1. EMedicine - Moyamoya Disease : Article By Roy Sucholeiki, MD An in depth look at moyamoya disease, an introduction, clinical features, differentials, work up, Category Health Conditions and Diseases Rare Disorders Moyamoya......moyamoya disease moyamoya disease (MMD) is a progressive occlusive disease ofthe cerebral vasculature with particular involvement of the circle of Willis http://www.emedicine.com/neuro/topic616.htm

document.write(''); (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Neurology Pediatric Neurology Moyamoya Disease Last Updated: January 18, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: arterial occlusive disease, primary cerebral, puff of smoke AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography Author: Roy Sucholeiki, MD , Assistant Professor, Department of Neurology, Loyola University Chicago Medical Center Coauthor(s): Jasvinder Chawla, MD , Staff Physician, Department of Neurology, Loyola University of Chicago Medical Center Roy Sucholeiki, MD, is a member of the following medical societies: American Academy of Neurology, and American Epilepsy Society Editor(s): Robert Rust, Jr, MD , Thomas E Worrell Jr Professor, Departments of Neurology and Pediatrics, University of Virginia Hospital and Clinics; Francisco Talavera, PharmD, PhD

2. NINDS Moyamoya Disease Information Page Information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).Category Health Conditions and Diseases Rare Disorders Moyamoya......moyamoya disease information page compiled by the National Institute of NeurologicalDisorders and Stroke (NINDS). More about moyamoya disease, http://www.ninds.nih.gov/health_and_medical/disorders/moyamoya.htm

National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health organizations More about Moyamoya Disease Studies with patients Research literature Press releases Search NINDS... (help) Contact us My privacy NINDS is part of the National Institutes of Health NINDS Moyamoya Disease Information Page Reviewed 12-14-2001 Get Web page suited for printing Email this to a friend or colleague Table of Contents (click to jump to sections) What is Moyamoya Disease? Is there any treatment? What is the prognosis? What research is being done? ... Related NINDS Publications and Information What is Moyamoya Disease? Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or occlusion of major blood vessels leading into the brain, and the formation of abnormal blood vessels called moyamoya vessels Is there any treatment? There is no cure for moyamoya disease. Treatment is symptomatic and supportive. Individuals experiencing TIAs and stroke may be given aspirin, vasodilators, or anticoagulants to reduce the risk of future attacks. There are several different types of revascularization (restoration of blood supply) surgery that may be performed in some cases. Children usually respond better to revascularization surgery than adults.

3. Moyamoya Disease Chorus offers a brief outline about the rare disease. Includes links to other areas of the nervous system. moyamoya disease. progressive obstructive/occlusive cerebral arteritis http://chorus.rad.mcw.edu/doc/01076.html

CHORUS Collaborative Hypertext of Radiology Nervous system Feedback Search moyamoya disease progressive obstructive/occlusive cerebral arteritis affecting distal ICA at bifurcation into its branches (ant. 2/3 of circle of Willis) usually involving both hemispheres etiology: unknown age: predominantly in children and young adults. clinical: headaches behavioral disturbances recurrent hemiparetic attacks radiographic findings: large network of vessels in BG (puff of smoke) and upper brainstem fed by basilar a. ACA MCA (dilatation of lenticulo-striates and ant. choroidal a.) anastomosis between leptomeningeal and dural meningeal arteries complication: subarachnoid hemorrhage (occasionally) Jon T. Lee, MD - 6 February 1995 Last updated 14 March 2001 Medical College of Wisconsin

4. Children's Hemiplegia And Stroke Association What is moyamoya disease? moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the http://www.chasa.org/moyamoya.htm

Home About CHASA Support Newly Diagnosed ... Contact CHASA moyamoya disease What is Moyamoya Disease? Is there any treatment? What research is being done? What is Moyamoya Disease? Is there any treatment? There is no cure for moyamoya disease. Treatment is symptomatic and supportive. Individuals experiencing TIAs and stroke may be given aspirin, vasodilators, or anticoagulants to reduce the risk of future attacks. There are several different types of revascularization (restoration of blood supply) surgery that may be performed in some cases. Children usually respond better to revascularization surgery than adults. What research is being done? Within the NINDS research programs, moyamoya disease is addressed primarily through studies associated with TIA and stroke. NINDS is the leading supporter of research on stroke and TIA in the United States and sponsors studies ranging from clinical trials to investigations of basic biological mechanisms. This article reprinted with permission from the National Institute of Neurological Disorders and Stroke.

5. 5-5-NSF-TOC TOPIC SECTION moyamoya disease. Introduction by Topic Editor Haruhiko Kikuchi, M.D. http://www.neurosurgery.org/journals/online_j/november98/5-5-NSF-TOC.html

Table of Contents for November 1998, Volume 5, Issue 5 ISSN #1092-0684 PREVIEW SECTION January draft of the Table of Contents for the Journal of Neurosurgery Article #1: Microvascular decompression of cranial nerves: lessons learned after 4400 operations. ... Andrew C. Papanicolaou, Panagiotis G. Simos, Joshua I. Breier, George Zouridakis, L. James Willmore, James W. Wheless, Jules E. C. Constantinou, William W. Maggio, and William B. Gormley TOPIC SECTION: Moyamoya Disease Introduction by Topic Editor: Haruhiko Kikuchi, M.D. Moyamoya disease in the midwestern United States. Nicholas M. Wetjen, P. Charles Garell, Nicholas V. Stence, and Christopher M. Loftus 2. Role of basic fibroblast growth factor in the pathogenesis of moyamoya disease. ... : Preoperative left common carotid artery angiogram showing the stenotic segment of the supraclinoid ICA. Moyamoya-like collateral branches can be seen throughout the region of the basal ganglia. The posterior branch of the STA (arrows) can be seen. See Article 7 in this issue by M. S. Schwartz and M. Scott.

6. Teaching File Case 26 Diagnosis moyamoya disease. moyamoya disease, taken from the Japanese meaning "a puff of smoke", is an idiopathic, http://www.uab.edu/pedradpath/case26.html

Radiology-Pathology Teaching File Case26 Recurrent Focal Neurologic Deficits Clinical Information: This 15-year-old white female who was otherwise normal presents with right-sided hemiparesis. Symptoms began approximately 10 days prior to evaluation and involved the right foot, arm and right side of the face. After a throrough history was obtained, it was discovered that the patient has had headaches associated with bilateral hand numbness in the past. T1/Gd MRA MRA/Gd MRI Findings: Displayed are MRI and MRA images from this patient. The axial and sagittal T1 images show an abnormal flow voids within the region of the basal ganglia, and probably some subacute hemorrhage, secondary to ischemia/infarction on the left. The T2 weighted axial image shows a focal area of ischemic infarction, corresponding to the small area of subacute hemorrhage in the left basal ganglia near the internal capsule (accounting for the patient's most recent stroke). The post Gd T1W coronal image shows some enhancement in this most recent area of ischemic infarction. Lastly, two MRA studies, 3D TOF with MT, pre and post Gd, demonstrate complete occlusion of both internal carotid arteries at their supraclinoid location. (Note the anatomic definition of the venous structures shining through on post Gd sagittal image). The posterior circulation is relatively intact. The patient underwent cerebral angiography for therapeutic planning and has received a pial synangiosis procedure discussed below. She is doing well.

7. Moyamoya Disease : A Review - J. Gosalakkal, - Neurology India Volume 50 Issue 1 Background moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular http://www.neurologyindia.com/vol50-1/1540ab.php

OFFICIAL JOURNAL OF NEUROLOGICAL SOCIETY OF INDIA Neurology India Volume 50 Issue 1 - March 2002 REVIEW ARTICLE VOL50-1 Moyamoya Disease : A Review J. A. Gosalakkal Department of Pediatric Neurology, NYU School of Medicine, New York - 10016, USA. Keywords : Vaso-occlusive disease, Moyamoya, Revascularization. Summary : Full Article Full Articles are available in .pdf format. To view these, you require Acrobat Reader Instructions to Authors Editor : S.Prabhakar neuroind@neurologyindia.com Neurology India Editorial Office for Volume 50 Issue 1 - March 2002 This is a Dynamic Site - PAGES GENERATED ON DEMAND - Developed by CPINDIA

8. NINDS - News And Events Health. Sorry! There are no press releases for moyamoya disease. Useyour browser's Back button to return to your previous activity. http://www.ninds.nih.gov/health_and_medical/news.htm?url=/health_and_medical/dis

9. NORD - Moyamoya Disease A general discussion about this disorder followed by resources. http://www.stepstn.com/cgi-win/nord.exe?proc=Redirect&type=rdb_sum&id=61

10. ** MOYAMOYA DISEASE ** Medical information of this disease is provided by a Japanese neurosurgeon. Also available in the Japanese language. http://www003.upp.so-net.ne.jp/moya-moya/

This home page is dedicated to the patients of moyamoya disease and their family Osaka City General Hospital, Department of Neurosurgery MASAKI KOMIYAMA, M.D. About Moyamoya Disease Diagnosis Treatment Prognosis ... To Japanese language page Open June 7, 2001 Last Update May 26, 2002 This home page has been visited times since June 28, 2001.

11. UCLA NEUROSURGERY | Cerebrovascular & Stroke Diseases & Disorder What is moyamoya disease? moyamoya disease is characterized by the spontaneous occlusion of one or usually both http://neurosun.medsch.ucla.edu/Diagnoses/Cerebrovascular/CerebroDis_7.html

Moyamoya Disease CEREBROVASCULAR PROGRAM CEREBROVASCULAR DIAGNOSES INDEX What is moyamoya disease? Moyamoya disease is characterized by the spontaneous occlusion of one or usually both internal carotid arteries in the intracranial region resulting in the formation of a fine network of neovascularity (blood vessel growth) at the base of the brain. The term "moyamoya" was introduced to describe the hazy, puff-of-smoke appearance of the neovascularization that develops. This condition has a higher incidence in Japan, but may be found in all nationalities. This disease has two age peaks: juvenile ( <10 years) and adult (3rd decade). What conditions is moyamoya disease associated with? Neurofibromatosis Tuberous sclerosis Sickle cell anemia Meningitis Retinitis pigmentosa Fibromuscular dysplasia Atherosclerosis Down's syndrome Fanconi's anemia Following radiation therapy to the skull base in children What are the symptoms or moyamoya disease? In children: Ischemic symptoms, consisting of recurrent, sometimes alternating episodes of focal cerebral deficit, especially weakness but also speech and sensory symptoms. These are the most typical symptoms.

12. HONselect - Moyamoya Disease Translate this page English moyamoya disease, - Cerebrovascular moyamoya disease - Progressive IntracranialOcclusive Arteropathy (Moyamoya) - Moya-Moya Disease - moyamoya disease http://www.hon.ch/HONselect/RareDiseases/C10.228.140.300.200.600.html

List of rare diseases: English Deutsch Language: MeSH term: Accepted terms: English: Moyamoya Disease - Cerebrovascular Moyamoya Disease - Progressive Intracranial Occlusive Arteropathy (Moyamoya) - Moya-Moya Disease - Moyamoya Disease, Classic - Moyamoya Disease, Primary - Moyamoya Disease, Secondary Français: MOYAMOYA, MALADIE Deutsch: Moyamoya-Krankheit - Progressive intrakranielle okklusive Arteriopathie (Moyamoya) - Zerebrovaskuläre Moyamoya-Krankheit Español: ENFERMEDAD DE MOYAMOYA - ENFERMEDAD DE MOYAMOYA CEREBROVASCULAR - ARTEROPATIA PROGRESIVA OCLUSIVA INTRACRANEAL (MOYAMOYA) Português: DOENCA DE MOYAMOYA - DOENCA CEREBROVASCULAR DE MOYAMOYA - ARTEROPATIA OCLUSIVA INTRACRANIANA PROGRESSIVA (MOYAMOYA) HONselect ressources Definition: Yes Articles: Yes Images: No News: No Conferences: No Clinical trials: No Web sites: English Yes Français No Deutsch No Español No Português No Home About us Site map Feedback ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C10.228.140.300.200.600.html Last modified: Thu Jul 25 2002

13. Moyamoya Disease moyamoya disease. need it. This procedure is made difficult by thesmall, delicate vessels found in moyamoya disease. In addition http://www.bcm.tmc.edu/pednsurg/disorder/moyamoya.htm

Moyamoya Disease (See also Vascular Malformations MOYAMOYA SYNDROME/ STROKE IN CHILDREN A stroke occurs when an area of brain tissue becomes permanently damaged by not receiving enough blood and oxygen. This can result in various degrees of paralysis, visual loss, speech deficits, seizures, and even headaches. Multiple small strokes can erode intellectual functions as well. Once these symptoms manifest, improvement may or may not occur, depending upon many individual factors. One cause of stroke in children is inflammation of the blood vessels supplying the brain, which can occur with infections from certain viruses or other rare organisms. Treatment of this problem is directed toward the causative organism. Children with sickle cell anemia have a high risk of stroke. This occurs in about 20% of patients with this disease, and these children often have multiple strokes. Treatment of this condition involves fluid administration, blood transfusions, and oxygen. Strokes also occur in children with birth defects of the heart, as small clots that originate in the heart can escape and block blood vessels that supply the brain. This can also happen with infections of the heart valves. Treatment of these conditions includes blood thinners, repair of the heart defect itself, or antibiotics against the heart valve infections. Rarely, migraine headache can cause stroke. Surgery plays little role in the treatment of these forms of stroke. There is however, a cause of stroke in children that can be treated with surgery. This is moyamoya syndrome, which is a progressive disease of the large arteries that supply the brain. These vessels slowly occlude over time, and little, if any, blood flows though them. The body tries to compensate by drawing blood from the vessels that supply the brain's surrounding structures instead. Although this is helpful, it is frequently not enough to adequately supply the brain. The brain will either suffer many small strokes, or will be forced to function with inadequate blood supply. In addition, some children will have temporary symptoms of stroke, which clear completely later. These are called transient ischemic attacks (TIA).

14. Canadian Journal Of Neurological Sciences - Article Matsushima Y. moyamoya disease. In Youmans JR. http://cjns.metapress.com/openurl.asp?genre=article&issn=0317-1671&volume=27&iss

15. Faculty: Chiu, D. stroke treatment, thrombolytic and neuroprotective therapies for stroke; intracerebralhemorrhage; cryptogenic stroke; patent foramen ovale; moyamoya disease. http://www.bcm.tmc.edu/neurol/faculty/Chiu.html

Assistant Professor M.D.(1991) Yale University School of Medicine Fax: 1-713-798-5030 email: dchiu@bcm.tmc.edu David Chiu, M.D. Clinical Interests: Cerebrovascular disease; acute stroke treatment, thrombolytic and neuroprotective therapies for stroke; intracerebral hemorrhage; cryptogenic stroke; patent foramen ovale; moyamoya disease. Research Interests: Thrombolysis and neuroprotection in stroke; clinical trials in stroke treatment and prevention; outcomes and epidemiology of stroke; cardiovascular and ECG sequelae of stroke; radiographic findings in stroke; risk factor treatment and stroke prevention. Selected Publications: Chiu, D. and Grotta, J.C. (1997) Clinical status of cytoprotection. In: Primer on Cerebrovascular Diseases, K.M.A. Welch, L.R. Caplan, D. Reis, B. Siesjo and B. Weir, Eds., Academic Press, San Diego, pp. 731-737. Grotta, J.C. and Chiu, D. (1997) Pharmacologic modification of acute cerebral ischemia. In: Stroke Pathophysiology, Diagnosis and Management, H.J.M. Barnett, J.P. Mohr, B.M. Stein, and F.M. Yatsu, Eds., Churchill Livingstone, New York. Chiu, D., Grotta, J.C., Krieger, D., Kasner, S., Villar, C., Bratina, P., and Vital, D. (1998) What role do neurologists play in determining the costs and outcome of stroke patients? J. Stroke Cerebrovasc. Dis. 7:267-271.

16. Moyamoya Disease Library M N. moyamoya disease. Web Sites. We are unaware of anyWeb Sites for moyamoya disease. If you know of any Web Sites for http://www.familyvillage.wisc.edu/lib_moya.htm

Moyamoya Disease Who to Contact Where to Go to Chat with Others Learn More About It Web Sites ... Search AltaVista for "Moyamoya Disease" Who to Contact Families with Moyamoya Support Network (MSN) 4900 McGowan Street SE Cedar Rapids, IA 52403 Where to Go to Chat with Others We are unaware of any discussion forums specifically for this diagnosis. However, there are several forums that may be of interest to parents of children with disabilities or special health care needs regardless of diagnosis. See General Disability On-Line Discussion Groups Learn More About It Moyamoya Disease information from Columbia-Presbyterian Medical Center Moyamoya Syndrome Moyamoya Disease information from OMIM Moyamoya Disease Web Sites We are unaware of any Web Sites for Moyamoya Disease. If you know of any Web Sites for Moyamoya Disease please let us know Back to [ M - N Family Village Home Library Coffee Shop ... Information Last Updated April 28, 1999 by familyvillage@waisman.wisc.edu Document Source: http://www.familyvillage.wisc.edu/lib_moya.htm

17. Children's Hemiplegia And Stroke Association clotting disorders are abnormalities that make a child more likely to have a bloodclot,. moyamoya disease (rare). infection such as meningitis. prematurity. http://www.chasa.org/etiology.htm

Home About CHASA Support Newly Diagnosed ... Contact CHASA etiology of pediatric stroke (what causes it?) Twenty-five per cent of all childhood strokes happen in infancy, often during childbirth and nobody knows why. Suspected causes of pediatric stroke are: congenital heart disease , probably the most common cause of stroke in children sickle cell disease probably a common cause of stroke in children, clotting disorders are abnormalities that make a child more likely to have a blood clot, moyamoya disease (rare) infection such as meningitis or chickenpox (varicella- roster virus) prematurity head trauma no cause can be detected in about a fifth of the children with ischemic infarction, yet many of these children seem to do well. Risk factors for pediatric cerebrovascular disease This article by Dr. E. S. Roach, lists different types of congenital heart disease, acquired heart disease, hematologic disorders and coagulopathies, systemic vascular disease, vasculitis, structural anomalies of the cerebrovascular system, vasculopathies, vasospastic disorders, and trauma as possible risk factors for pediatric stroke. Symptomatic Ischemic Stroke in Full-Term Neonates Why do newborn infants already suffer from "stroke". Studies of focal, arterial, ischemic infarct

18. UCLA NEUROSURGERY | Cerebrovascular & Stroke Diseases & Disorder CEREBROVASCULAR STROKE DISEASES DISORDERS moyamoya disease. What is moyamoyadisease? What conditions is moyamoya disease associated with? http://www.neurosurgery.medsch.ucla.edu/Diagnoses/Cerebrovascular/CerebroDis_7.h

Moyamoya Disease CEREBROVASCULAR PROGRAM CEREBROVASCULAR DIAGNOSES INDEX What is moyamoya disease? Moyamoya disease is characterized by the spontaneous occlusion of one or usually both internal carotid arteries in the intracranial region resulting in the formation of a fine network of neovascularity (blood vessel growth) at the base of the brain. The term "moyamoya" was introduced to describe the hazy, puff-of-smoke appearance of the neovascularization that develops. This condition has a higher incidence in Japan, but may be found in all nationalities. This disease has two age peaks: juvenile ( <10 years) and adult (3rd decade). What conditions is moyamoya disease associated with? Neurofibromatosis Tuberous sclerosis Sickle cell anemia Meningitis Retinitis pigmentosa Fibromuscular dysplasia Atherosclerosis Down's syndrome Fanconi's anemia Following radiation therapy to the skull base in children What are the symptoms or moyamoya disease? In children: Ischemic symptoms, consisting of recurrent, sometimes alternating episodes of focal cerebral deficit, especially weakness but also speech and sensory symptoms. These are the most typical symptoms.

19. AANS Online Marketplace moyamoya disease Edited by Kiyonobu Ikezaki MD, Christopher M. LoftusMD, FACS. Pricing Please select the appropriate price to purchase. http://www.neurosurgery.org/marketpl/product/default.asp?sku=804

20. Moyamoya Disease - Page 17 moyamoya disease. E. Kirsch1. Based on the clinical and radiological features,the diagnosis of moyamoya disease (MD) was made. Figure 1, Figure 2. http://www.rbrs.org/database/83-1/page17.html

MOYAMOYA DISEASE E. Kirsch Key-word: Cerebral blood vessels, stenosis or obstruction. Clinical history A 55-year-old man presented with sudden onset right-sided sensorimotoric hemiparesis. Ultrasonography showed a total occlusion of the right internal carotid artery (ICA) at the level of the carotid bifurcation. Laboratory findings were normal. Radiological diagnosis Axial SE T1-weighted MR image of the brain (fig. 1) shows acute bleeding in the centrum semiovale of the left hemisphere. Axial MIP-MR angiography of the brain (fig. 2) demonstrates an occluded ICA, anterior (ACA) and middle cerebral artery (MCA) on the right side. Occlusion of the left ACA and MCA, and intense signal of both posterior cerebral arteries (PCA) are seen. Some additional vessels at the level of the proximal PCA-portions are also visible. Intra-arterial DSA of the brain (fig. 3) includes left common carotid artery (CCA) arteriography, oblique projection (fig. 3 A) which showed occlusion of the proximal ACA and MCA. Additional abnormal vascular network in the region of the basal ganglia is seen. Right CCA-arteriography, AP projection

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