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Related Concept Videos

Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
Cushing Syndrome I: Introduction01:26

Cushing Syndrome I: Introduction

Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
Acute Coronary Syndrome I: Introduction01:30

Acute Coronary Syndrome I: Introduction

Acute Coronary Syndrome (ACS) encompasses a spectrum of heart conditions caused by sudden obstruction of coronary arteries, typically resulting from the rupture of an atherosclerotic plaque and subsequent thrombus (blood clot) formation. This obstruction can lead to partial or complete blockage of blood flow, causing varying degrees of myocardial ischemia or infarction.ACS includes the following clinical entities:Unstable Angina (UA)Non-ST-Elevation Myocardial Infarction (NSTEMI)ST-Elevation...
Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations01:19

Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations

The pathophysiology of Acute Coronary Syndrome [ACD] involves several key processes:The main underlying cause of ACD is atherosclerosis, a chronic inflammatory disease characterized by the buildup of lipid-laden plaques within the coronary arteries.As the atherosclerotic plaque grows in the coronary artery, it may become unstable due to the formation of a lipid-rich core and a thin fibrous cap. Inflammatory cells within the plaque, such as macrophages, secrete enzymes that degrade the...

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Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights
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Published on: October 13, 2023

Susac's Syndrome--update.

Robert Rennebohm1, John O Susac, Robert A Egan

  • 1Department of Pediatrics, Division of Pediatric Rheumatology Alberta Children's Hospital University of Calgary, Faculty of Medicine Calgary, Alberta, Canada. robert.rennebohm@albertahealthservices.ca

Journal of the Neurological Sciences
|September 22, 2010
PubMed
Summary
This summary is machine-generated.

Susac's Syndrome (SS) is an autoimmune condition affecting small brain, retina, and inner ear vessels. Diagnosis is possible with encephalopathy and characteristic MRI findings, even without branch retinal artery occlusion or hearing loss.

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Published on: January 22, 2017

Area of Science:

  • Neurology
  • Immunology
  • Vascular Medicine

Background:

  • Susac's Syndrome (SS) is a rare autoimmune endotheliopathy.
  • It primarily affects precapillary arterioles in the brain, retina, and inner ear.
  • SS typically presents with a triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss (HL).

Purpose of the Study:

  • To define diagnostic criteria for Susac's Syndrome.
  • To highlight key MRI findings, including callosal lesions and "string of pearls" sign.
  • To discuss a BRAO subset and preliminary treatment strategies.

Main Methods:

  • Clinical case review and analysis.
  • Magnetic Resonance Imaging (MRI) interpretation, including diffusion-weighted imaging.
  • Review of diagnostic criteria and proposed treatment approaches.

Main Results:

  • Encephalopathy with pathognomonic corpus callosum lesions can be sufficient for SS diagnosis.
  • The "string of pearls" sign in internal capsules is a significant diagnostic indicator.
  • Branch retinal artery occlusion (BRAO) represents a distinct subset of SS.

Conclusions:

  • Susac's Syndrome diagnosis can be established with encephalopathy and characteristic MRI findings alone.
  • Early recognition and diagnosis are crucial for managing SS.
  • Further research and international collaboration are vital for understanding and treating SS.