Sydenham chorea is a neurological disorder accompanied by erratic muscle contractions and motor disorders. Symptoms of the disease occur in the form of attacks of hyperkinetic activity; psychoemotional disorders are noted. The diagnosis is made based on the clinical picture, laboratory data, MRI or CT, electromyography, EEG. Treatment consists in prescribing antibiotics, nonsteroidal anti-inflammatory drugs, glucocorticosteroids, neuroleptics. Powerful hormonal drugs, anticonvulsant medications can also be used.
General information
Sydenham chorea is a disease that manifests itself in the form of hyperkinesis, developing as a result of damage to the structures responsible for muscle tone and coordination of movements. The treatment of the pathological process is within the competence of a neurologist. The disease is most often detected in childhood against the background of rheumatic changes. Girls get sick more often – this is due to the hormonal characteristics of the body and the production of female sex hormones. The involvement of the cerebellum and striar structures of the brain in the pathological process is of the greatest importance during the course of the disease. The duration of the choreic attack is approximately 12 weeks, it can be delayed up to 5-6 months, less often for years (1-2). Relapses of the disease are possible.
Causes
Sydenham chorea most often progresses in 10-12 years, against the background of a streptococcal infection (sore throat, pharyngitis or tonsillitis), after the appearance of complications of infectious processes. Rheumatism can provoke the development of the disease. Experts note a hereditary or familial predisposition to the progression of the disease. Modern neurology has not yet fully studied the nature of this neurological disorder. Research is still underway in this area, which should reveal all the features of Sydenham chorea and many other neurological disorders.
Risk factors of the disease: unfavorable heredity; hormonal disorders; rheumatism; the presence of carious defects and weak immunity; psychological abnormalities; chronic infectious processes, especially if they are localized in the organs of the upper respiratory tract. The pathological substrate of the Sydenham chorea is the tissues of the nervous system damaged as a result of inflammatory, degenerative and vascular changes.
Symptoms
The clinical picture of Sydenham chorea is characterized by various manifestations. There are attacks of hyperkinetic activity, followed by normal behavior and stabilization of the condition. Patients have uncoordinated movements, muscle tone decreases, there is psychoemotional instability, increased nervousness, a tendency to irritation, tearfulness.
The main symptoms of the pathological process can last several weeks or more. Patients make strange sounds (hyperkinesis of the larynx) that attract the attention of others and frighten them, so most children suffering from this neurological pathology cannot study at school, and with frequent relapses they are forced to be home-schooled. Hyperkinesis covers the muscles of the face, limbs, the whole body; at the end of the movement there is a short fading.
With a Sydenham chorea, mental disorders can develop. Patients experience emotional lability, increased anxiety, decreased memory and ability to concentrate attention. These manifestations develop at the very beginning of the disease and persist between hyperkinetic attacks. The severity of hyperkinesis varies. Sometimes sick children are not much different from their healthy peers. Like children with ADHD, children with Sydenham chorea are restless, restless, too active. In some patients, swallowing disorders and diction problems are detected.
Complications of the rheumatic process are acquired heart defects (mitral stenosis, aortic insufficiency). The consequences of the disease can also be general weakness, sleep disorders, neuropsychiatric disorders, etc.
Diagnostics
When treating a patient with suspected Sydenham chorea, the neurologist carefully examines the anamnesis, conducts an examination, and prescribes appropriate laboratory and diagnostic tests. First of all, the doctor determines whether the patient has signs of rheumatic damage to the body; looks for symptoms of concomitant pathologies (rheumocarditis, polyarthritis); prescribes additional studies. Laboratory blood tests make it possible to determine markers of streptococcal infection (antisteptolysin-O, C-reactive protein, rheumatoid factor), and with the help of electroencephalography (EEG), it is possible to determine the diffuse appearance of slow waves of bioelectric activity of the brain of a sick person.
The cerebrospinal fluid is also being studied (it has not been changed); electromyography is prescribed, which gives information about the biopotentials of skeletal muscles and violations in their work, characteristic of this pathology. To exclude focal changes in cerebral structures, MRI or CT scans of the brain are used, which can detect non-specific signal changes in the area of the shell and caudate nuclei. PET of the brain in the active stage of the Sydenham chorea determines the increased glucose metabolism in the striatum and thalamus. The disease is differentiated from tics that have a more stereotypical course (local lesion and negative Gordon’s symptom); viral encephalitis and dysmetabolic encephalopathies.
Treatment
Today, the possibilities of neurology allow us to suspect the development of Sydenham chorea long before the appearance of pronounced symptoms. To do this, you need to be diagnosed and consult an experienced neurologist or geneticist. Treatment should cover both the causes and signs of the disease, be comprehensive and as modern as possible.
Sydenham chorea may be accompanied by mental disorders. Sick children are often aggressive, quarrelsome, stubborn, which requires individual psycho-emotional adjustment and long work with child psychologists, psychiatrists and pediatric neurologists. Specialists prescribe immunosuppressants, sedatives to improve the patient’s sleep, relieve anxiety, and increase social adaptation.
To eliminate the signs of Sydenham chorea, hormonal drugs, anti-inflammatory, antibacterial agents are prescribed. Neuroleptics, sleeping pills, anticonvulsant medications are used. Patients with Sydenham chorea need constant professional monitoring and diagnostic supervision.
In the acute period, the patient should stay in bed, not overwork; stay in a separate room where there are no light, sound stimuli. Patients need constant care and attention. If sedatives do not allow to stop an attack of hyperkinesis, then corticosteroids are prescribed. Antihistamines are also used.
Prognosis and prevention
Sydenham chorea does not pose a particular threat to the patient’s life (the fatal outcome from heart disorders against the background of minor chorea is only 1-2% of the total number of sufferers from this pathology) and with high-quality treatment and care can self-withdraw or go into a long stage of remission. But even after full recovery, short-term relapses of Sydenham chorea are possible against the background of pregnancy, exacerbation of viral and infectious processes, especially streptococcal nature (group A streptococci).
Non-specific preventive measures for the development of Sydenham chorea include timely and adequate antibiotic therapy in patients with rheumatism and other streptococcal infections. Early detection and treatment of rheumatoid manifestations significantly reduces the incidence of Sydenham chorea among children aged 6-15 years.
