Sydenham chorea is a neurological disorder defined by rapid, irregular movements, primarily affecting children and teenagers. It is one of the major manifestations of acute rheumatic fever (ARF) and is often associated with a preceding Group A Streptococcal (GAS) infection, such as strep throat.
In this article, we will examine Sydenham chorea’s symptoms, causes, and various treatment options to provide a comprehensive understanding of this neurological disorder.
Sydenham chorea
Syndenhan chorea is a neurological condition defined by quick, uncontrollable, and uncoordinated movements. It mainly affects kids and teenagers, and it’s frequently linked to acute rheumatic fever (ARF), which happens after contracting a Group A streptococcus (GAS) infection that causes symptoms like strep throat or scarlet fever. The disorder derives its name from Thomas Sydenham, who first described it in the 17th century.
Symptoms
The following are the few symptoms of Sydenham chorea:
Chorea
The indicator of the disorder, chorea, involves rapid, uncontrollable, and irregular movements. These movements are often described as jerky and can affect the face, hands, feet, and other body parts. The severity may change over time, occasionally getting better or worse. Stress and exhaustion might aggravate these motions, making daily chores more difficult.
Muscle Weakness
Muscle strength can decrease in patients, which may impair their capacity to carry out everyday tasks. This shortcoming may make it difficult to complete tasks that call for accuracy or prolonged effort. As a result of decreased activity, muscle atrophy could develop over time.
Emotional Lability
Patients may have sudden and dramatic mood swings, frequently coming across as extremely sensitive or agitated. The patient and in-turn their family may experience disturbing mood fluctuations that affect relationships and social interactions. There may be periods of crying or laughter that have no apparent reason.
Attention Deficit
It is possible to experience difficulty focusing and paying attention, which can affect daily functioning and academic performance. It may result in subpar academic performance and trouble finishing assignments. In certain situations, hyperactivity and impulsivity may also be observed.
Speech Difficulties
A condition affecting the speech muscles can cause speech difficulties, resulting in slurred or stopped speech. If communication is challenging, speech treatment may be necessary. Patients may have trouble pronouncing words effectively, and their speech may become irregular or uncomfortable.
Difficulty Swallowing
When chorea is severe, it can affect the swallowing muscles, making it difficult to eat and drink. If neglected, this might result in weight loss and nutritional deficits. To prevent choking, patients might need to use particular swallowing skills or consume softer meals.
Causes
Sydenham chorea is primarily caused by an autoimmune reaction following an infection with Group A Streptococcus (GAS). The condition is a manifestation of acute rheumatic fever (ARF), a delayed autoimmune response to the streptococcal infection. Here are the steps leading to the development of Sydenham chorea:
Streptococcal Infection
The first cause is a Group A Streptococcus (GAS) infection, such as strep throat or scarlet fever. Symptoms of this infection, which usually affects the throat, include fever, swollen lymph nodes, and sore throat. Prompt treatment with antibiotics can help in preventing complications. If untreated, the infection can lead to ARF (acute rheumatic fever) within a few weeks.
Immune Response
In response to streptococcal bacteria, the body’s immune system produces antibodies to fight the infection. These antibodies treat the disease by targeting and neutralising the germs. However, the immune system might still attack the body’s own tissues when it becomes overactive.
Cross-Reactivity
Cross-reactivity occurs when some of the antibodies the immune system produces unintentionally target the body’s tissues. In cases of Sydenham chorea, these antibodies target the basal ganglia, a network of brain nuclei involved in motor control. This misdirected immune response is due to the structural similarities between streptococcal antigens and specific brain tissues.
Inflammation and Damage
The inflammatory and damaged basal ganglia caused by the autoimmune onslaught give rise to the typical chorea symptoms. This inflammation disrupts normal brain function, causing involuntary movements and other neurological symptoms. Variations in the level of damage can influence the severity and length of symptoms.
Treatment
Treatment concentrates on managing symptoms, preventing complications, and addressing the underlying cause. Options include:
Anti-inflammatory Medications
Corticosteroids, like prednisone, and nonsteroidal anti-inflammatory medications (NSAIDs), like ibuprofen, are used to lessen inflammation in the brain. These drugs work by reducing the autoimmune reaction that attacks the basal ganglia, which helps reduce symptoms. They may decrease involuntary motions and improve motor function more quickly.
Anticonvulsants
Drugs that regulate brain neuronal activity, such as carbamazepine or valproic acid, are used to inhibit involuntary movements. When chorea is severe and the motions are incapacitating, these medications are beneficial. Anticonvulsants lessen neuronal excitability, which reduces the erratic, irregular movements that are characteristic of Sydenham chorea.
Neuroleptics
The antipsychotic drugs haloperidol and risperidone work by inhibiting dopamine receptors in the brain, which helps treat chorea. Patients who have psychiatric symptoms or who do not respond well to anticonvulsants may benefit from these drugs. Neuroleptics relieve motor symptoms by reducing the activity of hyperactive brain circuits that result in involuntary motions.
Antibiotic Therapy
Antibiotics like penicillin are used to treat the original streptococcal infection and stop recurrences. They are essential to avoid acute rheumatic fever and the consequent development of Sydenham chorea. For those who have experienced rheumatic fever in the past, long-term antibiotic prophylaxis may also be advised to prevent infections and complications in the future.
Immunomodulatory Therapies
In severe cases, treatments such as plasmapheresis and intravenous immunoglobulin (IVIG) can be used to regulate the immune response. IVIG entails infusing antibodies made from donated blood to help neutralise the toxic antibodies targeting the basal ganglia. Plasmapheresis involves filtering the blood to get rid of these antibodies. The goal of both therapies is to reduce inflammation and autoimmune activity in the brain.
Conclusion
In conclusion, an immunological reaction to a streptococcal infection causes Sydenham chorea, a severe neurological condition that mainly affects children and teenagers. Effective therapy and prevention of this disorder depend on an understanding of its causes, symptoms, and prevention methods. Sydenham chorea can be greatly decreased by treating streptococcal infections as soon as possible, taking preventative measures, and offering complete care to those who are affected.
