021 Mar 1];14:427-9. Available from:

   Case report Top

8 yr old female patient had history of recurrent seizures and developed aphasia and quadriparesis after the seizure episode, at the age of 5 years.

MRI of the brain was performed in 0.5 Tesla Gyroscan. SE and FSE sequences were used to obtain T1, T2WI and FLAIR images in different planes. MR angiogram was performed using 3D phase contrast technique and MIP images were obtained.

MRI revealed areas of chronic infarcts in right frontoparietal region, left parietal region and right basal ganglia [Figure - 1]. There is nonvisualization of normal flow voids in the region of left internal carotid artery and right middle cerebral artery with multiple small flow voids in the region of the circle of Willis [Figure - 1],[Figure - 2],[Figure - 3].

MR angiography revealed narrowing of supraclinoid and intracavernous portion of left internal carotid artery and nonvisualization of right middle cerebral artery with extensive basal collateralization giving the characteristic "puff of smoke" appearance consistent with Moya Moya. Vertebrobasilar circulation appeared normal [Figure - 4]A,B. Patient had history of low grade fever in the past but there was no clinical or radiological evidence of tuberculosis. Hematological investigations revealed normal hematocrit, normal clotting time and sickling test was negative. As such, no primary cause was found for the patient's disease.

   Discussion Top

Moya Moya is a rare idiopathic vasoocclusive disease characterized by progressive irreversible occlusion of main blood vessels to the brain as they enter into the skull. The occlusive process stimulates the development of an extensive network of enlarged basal, transcortical and transdural collateral vessels [1],[2],[4],[6]. In Japanese, Moya Moya means hazy. The disease derives its peculiar name from the angiographic appearance of cerebral vessels in the disease that resembles "puff of smoke". Kudo first reported this pattern of collateral flow in 1960 [1] . The blockage tends to cause strokes or seizures [1],[6]The process of narrowing of cerebral vessels seems to be a reaction of brain blood vessels to a wide variety of external stimuli, injuries or genetic defects. In a series of patients the syndrome was associated with Asian birth (n=9), neurofibromatosis (n=12), Down syndrome (n=9) and following X-ray or chemotherapy treatment (n=10). There also seems to be an association with previous history of surgery for congenital heart disease suggesting that there may be a genetic defect in blood vessel wall in these patients. But more than half of the children seen with this disease have no cause for their Moya Moya syndrome. The process of blockage, once it begins tends to continue despite any known medical management unless treated with surgery [5],[6].

There is bimodal age presentation with first peak occurring in the first decade of life, associated with cerebral infarction as progressive carotid occlusion develops. Adult patient most often present in the fourth decade with intracranial hemorrhage arising from rupture of the delicate network of collateral vessels which is mostly intraparenchymal and may be intraventricular or occasionally subarachnoid [4].

CT scan of patients with Moya Moya syndrome is notable for cerebral infarctions most commonly in watershed vascular territories [1]

MRI not only reveals areas of infarctions but also allows direct visualization of these collateral vessels as multiple small flow voids at the base of brain and basal ganglia. MR angiography is used to confirm the diagnosis and to see the anatomy of the vessels involved. It typically reveals the narrowing and occlusion of proximal cerebral vessels and extensive collateral flow through the perforating vessels demonstrating the classic puff of smoke appearance. In the earlier stages of the disease, 3DFT TOF technique, to avoid severity of stenosis and in the late stages, given the slow flow 2DFT TOF technique is employed. IV contrast agent may be used to increase the intravascular contrast [4].

Often nuclear medicine studies such as SPECT are used to demonstrate the decrease in blood and oxygen supply to the areas of brain involved with the disease. The next step is for neurosurgeon to decide what type of operation is best suited for the child [6].

Many operations have been described and the most favoured are
" EDAS-encephaloduroarteriosynangiosis
" EMS-encephalomyosynangiosis
" STA-MCA-superficial temporal to middle cerebral artery anastomosis
" Multiple Burr holes.

The concept is to develop new and more efficient means of bring blood to the starved brain bypassing the areas of blockage [5],[6],[7]

   Conclusion Top

Acute stroke is an infrequent disease of pediatric age group patients. However, it is necessary to do an angiogram to rule out Moya Moya like vascular anomalies.

   References Top

1.Lee S H , Krishna C R, Robert A Z : Stroke, Cranial CT and MRI 4th edition,1999 ; 586  Back to cited text no. 1    
2.Olof F, Lars-Martin W, Pediatrics Neuroradiology in Grainger and Allison's diagnostic radiology 4th edition, Churchill Livingstone, 2001; 2506.  Back to cited text no. 2    
3.Rolf J, Dawn S ; Cranial and Intracranial pathology in Grainger and Allison's diagnostic radiology 4th edition, Churchill Livingstone, 2001; 2364.  Back to cited text no. 3    
4.Scott W. Atlas, MR angiography: Techniques and Clinical Applications MRI of the Brain and Spine 3rd edition, Lippincott Williams and Wilkins, Philadelphia, 2002, 1021-1023, 855-860.  Back to cited text no. 4    
5.Cerebrovascular and Stroke disease & Disorder in UCLA Neurosurgery , neurosun cerebrovascular/ Cerebrodis-,html/-11k  Back to cited text no. 5    
6.Moya moya syndrome .Boston Neurological Foundation .http: // www. boston - neurosurg,org/amphitheatre/online.html.Feb 15,1999  Back to cited text no. 6    
7.Piepgras D.G., Ueki.k, Moyamoya Disease in R.H.Wilkins & S.S. Rengachary neurosurgery, second edition, Mc GRAW HILL, Copyright 1996, 2125.  Back to cited text no. 7    

Correspondence Address:
A S Jain
14, Professor's Quarters, Civil Hospital Campus, Asarwa, Ahmedabad-380016
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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