  Case of the Week - Patient Summary 12927
Peer Reviewed and Certified - |
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| Demographics: 39 y.o. woman |
| History & Chief complaint: |
39 year old woman with a long history of TIA symptoms.
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| Physical exam and Laboratory: |
Non-contributory
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| 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
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| Differential Diagnosis: |
• Vasulitis
• Atherosclerotic vascular disease
• FMD with dissection
• Trauma
• Multiple Progressive Intracranial Arterial Occlusions
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| Diagnosis: |
 Moya-moya (Multiple Progressive Intracranial Arterial Occlusions)
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| Confirmed by: MRI, MRA and angiography |
| Treatment and Followup: |
Supportive
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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 |
