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Scientific Article |Figure 1.Presence of intraventricular hemorrhageFigure 2.Note increased collateral circulation onCT-AngiogramFigure 3.Stenosis of supraclinoid Internal CarotidArteryof age and adults around 40 yearsof age. 13-16 Children present withtransient ischemic effects exacerbatedby hyperventilation. 6 In addition,children may present with seizuresor choreiform movements dueto basal ganglia involvement. 18,19Adults present with altered mentalstatus due to intraventricular orintraparenchymal bleeding, howeverresearch suggests ischemia maybe observed in the majority ofall patients, regardless of age. 6,12Headache, similar to migraines,is a common symptom of MMDas a result of dural nociceptorstimulation from vessel dilation. 17Several risk factors for the diseasehave been proposed including,Trisomy 21, Neurofibromatosis 1,history of radiation to head and neck,and various forms of cerebrovascularinflammatory disease. 7 Etiology ofMMD remains unclear, howeverelevated levels of basic-fibroblastgrowth factor (b-FGF) are found inthe CSF of MMD patients. Increasedactivity of b-FGF and its receptor inthe superficial temporal artery implyangiogenic and cytokine participationin the disease process. 6 FamilialMMD may be linked to genetic lociof chromosomes 3, 6, and 17. 6The gold standard for diagnosingMMD requires catheter cerebralangiography. Cases shouldmeet the following findings:1. Bilateral symmetrical stenosesor occlusion of the terminalinternal carotid arteriesand proximal portionsof the anterior and/ormiddle cerebral arteries.2. Several enlargedlenticulostriate andthalamoperforating arteries(this criterion demonstrates“puff of smoke” appearance).3. Several transdural,leptomeningeal, and pialcollateral vessels. 6,9Bilateral involvement is considereda definite case, however unilateralinvolvement affects the contralateralside in 40 percent of patients. 21,22CT and MRI illustrate multipleinfarcts in more than 80 percentof patients, with intraventricularhemorrhage encountered mostoften. In addition, PET scan showsdecreased cortical blood flow fromnarrowed of cerebral blood vessels. 9At this time, no medicaltherapy has been established toalter progression of MMD. 12 Antiplatelettherapy can reduce ischemicsymptoms of emboli formed at sitesof stenosis, and calcium channelblockers can abort headache andmay reduce future transient ischemicattacks. 12 Surgical managementis performed to increase cerebralblood flow. A recent review ofsurgical management concluded thatdirect bypass surgery (STA-MCAanastamoses) or indirect bypasssurgery (laying a vascularizedsoft-tissue flap on the brain surface)improves revascularization. 11 Theseinterventions can prevent furtherischemic or hemorrhagic events.Once considered a diseaseexclusive to Asian patients, MMDis now observed in American andEuropean populations. Rates inAmerica are reported to be 0.086cases per 100,000 persons. 20 The agedistribution is bimodal – seen at fiveyears of age and in the mid-40’s. 13-16Adults are seven times more likelyto have hemorrhage than children.Consequently, we encouragephysicians to include MMD aspart of their differential in patients18 West Virginia Medical Journal
| Scientific ArticleFigure 4.“Puff of smoke” appearancewith hemorrhagic or ischemicevents, particularly in youngerpatients, regardless of ancestry.15. Han DH, Nam DH, Oh CW. Moyamoya disease inadults: characteristics of clinical presentation andoutcome after encephalo-duro-arteriosynangiosis.Clin Neurol Neurosurg1997;99:Suppl 2:S151-S15516. Han DH, Kwon OK, Byun BJ, et al. A co-operativestudy: clinical characteristics of 334 Koreanpatients with moyamoya disease treated atneurosurgical institutes (1976-1994). ActaNeurochir (Wien) 2000;142:1263-127317. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ,Cho BK. Headache in pediatric moyamoyadisease: review of 204 consecutive cases. JNeurosurg 2005;103:Suppl:439-44218. Scott RM, Smith JL, Robertson RL, Madsen JR,Soriano SG, Rockoff MA. Long-term outcome inchildren with moyamoya syndrome after cranialrevascularization by pial synangiosis. J Neurosurg2004;100:Suppl:142-14919. Parmar RC, Bavdekar SB, Muranjan MN, LimayeU. Chorea: an unusual presenting feature inpediatric Moyamoya disease. Indian Pediatr2000;37:1005-100920. Uchino K, Johnston SC, Becker KJ, Tirschwell DL.Moyamoya disease in Washington State andCalifornia. Neurology 2005;65:956-95821. Kelly ME, Bell-Stephens TE, Marks MP, Do HM,Steinberg GK. Progression of unilateralmoyamoya disease: a clinical series. CerebrovascDis 2006;22:109-11522. Smith ER, Scott RM. Progression of disease inunilateral moyamoya syndrome. Neurosurg Focus2008;24:E17-E17.References1. Yonekawa, Y, Kahn, N. Moyamoya Disease.Ischemic Stroke: Advances in Neurology; 2003:92:113-118.2. Khan, N, Yonekawa Y. Moyamoya angiopathy inEurope. Acta Neurochir. 2005; 94:149-152.3. Yonekawa, Y, Nobuyoshi O, et al. Moyamoyadisease in Europe, past and present status.Clinical Neurology and Neurosurgery. 1997;99:S58-S60.4. Chiu D, Shedden P, Bratina P, Grotta J. Clinicalfeatures of moyamoya disease in the UnitedStates. 1998;29:1347-1351.5. Yonekawa Y, Taube E. Moyamoya disease: status1998. Neurologist. 1999. 5:13-23.6. Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoyadisease. Neuropathology. 2000; 20:S61-S64th7. Greenberg, M. Handbook of Neurosurgery 6Edition. 2006;30:892-894.8. Rengachary, S, Wilkins, R, Wonsiewicz, M,Ramos, M. Neurosurgery. 2 nd , ed. 1996; 2:2090-2092.9. Taki, W, Yonekawa, Y, et al. Cerebral Circulationand Metabolism in Adults’ Moyamoya Disease–PET Study. Acta Neurochir. 1989. 100:150-154.10. Chang S, Steinberg G. Surgical management ofmoyamoya disease. Contemporary Neurosurgery.2000. 10: 1-9.11. Baaj A, et al. Surgical management of Moyamoya.Neurosurgical Focus. 2009. 26; 4: 1-7.12. Scott, R.M., Smith, E.R. Moyamoya disease andmoyamoya syndrome. New England Journal ofMedicine. 2009. 360: 1226-1237.13. Baba T, Houkin K, Kuroda S. Novelepidemiological features of moyamoya disease. JNeurol Neurosurg Psychiatry 2008;79:900-90414. Wakai K, Tamakoshi A, Ikezaki K, et al.Epidemiological features of moyamoya disease inJapan: findings from a nationwide survey. ClinNeurol Neurosurg 1997;99:Suppl 2:S1-S5JoinWESPAC Now!West Virginia Medical PoliticalAction CommitteeVisit www.wvsma.com orCall 304.925.0342, ext. 25March/April 2012 | Vol. 108 19
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