Annals of Indian Academy of Neurology
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Year : 2015  |  Volume : 18  |  Issue : 3  |  Page : 353-354

Moyamoya disease; suspecting on conventional MRI brain without angiography

1 Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

Date of Submission19-Oct-2014
Date of Decision20-Nov-2014
Date of Acceptance04-Dec-2014
Date of Web Publication6-Aug-2015

Correspondence Address:
Sheffali Gulati
Department of Pediatrics, Division of Child Neurology, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.160080

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How to cite this article:
Israni A, Chakrabarty B, Gulati S, Kumar A. Moyamoya disease; suspecting on conventional MRI brain without angiography. Ann Indian Acad Neurol 2015;18:353-4

How to cite this URL:
Israni A, Chakrabarty B, Gulati S, Kumar A. Moyamoya disease; suspecting on conventional MRI brain without angiography. Ann Indian Acad Neurol [serial online] 2015 [cited 2021 Mar 9];18:353-4. Available from:

   Clinical Description Top

A 10 year old, premorbidly normal girl presented with history of weakness of right half of the body, right focal seizures, and absent speech for 1 week. There was no associated history suggestive of encephalopathy, cranial nerve deficit, impairment of sensations or coordination, or raised intracranial tension. There was no prior history suggestive of transient ischemic attacks. Family history was unremarkable.

Salient features on examination were right hemiparesis with complete aphasia. There was no evidence of any neurocutaneous stigmata or dysmorphism. There was no other abnormality on general physical or systemic examination.

Her magnetic resonance imaging (MRI) brain showed left middle cerebral artery territory (MCA) infarct with attenuation of left internal carotid artery (ICA) flow void and presence of multiple flow voids around left ICA, suggesting presence of multiple collaterals hinting to diagnosis of Moyamoya disease. Magnetic resonance (MR) angiography done subsequently was confirmatory, revealing stenosis of supraclinoid left ICA with typical "puff of smoke" appearance of collaterals [Figure 1]a and b.
Figure 1: MRI brain showing left middle cerebral artery (MCA) territory infarct with multiple small tortuous flow voids in suprasellar and perimesencephalic cistern (arrows in Figure 1a) and loss of internal carotid artery and MCA flow voids hinting towards an underlying Moyamoya disease. Magnetic resonance angiography is confirmatory (Figure 1b)

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   Discussion Top

Moyamoya disease is a cerebrovascular condition characterized by progressive stenosis of distal ICA and/or MCA and anterior cerebral artery with compensatory formation of a network of perforating blood vessels providing collateral circulation. Moyamoya, in Japanese, means "hazy." The disease derives its peculiar name from the angiographic appearance of cerebral vessels in the disease that resembles a "puff of smoke." 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. Conditions such as sickle cell anemia, neurofibromatosis-1, Down's syndrome, congenital heart defects, antiphospholipid syndrome, renal artery stenosis, and thyroiditis have been found to be associated with Moyamoya disease. [1],[2],[3] Stenosis of ICA is visualized as attenuated flow void on MRI brain and the frequent collaterals appear as multiple flow voids around the ICA flow void. The same can be visualized as stenosis of ICA and " puff of smoke" like multiple collateral vessels on cerebral angiography. [4],[5]

Digital subtraction angiography (DSA) is an invasive procedure compared to routine MRI brain. The emphasis here is on the fact that even in a child with unilateral focal neurological deficits, a meticulous evaluation of a non-invasive conventional MRI brain can points toward the diagnosis of Moyamoya disease. Early diagnosis and appropriate intervention is of paramount importance to prevent devastating long-term sequelae.

   References Top

Achrol AS, Guzman R, Lee M, Steinberg GK. Pathophysiology and genetic factors in moyamoya disease. Neurosurg Focus 2009;26:E4.  Back to cited text no. 1
Uchino K, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington state and California. Neurology 2005;65:956-8.  Back to cited text no. 2
Kuroda S, Houkin K. Moyamoya disease: Current concepts and future perspectives. Lancet Neurol 2008;7:1056-66.  Back to cited text no. 3
Fujita K, Shirakuni T, Kojima N, Tamaki N, Matsumoto S. Magnetic resonance imaging in moyamoya disease. No Shinkei Geka 1986;14:324-30.  Back to cited text no. 4
Yamada I, Suzuki S, Matsushima Y. Moyamoya disease: Comparison of assessment with MR angiography and MR imaging versus conventional angiography. Radiology 1995;196:211-8.  Back to cited text no. 5


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