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Type:
Educational Exhibit
Keywords:
Dysplasias, Dissection, Arteriosclerosis, Contrast agent-intravenous, Ultrasound, MR-Angiography, CT-Angiography, Vascular, Head and neck, CNS
Authors:
I. Etxegoien Juaristi, C. Biurrun Mancisidor, J. A. Vega, E. Pardo Zudaire, G. Vega-Hazas Porrúa, F. J. Borja Consigliere, A. Borja Consigliere; San Sebastian/ES
DOI:
10.1594/ecr2015/C-1412
Background
They represent a heterogeneous group of diseases that affect the wall of supraaortic arteriopathies and intracranial arteries.
- CLASSIFICATION:
1.
Non-Inflammatory:
1.1.
Atherosclerosis:
- Atheroma is an accumulation of cells,
lipids,
extracelular matrix and necrotic debris within the intima.
- Diffuse intimal thickening can result on outward and constrictive remodeling of the vessel wall.
1.2.
Arterial dissection/intramural hematoma:
- break in the arterial wall causes blood to accumulate in a false lumen (located within the media).
- imaging findings: long segment stenosis in true lumen,
high T1 signal from methemoglobin in false lumen.
1.3.
Fibromuscular dysplasia:
- Rare arteriopathy affecting small to medium sized vessels.
- Abormal fibrous/collagen proliferation in the media (type 1,
85%),
intima (type 2,
<10%) and adventicia (type 3,
<5%).
- More frecuent in women 20-40 years old.
- 60-75% in renal arteries.
- 25-30 % in cervicocephalic arteries.
- Imaging shows a long segment area of multifocal stenosis and dilatatoins,
"string of beads" sign.
- Increases the risk of stenosis,
dissection or embolus formation.
1.4.
Reversible vasoconstriction syndrome:
- Severe "thunderclap" headache.
- Transient multifocal segmental vasoconstriction of cerebral arteries.
- Focal neurologic symptoms with or without strokes.
- Convexal SAH are common.
- Spontaneously reversible in weeks to months.
- Treatment:nimodipine.
1.5.
CADASIL:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy.
- Age of onset 40-60.
- Characterized by migraine,
depression,
progressive dementia,
inability to walk and death.
- Imaging findings:
- diffuse white matter involvement not typical of common demyelinating diseases and multiple subcortical infarcts more than expected for age.
- T2 hyperintensities in the anterior temporal and superior frontal white matter and external capsule-insula are non-specific but highlly characteristic.
2.
Inflammatory:
2.1.
Vasculitis:
- Heterogeneous group of inflammatory diseases that primarily affect the small leptomeningeal and parenchymal vessels of the brain including infection,
ionizing radiation,
cocaine ingestion,
paraneoplasic syndrome and autoinmune disease (primary CNS angiitis or systemic).
- Imaging findings between these ethiologies is indistinct and characterized by stenosis with vessel wall thickening.
Dissection,
fusiform aneurysms and infarcts in multiple vascular territories are also commonly seen.
- Treatment: immunosuppresive agents.
2.2. Carotidinia:
- Idiopathic neck pain syndrome associated with tenderness to palpation.
- MRI may show carotid wall thickening with enhacement.
- Diagnosis is clinical.
3.
Unknown:
3.1.
Moya-moya disease:
- Idiophatic cerebrovascular occlusive disorder characterized by progresive stenosis of bilateral ICA and collateral vessel formation.
- "Puff of smoke sign" in cerebral arteriography corresponding to collateral vessels:tangle of tinny vessels.
3.2.
Moya-moya syndrome: moya-moya vasculopathy with well recognized associated conditions.