Moyamoya disease in a primarily white, midwestern US population: increased prevalence of autoimmune disease

Regina S Bower1, Grant W Mallory, Macaulay Nwojo

  • 1Department of Neurosurgery, Mayo Clinic College of Medicine, Rochester, MN, USA.

Stroke
|May 9, 2013
PubMed
Abstract

Insights

This study found a higher prevalence of autoimmune diseases, including type 1 diabetes and thyroid disease, in a Midwestern moyamoya disease patient cohort. These findings suggest a potential autoimmune link in moyamoya pathogenesis.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Autoimmune Diseases

Background:

  • Moyamoya disease is a rare cerebrovascular occlusive condition with unknown pathogenesis.
  • Existing research on moyamoya disease cohorts is predominantly from Asian or ethnically diverse North American populations.

Purpose of the Study:

  • To investigate the clinical characteristics of moyamoya disease in a primarily white, Midwestern US population.
  • To identify potential insights into the pathogenesis of moyamoya disease.

Main Methods:

  • Retrospective analysis of patients with angiographically confirmed moyamoya disease.
  • Comparison of comorbidity, cerebrovascular risk factor, and autoimmune disease prevalence with the general population.

Main Results:

  • The study evaluated 94 moyamoya patients, predominantly female (72.3%) and white (85%).
  • A significantly higher prevalence of autoimmune diseases, including type 1 diabetes mellitus (8.5% vs. 0.4%) and thyroid disease (17.0% vs. 8.0%), was observed compared to the general population.
  • Increased prevalence of hyperlipidemia (27.7% vs. 16.3%) was also noted.

Conclusions:

  • This study highlights a significantly higher prevalence of autoimmune disease in a unique, primarily white, Midwestern moyamoya patient population.
  • The findings support a potential autoimmune component in the pathogenesis of moyamoya disease.

Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Atherosclerosis II: Clinical Manifestations and Diagnostic Tests01:27

Atherosclerosis II: Clinical Manifestations and Diagnostic Tests

Atherosclerosis is a progressive disorder that leads to the thickening and narrowing of arterial walls due to plaque buildup. This condition can cause various symptoms depending on the arteries affected:Coronary Artery Disease (CAD): This condition affects the coronary arteries and may lead to chest pain (angina), shortness of breath (dyspnea), heart attacks, and other heart disease symptoms.Cerebrovascular Disease: This affects blood flow to the brain, causing transient ischemic attacks (TIAs)...
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...