MedPix® Patient Chart - Case No: 12927 :: Imaging - Review Images

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History

Age: 39 :: Gender: woman

Patient History

39 year old woman with a long history of TIA symptoms.

Exam


Physical Exam and Laboratory

Non-contributory


Findings


Summary of Findings

• Angiogram:
Occlusion of right ICA in neck
High grade stenosis left ICA just proximal to bifurcation
Leptmeningeal collateral vessels from posterior circulation and lenticulostriate arteries.

• MRI & MRA
Occlusion of right ICA
Enlarged collateral vessels in the Sylvian fissures
Chronic basal ganglionic infarcts.

• CTA
Occlusion of right ICA in neck
High grade stenosis left ICA just proximal to bifurcation



Diffferential


Differential Diagnosis

• Vasulitis
• Atherosclerotic vascular disease
• FMD with dissection
• Trauma
• Multiple Progressive Intracranial Arterial Occlusions


Diagnosis


Case Diagnosis

Dx: Moya-moya (Multiple Progressive Intracranial Arterial Occlusions)


Dx Confirmed by: MRI, MRA and angiography

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Followup


Followup and Treatment

Supportive

Topic


Moya-moya

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slow progressive occlusion of the major trunks of the intracerebral arteries, beginning with the carotid in the cavernous sinus, the abnormal small distal vessels are secondary collaterals, pathologically the occluded vessels show intimal thickening and medial thinning (without inflammation), high incidence in Down syndrome, first decade or fourth decade, F > M (48:1), symptoms include TIA and stroke induced by crying, coughing or straining, seizures, in adults hemorrhage is common, from the fragile [collateral] vessels into the basal ganglia, thalamus, and ventricle, SAH also occurs, the association with AVM's is rare, but aneurysms occur, prognosis is poor

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History:
39 year old woman with a long history of TIA symptoms.

Exam:
Non-contributory

Findings:
• Angiogram:
Occlusion of right ICA in neck
High grade stenosis left ICA just proximal to bifurcation
Leptmeningeal collateral vessels from posterior circulation and lenticulostriate arteries.

• MRI & MRA
Occlusion of right ICA
Enlarged collateral vessels in the Sylvian fissures
Chronic basal ganglionic infarcts.

• CTA
Occlusion of right ICA in neck
High grade stenosis left ICA just proximal to bifurcation




Differential:
• Vasulitis
• Atherosclerotic vascular disease
• FMD with dissection
• Trauma
• Multiple Progressive Intracranial Arterial Occlusions

Diagnosis:
Moya-moya (Multiple Progressive Intracranial Arterial Occlusions)
Confirmed by:MRI, MRA and angiography

Treatment and Followup:
Supportive

Discussion:

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Case Contributor and Editor
Topic Author: James George Smirniotopoulos, M.D.
Submitted by: Steven J Goldstein - Author Info
Case/Image Editor: James G. Smirniotopoulos, M.D. - Editor Info
Case Accepted: 2009-04-15 15:34:16-04 :: Revised: :: Submitted:
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