Review article NEJM 2009; 360:1226-1237
1. There are many disease associations esp SCD, NF1, irradiation, and Down's syndrome, and less commonly cardiac anomaly, renal artery stenosis, giant cervicofacial hemangioma and hyperthyroidism.
2. It occurs throughout the world but especially in northeast Asia, 10x frequency as in N America
3. In Japan the responsible gene is 17q25. Other genetic association is RNF 213 autosomal dominant AD
4. The disease is more fulminant in children ; can be triggered by exertion, crying, hyperventilation, dehydration, or anaesthesia, presumably related to decreased CO2 tension in a compensated and fragile system.
5. Ischemic stroke is more common in children and adults, but hemorrhagic stroke occurs much more frequently in adults than in children
6. Headache, often refractory, occurs in two thirds, and may resolve within a year of revascularization surgery
7. Choreiform movement disorders in children occassionally resolve with revascularization surgery
8. Morning glory disc is associated and should prompt a consideration of the diagnosis
http://images.google.com/imgres?imgurl=http://www.nature.com/eye/journal/v21/n10/images/6702851f7.jpg&imgrefurl=http://www.nature.com/eye/journal/v21/n10/fig_tab/6702851f7.html&usg=__ycWmZRS6FxhD_gWU8YhCPzJQ1v8=&h=306&w=390&sz=112&hl=en&start=2&sig2=ybxbCPAci2WQ5GHSHYrFqQ&tbnid=oCbcKU1tcEBAIM:&tbnh=97&tbnw=123&prev=/images%3Fq%3Dmorning%2Bglory%2Bdisc%26gbv%3D2%26hl%3Den&ei=OGPJSY_1M5usMvW3wdYD
9. Pathology is smooth muscle proliferation and caspace dependent apoptosis. Some walls are stressed with microaneurysms which can explain hemorrhage.
10. EC IC bypass may be indicated in some . This is because the disease is inexorably progressive without surgical treatment and standard medical treatment does not work. Metaanalysis studies suggest treatment can halt progression.
11. Suzuki.Takaku developed a grading system for arterial changes
http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=119568&Ausgabe=235294&ProduktNr=224273&filename=119568.pdf
10. Cessation of smoking and birth control pills is indicated
11. "Ivy sign" radiographically may be seen in 70 % and resolve with bypass, consists of leptomeningeal enhancement with pial engorgement going into cortex. This is seen on FLAIR and post contrast images and resembles ivy creeping up on stone.
morning glory disc
1. There are many disease associations esp SCD, NF1, irradiation, and Down's syndrome, and less commonly cardiac anomaly, renal artery stenosis, giant cervicofacial hemangioma and hyperthyroidism.
2. It occurs throughout the world but especially in northeast Asia, 10x frequency as in N America
3. In Japan the responsible gene is 17q25. Other genetic association is RNF 213 autosomal dominant AD
4. The disease is more fulminant in children ; can be triggered by exertion, crying, hyperventilation, dehydration, or anaesthesia, presumably related to decreased CO2 tension in a compensated and fragile system.
5. Ischemic stroke is more common in children and adults, but hemorrhagic stroke occurs much more frequently in adults than in children
6. Headache, often refractory, occurs in two thirds, and may resolve within a year of revascularization surgery
7. Choreiform movement disorders in children occassionally resolve with revascularization surgery
8. Morning glory disc is associated and should prompt a consideration of the diagnosis
http://images.google.com/imgres?imgurl=http://www.nature.com/eye/journal/v21/n10/images/6702851f7.jpg&imgrefurl=http://www.nature.com/eye/journal/v21/n10/fig_tab/6702851f7.html&usg=__ycWmZRS6FxhD_gWU8YhCPzJQ1v8=&h=306&w=390&sz=112&hl=en&start=2&sig2=ybxbCPAci2WQ5GHSHYrFqQ&tbnid=oCbcKU1tcEBAIM:&tbnh=97&tbnw=123&prev=/images%3Fq%3Dmorning%2Bglory%2Bdisc%26gbv%3D2%26hl%3Den&ei=OGPJSY_1M5usMvW3wdYD
9. Pathology is smooth muscle proliferation and caspace dependent apoptosis. Some walls are stressed with microaneurysms which can explain hemorrhage.
10. EC IC bypass may be indicated in some . This is because the disease is inexorably progressive without surgical treatment and standard medical treatment does not work. Metaanalysis studies suggest treatment can halt progression.
11. Suzuki.Takaku developed a grading system for arterial changes
http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=119568&Ausgabe=235294&ProduktNr=224273&filename=119568.pdf
10. Cessation of smoking and birth control pills is indicated
11. "Ivy sign" radiographically may be seen in 70 % and resolve with bypass, consists of leptomeningeal enhancement with pial engorgement going into cortex. This is seen on FLAIR and post contrast images and resembles ivy creeping up on stone.
morning glory disc
1 comment:
Ivy sign refers to pial enhancement and slow flow. Usually does not mean coexistent meningitis is present. In one study perfusion imaging showed slow flow, which would not be expected to be present in meningitis or carcinomatosis.
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