Moyamoya disease

Case contributed by Bouhouche Abdeldjalil
Diagnosis almost certain

Presentation

Headache and transient ischaemic attacks since the age of 6 years.

Patient Data

Age: 9 years
Gender: Female
mri
This study is a stack
Axial
FLAIR
This study is a stack
Axial
DWI
This study is a stack
Axial
Gradient Echo
This study is a stack
Axial
T1 C+
This study is a stack
Coronal
MRA (TOF)
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Info

FLAIR images show bilateral multiple subcortical abnormal high signals located at the border zones between MCA and ACA as well as MCA and PCA and bilateral centrum semiovale, likely related to prior watershed ischaemic events.

No acute ischaemia or intracranial haemorrhage. 

MR angiography of the brain shows:

  • occluded supraclinoid segments of both internal carotid arteries

  • stenosis of the proximal segments of PCAs

  • the circle of Willis and M1 segment of MCAs are replaced by a tortuous network of collaterals along their expected paths

  • extensive lenticulostriate, thalamostriate and cortical collaterals (puff of smoke sign)

  • no aneurysmal dilatation or arteriovenous malformation

Case Discussion

MRI findings are reflective of moyamoya disease.

The patient underwent surgery and had multiple skull burr holes (8 burr holes on each side) to allow the formation of local collaterals.

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