Rheumatic chorea, also known as Sydenham's chorea, is a neurological disorder characterized by rapid, involuntary movements, emotional instability, and muscle weakness. It is a major manifestation of rheumatic fever, which is a complication of untreated streptococcal throat infections. The condition primarily affects children and adolescents and is more common in females than males.
Presentation
Patients with rheumatic chorea typically present with sudden, jerky, and uncoordinated movements that can affect any part of the body. These movements are often more pronounced in the face, hands, and feet. In addition to motor symptoms, patients may experience emotional lability, such as sudden bouts of crying or laughing, and cognitive difficulties. Muscle weakness and a decline in handwriting skills are also common. Symptoms can vary in severity and may fluctuate over time.
Workup
The diagnosis of rheumatic chorea is primarily clinical, based on the characteristic symptoms and a history of recent streptococcal infection. Laboratory tests may include throat cultures or rapid antigen tests to confirm a recent streptococcal infection. Blood tests may show elevated levels of anti-streptolysin O (ASO) antibodies, indicating a recent infection. Imaging studies, such as MRI, are generally not required but can be used to rule out other neurological conditions.
Treatment
Treatment of rheumatic chorea focuses on managing symptoms and preventing further episodes of rheumatic fever. Medications such as haloperidol or valproic acid may be used to control involuntary movements. Long-term antibiotic prophylaxis with penicillin is recommended to prevent recurrent streptococcal infections and subsequent rheumatic fever episodes. Supportive care, including physical therapy and psychological support, can help manage symptoms and improve quality of life.
Prognosis
The prognosis for rheumatic chorea is generally favorable, with most patients experiencing a complete resolution of symptoms within a few months to two years. However, some individuals may have persistent symptoms or experience relapses. Early diagnosis and treatment of streptococcal infections are crucial in preventing rheumatic fever and its complications, including rheumatic chorea.
Etiology
Rheumatic chorea is caused by an autoimmune reaction following a streptococcal throat infection. The body's immune system mistakenly attacks its own tissues, including the brain, leading to the characteristic symptoms of chorea. This autoimmune response is part of the broader spectrum of rheumatic fever, which can also affect the heart, joints, and skin.
Epidemiology
Rheumatic chorea is most commonly seen in children and adolescents, particularly those aged 5 to 15 years. It is more prevalent in females than males. The incidence of rheumatic fever, and consequently rheumatic chorea, has declined significantly in developed countries due to improved diagnosis and treatment of streptococcal infections. However, it remains a significant health concern in developing regions with limited access to healthcare.
Pathophysiology
The pathophysiology of rheumatic chorea involves an autoimmune response triggered by a streptococcal infection. Antibodies produced against the bacteria cross-react with neuronal tissues in the basal ganglia, a region of the brain involved in movement control. This cross-reactivity leads to the dysfunction of neural circuits, resulting in the involuntary movements and other symptoms associated with chorea.
Prevention
Preventing rheumatic chorea involves the prompt diagnosis and treatment of streptococcal throat infections. Antibiotic therapy, typically with penicillin, is effective in eradicating the bacteria and preventing the development of rheumatic fever. Long-term antibiotic prophylaxis is recommended for individuals with a history of rheumatic fever to prevent recurrence and associated complications.
Summary
Rheumatic chorea is a neurological disorder resulting from an autoimmune reaction to a streptococcal infection. It is characterized by involuntary movements, emotional instability, and muscle weakness. Early diagnosis and treatment of streptococcal infections are crucial in preventing rheumatic fever and its complications. While the prognosis is generally good, ongoing management and prevention strategies are essential to reduce the risk of recurrence.
Patient Information
If you or your child experiences sudden, involuntary movements, emotional changes, or muscle weakness following a sore throat, it may be important to consider the possibility of rheumatic chorea. This condition is linked to a previous streptococcal infection and is part of the broader spectrum of rheumatic fever. Treatment involves managing symptoms and preventing further infections with antibiotics. With appropriate care, most individuals recover fully, although some may experience persistent symptoms or relapses.