1. NINDS Moyamoya Disease Information Page moyamoya disease information page compiled by the National Institute of Neurological Disorders 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.

2. EMedicine - Moyamoya Disease : Article By Roy Sucholeiki, MD moyamoya disease moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis http://www.emedicine.com/neuro/topic616.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Neurology Pediatric Neurology Moyamoya Disease Last Updated: November 12, 2003 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 S Rust, Jr, MD , Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-director, FE Dreifuss Child Neurology and Epilepsy Clinic, University of Virginia; Clinical and Residency Training Director, Child Neurology, University of Virginia Hospital and Clinics; Francisco Talavera, PharmD, PhD

3. Moyamoya Disease Definition, clinical symptoms and radiographic finding. 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 26 May 2004 Medical College of Wisconsin

4. NORD - National Organization For Rare Disorders, Inc. Sample report, plus links to organisations. Fee required for full report http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Moyamoya Synd

5. Moyamoya Disease - Internet Support Group For MoyaMoya Families Internet support for those diagnosed with moyamoya disease. I spent the next two months searching the internet for everything I could find on moyamoya disease. http://www.moyamoya.com/

var keepstatic=1 //specify whether menu should stay static (works only in IE4+) var menucolor="#00026C" //specify menu color var submenuwidth=125 //specify sub menus' color Internet support for those diagnosed with MoyaMoya Disease "Maui Maui? What the hell is that!!" Those were my exact words when I first heard of MoyaMoya in June 2001. I spent the next two months searching the internet for everything I could find on MoyaMoya Disease. My name is DJ and I've been "blessed" with MoyaMoya. If you're here, it's probably because you're like me and came to the internet to find out more about this rare disorder. If you're like me, you also didn't have much success looking through the search engines. There's not a huge amount of information out there huh? Well, that's all about to change! Probably the worst part about being diagnosed with a rare disease (especially an estimated 1 in 2,000,000 disease in the USA) is thinking you are the only one in the world who is dealing with the problems associated with the disease. I hope to change all that! I would like to build the world's largest support group for family members and those that suffer from MoyaMoya.

6. ** MOYAMOYA DISEASE ** Diagnosis, treatment and prognosis, plus problems specific to women. 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.

7. UCLA NEUROSURGERY | Cerebrovascular & Stroke Diseases & Disorder CEREBROVASCULAR STROKE DISEASES DISORDERS moyamoya disease. What is moyamoya disease? What conditions is moyamoya disease associated with? 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.

8. Moyamoya Disease - Eurorad - Clinical Case 946 - Resident teaching files radiology moyamoya disease presented with sudden onset rightsided sensorimotoric hemiparesis. Ultrasonography showed a total occlusion of the right internal carotid artery (ICA) at http://www.eurorad.org/case.cfm?uid=946

9. Teaching File Case 26 Diagnosis moyamoya disease. moyamoya disease, taken from the Japanese meaning "a puff of Technique and Complications in Childhood moyamoya disease". Robertson RL, Chavali R, Robson 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.

10. 5-5-NSF-TOC 1. moyamoya disease in the midwestern United States fibroblast growth factor in the pathogenesis of moyamoya disease. Kiyohiro Houkin, Tetsuyuki Yoshimoto, Hiroshi Abe 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.

11. NINDS - News And Events Health. Sorry! There are no press releases for moyamoya disease. Use your 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

12. Moyamoya Disease - Internet Support Group For MoyaMoya Families Worldwide Support Group for sufferers of moyamoya disease and their famlies Internet support for those diagnosed with moyamoya disease " Maui Maui searching the internet for everything I could find on moyamoya disease. My name is DJ and I've been "blessed http://rdre1.inktomi.com/click?u=http://www.moyamoya.com/&y=0230590DC91CBC20

13. Moyamoya Disease - Story Of A MoyaMoya Sufferer Internet support for those diagnosed with moyamoya disease. What is moyamoya disease? The pathogenesis of moyamoya disease is unknown. http://www.moyamoya.com/whatis.html

var keepstatic=1 //specify whether menu should stay static (works only in IE4+) var menucolor="#00026C" //specify menu color var submenuwidth=125 //specify sub menus' color Internet support for those diagnosed with Moyamoya Disease What is Moyamoya Disease? Moyamoya is an extremely rare disorder in most parts of the world except in Japan. The pathogenesis of Moyamoya disease is unknown. Moyamoya disease is characterized by progressive intracranial vascular stenoses of the circle of Willis, resulting in successive ischemic events. The condition leads to irreversible blockage of the main blood vessels to the brain as they enter into the skull. It is a disease that tends to affect children and adults in the third to fourth decades of life. In children it tends to cause strokes or seizures. In adults it tends to cause bleeding or strokes. The clinical features are cerebral ischaemia (strokes), recurrent transient ischaemic attacks (TIA's or "mini strokes"), sensorimotor paralysis (numbness in the extremeties), convulsions and/or migraine-like headaches. The process of blockage (vascular occlusion) once it begins tends to continue despite any known medical management unless treated with surgery. The repeated strokes can lead to severe functional impairment or even death so that it is important to recognize these lesions and treat them early on. Without treatment, there is progressive deterioration of neurologic function and re-hemorrhage.

14. Moyamoya Disease Information Diseases Database moyamoya disease MoyaMoya disease, Disease Database Information http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=8384

15. Introduction: Moyamoya Disease - WrongDiagnosis.com Introduction to moyamoya disease as a medical condition including symptoms, diagnosis, misdiagnosis, treatment, prevention, and prognosis. http://www.wrongdiagnosis.com/m/moyamoya_disease/intro.htm

Diseases IMPORTANT! Use of this site is subject to our and Home Symptoms Diseases Risks Search: We show you all these ads in order to provide this free site; give your feedback WrongDiagnosis TM Premium Report: Diabetes Diagnosis and Misdiagnosis Available Now! Condition Lists By Organ By Symptom By Class By Prevalence ... List A-Z Current chapter: Moyamoya Disease Next sections Basic Summary for Moyamoya Disease Prevalence and Incidence of Moyamoya Disease Prognosis of Moyamoya Disease Types of Moyamoya Disease ... Causes of Moyamoya Disease Next chapters: Mucopolysaccharidoses Mucopolysaccharidosis I Mucopolysaccharidosis II Mucopolysaccharidosis III ... Feedback Introduction: Moyamoya Disease Moyamoya Disease: Brain blood vessel disorder. 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 . First described in Japan in the 1960’s, the disease has since been found in the United States, Europe, Australia, and Africa. Contents for Moyamoya Disease: Basic Summary for Moyamoya Disease Prevalence and Incidence of Moyamoya Disease Prognosis of Moyamoya Disease Types of Moyamoya Disease ... Statistics about Moyamoya Disease Footnotes: 1. excerpt from

16. MOYAMOYA DISEASE About moyamoya disease In 1950 s, Japanese leading neurosurgeons at that time began to notice the new clinical entity of moyamoya disease. http://www003.upp.so-net.ne.jp/moya-moya/E-Dx1.htm

About Moyamoya Disease In 1950's, Japanese leading neurosurgeons at that time began to notice the new clinical entity of moyamoya disease. Since its etiology was unknown, it was named in various ways, such as moyamoya disease, chiri-chiri disease, etc. Moyamoya disease is now officially named in Japan as "the spontaneous occlusion of the circle of Willis." The terminal portions of the intracranial internal carotid arteries progressively become stenotic (narrowing) or occlusive. Due to reduced blood flow to the brain, tiny collateral vessels at the base of the brain enlarge to become as collateral pathways. These vessels are called "moyamoya vessels" because the angiographic appearance of these vessels resemble the "cloud" or "puff" of the cigarette smoke, which is described as "moya-moya" in Japanese language ( Suzuki J, Takaku A About the name of moyamoya disease Moyamoya Vessels (Abnormally proliferating arteries?) In 1960's, moyamoya disease became the main interest for the neurosurgeons and neurologists because this disease was prevalent solely in Japan and/or Japanese ethnicity. In 1994, the number of the annual patients having moyamoya disease was estimated about 3,900. Normal cerebral angiogram and that of moyamoya patient Symptoms of moyamoya disease include brain ischaemia and hemorrhage. There are two peaks in age distribution: below 10 and at 30-40 years old, with the highest peak in the first decade and lower peak in the adults. Under the age of 10, brain ischemia (transient ischemic attack and cerebral infarction) is common whilst brain hemorrhage is more common at the age of 30-40 years old.

17. Moyamoya Disease / Family Village Library M N. moyamoya disease. Web Sites. Moya Moya.Com Internet support for those diagnosed with moyamoya disease. Children s Hemiplegia http://www.familyvillage.wisc.edu/lib_moya.htm

Moyamoya Disease Where to Go to Chat with Others Learn More About It Web Sites Search Google for "Moyamoya Disease" Where to Go to Chat with Others Parenting Children with Moyamoya This group is for parents of children with this rare disease to come together and share their experiences and any information that they may have. This is also a place to find support and compassion from others who know exactly what you are going through. Moyamoya Message Board Learn More About It Moyamoya Disease information from Columbia-Presbyterian Medical Center FAQs About Moyamoya Syndrome Moyamoya Disease information from OMIM Moyamoya Disease ... Moyamoya Disease / Stroke in Children Web Sites Moya Moya.Com Internet support for those diagnosed with Moyamoya Disease Children's Hemiplegia and Stroke Association A non-profit organization, offering support and information to families of infants, children, and young adults who have hemiplegia, hemiparesis, hemiplegic cerebral palsy, childhood stroke, infant stroke, or in utero stroke. The Brain Attack Coalition Recognition and Treatment of Stroke in Children Back to [ M - N Family Village Home Library Coffee Shop ... Information Last Updated 4/1/2004 by familyvillage@waisman.wisc.edu

18. Children's Hemiplegia And Stroke Association moyamoya disease. What is moyamoya disease? Is there any treatment? There is no cure for moyamoya disease. Treatment is symptomatic and supportive. http://www.hemikids.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.

19. Moyamoya Disease moyamoya disease. need it. This procedure is made difficult by the small, 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).

20. Radiology In Ped Emerg Med, Vol 3, Case 9 moyamoya disease Radiology Cases in Pediatric Emergency Medicine Volume 3, Case 9 Karen R. Sevigny Brown, MD Loren G. Yamamoto, MD, MPH Kapiolani Medical http://www.hawaii.edu/medicine/pediatrics/pemxray/v3c09.html

Moyamoya Disease Radiology Cases in Pediatric Emergency Medicine Volume 3, Case 9 Karen R. Sevigny Brown, MD Loren G. Yamamoto, MD, MPH Kapiolani Medical Center For Women And Children University of Hawaii John A. Burns School of Medicine L = Lateral ventricles. The anterior horns and the posterior horns are shown in this diagram. 3 = Third ventricle. CC = Corpus callosum. C = Caudate nucleus. P = Putamen G = Globus Pallidus. The putamen and globus pallidus together form the lenticular (lentiform) nucleus. T = Thalamus Arrows = Internal Capsule [anterior limb, posterior limb, genu (bend)]. O = Optic radiations. A = Auditory radiations. The corticospinal tract originates from the motor strip of the cerebral cortex. The fibers collect as they traverse through the posterior limb of internal capsule. The tract largely crosses the midline in the decussation of the pyramids. Fibers exit the spinal cord at their respective levels. View our patient's CT scan. T1 (left image) and T2 (right image) weighted axial images are shown (different levels). On the T1 image, the ventricles appear to be dark and the infarct seen in the left lenticular nucleus is dark as well. The T2 image is a lower cut through the center of the infarct. The T2 image shows the CSF within the ventricles to be white. The infarct appears as a white lesion in the caudate nucleus and the left putamen. In the T2 image, internal capsule is dark. Note the obvious distortion of the anterior limb of the left internal capsule, compared to the right. The posterior limb of the left internal capsule is also slightly distorted (compared to the right) adjacent to the infarct in the putamen. This study is read as an infarct in the left basal ganglia, the posterior limb of internal capsule, and the head of the caudate. The structures of this T2 image are labeled if you have difficulty identifying the structures.

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