Sydenham Chorea

Sydenham Chorea, also known as St. Vitus Dance or Rheumatic Chorea, is a rare movement disorder. It mainly affects children. The condition causes quick, unwanted movements of the face, arms, and legs. It also leads to emotional and behavioral changes.

Thomas Sydenham, a British physician, first described it in the 17th century. It is linked to streptococcal infections, like Group A Streptococcus. These infections can start an autoimmune response in some people. This reaction is thought to cause inflammation in the basal ganglia, a part of the brain that controls movement.

Sydenham Chorea is seen as a sign of acute rheumatic fever. This is a disorder that can happen after a streptococcal infection is not treated. The exact number of children affected by Sydenham Chorea is not known. But it’s thought to be about 1 in 5,000 worldwide, more in places where antibiotics are hard to get.

Research is also looking into a possible link with Pediatric Autoimmune Neuropsychiatric Disorders (PANDAS). PANDAS is a diagnosis that suggests streptococcal infections might cause sudden neuropsychiatric symptoms in kids.

What is Sydenham Chorea?

Sydenham Chorea, also known as St. Vitus Dance or Rheumatic Chorea, is a neurological disorder. It causes rapid, uncoordinated jerking movements in the face, hands, and feet. These movements are often accompanied by muscle weakness, slurred speech, and emotional instability.

The disorder mainly affects children and adolescents. It is more common in females.

Definition and Overview

Sydenham Chorea is an autoimmune disorder caused by streptococcal infections, like rheumatic fever. It happens when antibodies attack certain brain areas, causing inflammation and disrupting motor control. Symptoms start slowly and can get worse over time, lasting several months.

While most cases get better on their own, some people may have recurring symptoms or lasting effects.

Historical Background and Naming

The condition was first described by Thomas Sydenham, an English physician, in the 17th century. The name “chorea” comes from the Greek word for dance, because the movements look like dancing. It’s also known as St. Vitus Dance, named after a Christian saint who was believed to protect against nervous disorders.

For a long time, Sydenham Chorea was linked to rheumatic fever, a disease caused by streptococcal infections. This connection led to the name Rheumatic Chorea. Today, treating streptococcal infections early and better living conditions have lowered Sydenham Chorea cases in developed countries. But, it’s a big concern in places with less access to healthcare and untreated streptococcal infections.

Causes and Risk Factors

Sydenham Chorea is a neurological disorder linked to Group A Streptococcus bacteria. This bacteria causes strep throat and other infections. The body’s immune response to this infection may harm certain brain areas. This leads to the movement and behavioral symptoms seen in Sydenham Chorea.

Rheumatic fever is a big risk factor for Sydenham Chorea. It happens when streptococcal infections are not treated. Sydenham Chorea is a key sign of rheumatic fever. Here’s how they are connected:

Research shows genetics might also affect Sydenham Chorea. Some people might be more likely to get it because of their genes. These genes could affect how the immune system and brain work. But, we need more research to understand this fully.

PANDAS and Sydenham Chorea

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) is similar to Sydenham Chorea. Both are linked to an abnormal immune response to streptococcal infections. They both cause neurological and behavioral symptoms. But, the exact symptoms and how to diagnose PANDAS are topics of ongoing research.

Symptoms and Presentation

Sydenham Chorea shows a mix of neurological and psychiatric signs. These can really affect how a person lives their day-to-day life. The main sign is involuntary movements and tics, which can happen anywhere in the body.

Involuntary Movements and Tics

People with Sydenham Chorea have movements that seem jerky and uncontrolled. These can look like dancing and happen in the face, arms, legs, and trunk. How often and how bad these tics are can change for each person.

Behavioral and Emotional Changes

People with Sydenham Chorea also see changes in how they act and feel. They might get irritable, have mood swings, or feel emotional easily. Some might act impulsively or have obsessive thoughts. These changes can be hard for both the person and their family, and they might need help from mental health experts.

Severity and Duration of Symptoms

The symptoms of Sydenham Chorea can vary a lot. Some people might just have small movements, while others might have big problems with their tics. How long symptoms last can also vary, but most cases get better in a few months. But, sometimes symptoms can last longer or come back. It’s important to treat the infection and rheumatic fever quickly to help symptoms not last as long.

Diagnosis and Differential Diagnosis

To diagnose Sydenham Chorea, doctors use a detailed approach. They do a thorough clinical exam, neurological assessment, and run lab tests and imaging studies. It’s important to look at the patient’s symptoms, medical history, and physical signs. This helps doctors tell Sydenham Chorea apart from other movement disorders.

Clinical Examination and Neurological Assessment

The first step is a detailed clinical exam. Doctors focus on the patient’s involuntary movements and any emotional or behavioral changes. They check how severe and widespread the movements are. They also test the patient’s coordination, balance, and fine motor skills.

Laboratory Tests and Imaging Studies

Lab tests are key in confirming Sydenham Chorea and ruling out other causes. Important tests include:

• Streptococcal antibody tests (ASO, anti-DNase B) to detect recent streptococcal infection
• Inflammatory markers (ESR, CRP) to assess the presence of inflammation
• Antineuronal antibody tests to identify autoimmune processes targeting the nervous system

Imaging studies like MRI or CT scans of the brain might be done. They help rule out other conditions that could look like Sydenham Chorea.

Ruling Out Other Movement Disorders

It’s important to rule out other movement disorders that might look similar. Some conditions to consider include:

Doctors look at the patient’s age, symptoms, and lab results to make an accurate diagnosis. This helps them start the right treatment to manage symptoms and prevent problems.

Treatment Options for Sydenham Chorea

Effective treatment for Sydenham Chorea involves a few key steps. It targets the underlying streptococcal infection and manages symptoms. Early diagnosis and treatment are key to good outcomes and avoiding long-term problems.

Antibiotics and Infection Control

The first step is using antibiotics to fight the streptococcal infection. Penicillin or similar antibiotics are given for 10-14 days. This helps stop the infection and lowers the chance of more episodes.

Medications for Symptom Management

Along with antibiotics, medications help manage symptoms. Antiepileptic drugs like valproic acid control involuntary movements. Dopamine-depleting agents like tetrabenazine reduce chorea severity. Benzodiazepines help with anxiety and muscle relaxation.

Supportive Care and Rehabilitation

Supportive care and rehabilitation are essential. Occupational therapy helps with daily activities and fine motor skills. Physical therapy keeps muscles strong and balanced. Speech therapy is needed for speech issues. Psychological support helps with emotional and behavioral changes.

Prognosis and Long-term Outlook

People with Sydenham Chorea usually get better within months to a year. Their symptoms can be tough to deal with, but they often go away without lasting harm. This happens when they get the right treatment quickly.

The outlook can change based on how bad the symptoms were at first and how well treatment works. Some might have Sydenham Chorea again if they get strep infections. To avoid this, it’s key to treat strep throat right away.

Even though most people get better, some might face lasting effects. These can include problems with movement, feelings, and school work. Getting help from physical, occupational therapy, and counseling can make a big difference.

Regular check-ups with a neurologist or pediatric specialist are very important. They help keep an eye on how you’re doing and make any needed changes. By staying close to your healthcare team, you can have a better future and a good quality of life.

Complications and Associated Conditions

Sydenham Chorea can cause many complications and is linked to other health issues. It’s key for patients and their families to know about these problems. This helps manage the disorder and keep overall health good.

Neuropsychiatric Comorbidities

People with Sydenham Chorea might also face neuropsychiatric issues. These can include:

• Attention deficit hyperactivity disorder (ADHD)
• Obsessive-compulsive disorder (OCD)
• Anxiety disorders
• Depression
• Emotional lability and mood swings

It’s important to tackle these issues to improve treatment results and life quality for those with Sydenham Chorea.

Cardiac Involvement in Rheumatic Fever

Sydenham Chorea is often tied to rheumatic fever. This can lead to heart problems. Rheumatic fever can harm the heart, causing issues like:

• Valvular heart disease
• Myocarditis (inflammation of the heart muscle)
• Pericarditis (inflammation of the outer lining of the heart)
• Heart failure in severe cases

It’s vital to keep an eye on the heart for those with rheumatic fever history. This helps catch and manage heart problems early.

Healthcare teams should watch for these complications and related conditions in Sydenham Chorea patients. Early spotting and treatment of neuropsychiatric and heart issues can make a big difference. It can lead to better outcomes and a better life for those affected.

Sydenham Chorea and PANDAS

Sydenham chorea and PANDAS are two neurological disorders with some similarities but key differences. Both are linked to streptococcal infections, but they impact different age groups and show unique symptoms. The study of their connection is ongoing and sparks debate.

Similarities and Differences

Here are the main similarities and differences between Sydenham chorea and PANDAS:

Both disorders are linked to neuropsychiatric symptoms caused by streptococcal infections. PANDAS is marked by sudden OCD and/or tics, while Sydenham chorea is known for involuntary movements and behavioral changes. The connection between PANDAS and rheumatic fever is also debated.

Controversies and Ongoing Research

The diagnosis and treatment of PANDAS are topics of debate. Some see it as a unique disorder, while others think it’s a subtype of other conditions. Research aims to uncover the underlying causes and the link to streptococcal infections.

The use of antibiotics in treating Sydenham chorea and PANDAS is also unclear. Some studies suggest antibiotics can help, but others show no benefit. More research is needed to guide treatment.

As scientists study Sydenham chorea and PANDAS, patients and families look forward to new discoveries and treatments. Collaboration between researchers, doctors, and patient groups is key to understanding and treating these disorders.

Living with Sydenham Chorea

Sydenham Chorea can be tough for patients and their families. It brings involuntary movements, behavioral changes, and emotional challenges. But, there are coping strategies and resources to help manage these issues.

Coping Strategies for Patients and Families

Living with Sydenham Chorea means finding ways to cope. Here are some strategies:

• Keeping a routine helps with structure and predictability.
• Relaxation techniques like deep breathing or meditation can reduce stress.
• Modifying your home for safety and mobility is important.
• Open communication within the family helps express feelings and concerns.
• Professional counseling or therapy can address emotional and psychological needs.

Support Groups and Resources

Connecting with others who face similar challenges is valuable. Support groups, both in-person and online, provide a space to share and support each other. Some key organizations and resources include:

By using these coping strategies and accessing support groups and resources, patients and families can find the strength and support needed. This helps them navigate the challenges of Sydenham Chorea.

Advances in Research and Treatment

In recent years, we’ve made big steps in understanding Sydenham Chorea. Researchers are diving deep into how streptococcal infections, the immune system, and brain function are linked. Their goal is to find treatments that can ease symptoms and help patients feel better.

Emerging Therapies and Interventions

New treatments for Sydenham Chorea aim to control the immune system and reduce brain inflammation. Treatments like IVIG and plasma exchange are showing promise. Also, scientists are looking into new medicines that target brain chemicals involved in movement. These efforts aim to offer more tailored treatments for patients.

Future Directions and Challenges

Even with progress, there are many challenges ahead. We need more research to fully understand Sydenham Chorea and find reliable ways to diagnose and track it. Working together, experts from different fields will help us make big strides. Creating clear guidelines for diagnosis and treatment is key to giving patients the best care possible.