Moyamoya Disease (Moyamoya Syndrome) and Coarctation of the Aorta

Objective: To determine if elevated rates of autoimmune disease are present in children with both Down syndrome and moyamoya disease given the high rates of autoimmune disease reported in both conditions and unknown etiology of angiopathy in this population.Methods: A multi-center retrospective case-control study of children with Down syndrome and moyamoya syndrome, idiopathic moyamoya disease, and Down syndrome without cerebrovascular disease was performed. Outcome measures included presence of autoimmune disease, presence of autoantibodies and angiopathy severity data. Comparisons across groups was performed using the Kruskal-Wallis, χ2 and multivariate Poisson regression.Results: The prevalence of autoimmune disease were 57.7, 20.3, and 35.3% in persons with Down syndrome and moyamoya syndrome, idiopathic moyamoya disease, and Down syndrome only groups, respectively (p < 0.001). The prevalence of autoimmune disease among children with Down syndrome and moyamoya syndrome is 3.2 times (p < 0.001, 95% CI: 1.82–5.58) higher than the idiopathic moyamoya group and 1.5 times (p = 0.002, 95% CI: 1.17–1.99) higher than the Down syndrome only group when adjusting for age and sex. The most common autoimmune diseases were thyroid disorders, type I diabetes and Celiac disease. No individuals with idiopathic moyamoya disease had more than one type of autoimmune disorder while 15.4% of individuals with Down syndrome and moyamoya syndrome and 4.8% of individuals with Down syndrome only had >1 disorder (p = 0.05, 95%CI: 1.08–6.08).Interpretation: This study reports elevated rates of autoimmune disease in persons with Down syndrome and moyamoya syndrome providing a nidus for study of the role of autoimmunity in angiopathy in this population.

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Benefits of outpatient cardiac rehabilitation in an adult patient with coarctation of the aorta and moyamoya disease

Journal of Rehabilitation Medicine – Clinical Communications ◽

10.2340/20030711-1000069 ◽

2021 ◽

Vol 4 (1) ◽

pp. jrmcc00070

Author(s):

R Zhang ◽

C Chen ◽

E Yeung ◽

K Yiu

Keyword(s):

Cardiac Rehabilitation ◽

Adult Patient ◽

Moyamoya Disease ◽

Coarctation Of The Aorta

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Case Report: Acute Stroke in Young Adult Patient with Moyamoya Syndrome Secondary to Sickle Cell Disease

Blood ◽

10.1182/blood-2020-134202 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 13-13

Author(s):

Oladipo Cole ◽

Asia Filatov ◽

Javed Khanni ◽

Patricio Espinosa

Keyword(s):

Case Report ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Moyamoya Disease ◽

Conflicts Of Interest ◽

Acute Chest Syndrome ◽

African American Female ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Radiographic Findings

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

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Moyamoya in Hispanics: not only in Japanese

Neurology International ◽

10.4081/ni.2014.5369 ◽

2014 ◽

Vol 6 (2) ◽

Cited By ~ 2

Author(s):

Sarmad Said ◽

Chad J. Cooper ◽

Haider Alkhateeb ◽

Juan M. Galvis ◽

German T. Hernandez ◽

...

Keyword(s):

Cigarette Smoke ◽

Moyamoya Disease ◽

Carotid Arteries ◽

Internal Carotid ◽

Prolonged Survival ◽

Moyamoya Syndrome ◽

Characteristic Appearance ◽

Hispanic Patients ◽

Collateral Vessels ◽

Internal Carotid Arteries

Moyamoya disease was first described in 1957 as <em>hypoplasia of the bilateral internal carotid arteries</em>, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is <em>moyamoya</em>. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.

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Moyamoya Syndrome: A Window of Moyamoya Disease

Journal of Korean Neurosurgical Society ◽

10.3340/jkns.2015.57.6.408 ◽

2015 ◽

Vol 57 (6) ◽

pp. 408 ◽

Cited By ~ 37

Author(s):

Ji Hoon Phi ◽

Kyu-Chang Wang ◽

Ji Yeoun Lee ◽

Seung-Ki Kim

Keyword(s):

Moyamoya Disease ◽

Moyamoya Syndrome

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Abstract TP397: Clinical And Functional Outcome Of Adult Patients Diagnosed With Moyamoya Disease In Childhood

Stroke ◽

10.1161/str.48.suppl_1.tp397 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

David Fam ◽

Nomazulu Dlamini ◽

Cheryl Jaigobin

Keyword(s):

Ischemic Stroke ◽

North America ◽

Natural History ◽

Functional Outcome ◽

Moyamoya Disease ◽

Vascular Event ◽

Moyamoya Syndrome ◽

The Mean

Background: Moyamoya disease is a progressive occlusive arteriopathy of the terminal ICA and its branches, leading to the formation of unstable collateral vessels. The disease is found worldwide, and is associated with a number of predisposing conditions, termed moyamoya syndrome. Currently there is a paucity of data on the natural history of moyamoya disease in North America, especially the long-term outcome of pediatric moyamoya. Objective: Our objectives were to determine the rate of recurrent TIA, ischemic stroke and intracerebral hemorrhage in patients presenting with pediatric Moyamoya disease and to assess long-term functional outcome. Methods: This study was a retrospective chart review of 52 patients presenting with pediatric moyamoya disease. We included patients aged 18 or older with a diagnosis of moyamoya disease or syndrome. All patients had confirmatory baseline vascular imaging (MRA or CTA +/- cerebral angiography). Baseline demographic variables and annual vascular event recurrence risk were obtained from the records. Modified Rankin Score (MRS) at presentation and last follow-up were determined from clinical records. Results: Of the original cohort, 34 patients were included for analysis. The mean age of the patients was 23.9 years. The mean age at presentation was 9 years (SD=4) with an average follow-up of 11.3 years (SD=5.1) for a total of 383 patient years. There was slight female predominance (1.4:1). Fifty percent of patients (17) presented with ischemic stroke. A total of 26 patients (76%) underwent surgery, with 7 (21%) requiring repeat surgery. The annual vascular recurrent event rate was 5.0% (19/383), which was not significantly different between surgical and non-surgical groups or between different moyamoya syndrome subtypes. Most of the recurrent events were TIA (annual recurrence 3.7%). There was no significant difference between initial MRS, MRS at last follow-up and mean change in MRS between surgical and non-surgical groups. Conclusion: Our study is the largest North American natural history study of pediatric moyamoya. Our observations indicate that pediatric moyamoya in North America have low recurrent vascular event rates and long-term functional outcomes are good, even in conservatively managed patients.

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Response to letter to the editor: High prevalence of pro-thrombotic conditions in adult patients with moyamoya disease and moyamoya syndrome: a single center study

Acta Neurochirurgica ◽

10.1007/s00701-020-04564-7 ◽

2020 ◽

Vol 162 (12) ◽

pp. 3137-3138

Author(s):

Anthony S. Larson ◽

James P. Klaas

Keyword(s):

Moyamoya Disease ◽

Adult Patients ◽

Moyamoya Syndrome ◽

Single Center ◽

Letter To The Editor ◽

Single Center Study ◽

High Prevalence ◽

Center Study

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Moyamoya syndrome associated with neurofibromatosis type I in a pediatric patient

Sao Paulo Medical Journal ◽

10.1590/s1516-31802011000200010 ◽

2011 ◽

Vol 129 (2) ◽

pp. 110-112 ◽

Cited By ~ 7

Author(s):

Luiz Guilherme Darrigo Júnior ◽

Elvis Terci Valera ◽

André de Aboim Machado ◽

Antonio Carlos dos Santos ◽

Carlos Alberto Scrideli ◽

...

Keyword(s):

Moyamoya Disease ◽

Autosomal Dominant ◽

Genetic Disorder ◽

Neurofibromatosis Type ◽

Type I ◽

Moyamoya Syndrome ◽

Radiological Findings ◽

Convulsive Seizures ◽

Age Range

CONTEXT: Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT: An eight-month-old female presented convulsive seizures with clonic movements. The patient suffered an ischemic stroke with hemiparesis. Magnetic resonance imaging revealed radiological findings compatible with moyamoya disease. The diagnosis of NF-1 was made at the age of 20 months. CONCLUSION: Despite the rarity of this association in childhood, children with focal neurological symptoms and a diagnosis of NF-1 deserve to be investigated for moyamoya syndrome.

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Advances and Surgical Considerations in the Treatment of Moyamoya Disease

Neurosurgery ◽

10.1227/neu.0000000000000229 ◽

2014 ◽

Vol 74 (suppl_1) ◽

pp. S116-S125 ◽

Cited By ~ 30

Author(s):

Eric J. Arias ◽

Colin P. Derdeyn ◽

Ralph G. Dacey ◽

Gregory J. Zipfel

Keyword(s):

Moyamoya Disease ◽

Clinical Characteristics ◽

Surgical Techniques ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Anterior Circulation ◽

Underlying Condition ◽

Underlying Pathology ◽

Shed Light

Abstract Moyamoya is a rare disorder that involves steno-occlusive arterial changes of the anterior circulation, along with proliferative development of basal arterial collaterals. It is either idiopathic (called moyamoya disease) or the result of a specific underlying condition such as atherosclerosis, radiation therapy, or sickle cell disease (called moyamoya syndrome or phenomenon). In recent years, numerous insights into and advances in the understanding, evaluation, and management of moyamoya patients have occurred. This article briefly reviews the spectrum of moyamoya conditions and then provides a synopsis of numerous recent investigations that shed light on various aspects of the disease, including its clinical characteristics, natural history, underlying pathology, imaging, surgical techniques, and long-term patient outcome.

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