Korea Syndrome Sydenham’s chorea —also known as “minor chorea”, “St. Vitus’s disease” or “St. Vitus’s dance”, chorea sancti viti, “acute chorea” or “rheumatic chorea” is an infectious disease of the nervous system central , due to rheumatic fever after pharyngotonsillitis caused by the bacterium Streptococcus pyogenes . Some rare cases may be associated with pregnancy, and in this case they are called “chorea gravidarum” or chorea gravidarum.
Summary
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- 1 Definition
- 2 Clinical picture
- 3 Etiology
- 4 Benign Hereditary Korea
- 5 Sydenham Korea
- 6 Pathophysiology
- 7 Clinical picture
- 8 Diagnosis
- 9 Complementary exams
- 10 Differential diagnosis
- 11 Complications
- 12 Evolution and prognosis
- 13 Prevention
- 14 External links
- 15 Sources
Definition
The Latin term chorea, in turn from the Greek khoreia (χορεία), comes from the same root as chorus (khoros, χορός), chorus girl, choreographer and choreography, and refers to the involuntary movements of the disease, which simulate a violent dance . The disease is named after the physician Thomas Sydenham (1624 – 1689), called “the English Hippocrates”. However, it had been described before him by the German Gregor Horstius. Due to the vulnerability of the basal ganglia and their connections to a wide variety of pathologies, the differential diagnosis of chorea is very wide. Chorea in childhood arises as a consequence of many different causes, both acquired and hereditary., including metabolic, endocrine diseases , infections , autoimmune diseases , cerebrovascular diseases , neoplasms , toxins, neurodegenerative diseases or trauma.
Clinical picture
Throat pain and irritation often precede the clinical manifestations of chorea itself. This is due to oropharyngeal infection with S. pyogenes. After 1 to 5 weeks, there are sudden and acute symptoms of rheumatic fever. Normally, the first and main specific symptom of Sydenham’s chorea usually refers to inexplicable changes in the stroke of the handwriting. Choreic signs consist of uncontrollable and spasmodic contractions of various muscle groups, ineffective and similar to fasciculations (the latter do not cause displacement of the joint). Almost always, the patient has lost fine motor skills in the fingers and hands, which explains the pathological changes in handwriting. The choreic movements are continuous, incessant, repetitive and, Although they are usually located on the limbs, it is not uncommon to also see them on the face and neck. This phenomenon is more common in the gravity form. Although they do not normally seriously affect the life of the individual, in severe cases they can cause disorders of speech, locomotion, the use of the arms, and, in general, disrupt the physical capacity of the patient. Clinical manifestations may also include emotional or mental disturbances: loss of emotional control, unwarranted fits of crying or laughing, as well as disrupt the physical capacity of the patient. Clinical manifestations may also include emotional or mental disturbances: loss of emotional control, unwarranted fits of crying or laughing, as well as disrupt the physical capacity of the patient. Clinical manifestations may also include emotional or mental disturbances: loss of emotional control, unwarranted fits of crying or laughing, as well asobsessive-compulsive disorders.
Etiology
Due to the vulnerability of the basal ganglia and their connections to a wide variety of pathologies, the differential diagnosis of chorea is very wide. Chorea in childhood arises as a consequence of very diverse causes, both acquired and hereditary, including metabolic, endocrine diseases, infections, autoimmune diseases, cerebrovascular diseases, malignancies, toxins, neurodegenerative diseases or trauma. The most frequent are those secondary to fixed encephalopathies, secondary to drugs and Sydenham’s Korea (in the context of rheumatic fever), although the latter is less and less frequent in developed countries. Table 1 shows the fundamental causes of chorea as the predominant or initial symptom. Other pathologies can occur with chorea,
Benign Hereditary Korea
It is a rare, autosomal dominant disease (although there are cases described with autosomal recessive or X-linked inheritance), non-progressive, characterized by the early onset (between 1 and 5 years of age) of chorea. The course is initially stable, with subsequent improvement in adulthood. The diagnosis is suspected in children with an onset Korea before 5 years of age, with normal intellectual development, without regression or loss of cognitive abilities, without other associated severe neurological complications such as epilepsy, and in general and neurological examination normal to except for chorea, although it can associate other symptoms and signs such as ataxia, tremor and dysarthria. MRI is generally normal. Benign hereditary chorea is caused, in part, but not all cases, by a mutation in the NKX2 gene.
Sydenham’s Korea
Sydenham’s chorea is a late complication of group A hemolytic streptococcal infections and is one of the main criteria for rheumatic fever. It is the most common form of chorea produced by immunological mechanisms. It predominates in women and its highest incidence is between 5 and 15 years. The clinic usually begins four to eight weeks after group A beta-hemolytic strep throat. Most patients with Sydenham’s chorea develop other symptoms of rheumatic fever, such as heart involvement (60-80% of cases, mitral valve disease). ) and arthritis (30%). Chorea as the only finding occurs in approximately 20% of cases. It has an insidious onset, associating behavioral disorders and of highly variable intensity, which may initially be unilateral, but finally it is generalized in the majority of patients. It usually causes difficulties in activities of daily living and fine motor skills. Abnormal movements decrease or subside during sleep. They usually associate neuropsychiatric symptoms that include inattention, anxiety, obsessive-compulsive disorder, paranoia. Regarding the pathophysiology, antibodies have been identified against the caudate and subthalamic nucleus in children with Sydenham’s Korea, although these are not specific, having been found in Parkinson’s disease, as well as in a lower proportion of healthy people. Identifying streptococcal M proteins that can cause antibodies that cross-reacted with the human brain suggests that molecular mimicry may play a role. When an acute or subacute chorea appears, it is important to ask about the history of the infection during the previous 6 months with headache, fever and sore throat. A previous positive culture is of great help. The determination of ASLO antibodies and anti DNAs can be useful, although the interpretation of the results must be careful, since their elevation is not specific. On examination, it is essential to evaluate the child carefully to detect the presence of a systolic heart murmur, arthritis or arthralgias. The high prevalence of neuropsychiatric disorders in children with Sydenham’s chorea and in a group of children after streptococcal infections has been the subject of intense investigation, defining an entity parallel to Sydenham’s chorea, but distinct, called PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections). Sydenham’s chorea is usually a self-limiting disorder with spontaneous remission after eight to nine months. When symptomatic treatment is necessary, the most effective drugs are neuroleptics and tetrabenacin. When not useful, another option is benzodiazepines. Prophylactic treatment with penicillin is indicated until age 21. Based on the pathophysiology of Sydenham’s chorea, it is reasonable to consider immunomodulatory therapy to shorten the course of the disease. Benefits with such therapies have been reported in clinical case series and small clinical trials (primarily prednisone and immunoglobulins). Sydenham’s chorea is usually a self-limiting disorder with spontaneous remission after eight to nine months. When symptomatic treatment is necessary, the most effective drugs are neuroleptics and tetrabenacin. When not useful, another option is benzodiazepines. Prophylactic treatment with penicillin is indicated until age 21. Based on the pathophysiology of Sydenham’s chorea, it is reasonable to consider immunomodulatory therapy to shorten the course of the disease. Benefits with such therapies have been reported in clinical case series and small clinical trials (primarily prednisone and immunoglobulins). Sydenham’s chorea is usually a self-limiting disorder with spontaneous remission after eight to nine months. When symptomatic treatment is necessary, the most effective drugs are neuroleptics and tetrabenacin. When not useful, another option is benzodiazepines. Prophylactic treatment with penicillin is indicated until age 21. Based on the pathophysiology of Sydenham’s chorea, it is reasonable to consider immunomodulatory therapy to shorten the course of the disease. Benefits with such therapies have been reported in clinical case series and small clinical trials (primarily prednisone and immunoglobulins). When symptomatic treatment is necessary, the most effective drugs are neuroleptics and tetrabenacin. When not useful, another option is benzodiazepines. Prophylactic treatment with penicillin is indicated until age 21. Based on the pathophysiology of Sydenham’s chorea, it is reasonable to consider immunomodulatory therapy to shorten the course of the disease. Benefits with such therapies have been reported in clinical case series and small clinical trials (primarily prednisone and immunoglobulins). When symptomatic treatment is necessary, the most effective drugs are neuroleptics and tetrabenacin. When not useful, another option is benzodiazepines. Prophylactic treatment with penicillin is indicated until age 21. Based on the pathophysiology of Sydenham’s chorea, it is reasonable to consider immunomodulatory therapy to shorten the course of the disease. Benefits with such therapies have been reported in clinical case series and small clinical trials (primarily prednisone and immunoglobulins).
Pathophysiology
Group A beta hemolytic streptococcus represents antigenic stimulation through some membrane proteins (Protein M) that, when contacting monocytes or macrophages, activates them by fixing on their membrane. The activated cell becomes an activated monocyte capable of presenting the antigen to which B lymphocytes (humoral immunity) react, which are responsible for the production of antibodies against the microorganism (anti-streptolysins). In tissues, the monocyte becomes a macrophage and presents the antigen attached to the T lymphocyte (cellular immunity) on its membrane. Activated macrophages are likely to become giant Aschoff cells, which will be part of the formation of granulomatous lesions; these are pathognomonic of rheumatic carditis and are known as Aschoff’s nodules, They can remain for many years after a rheumatic attack. Activated T lymphocytes have been found in large numbers in the heart valves of subjects with active rheumatic fever, mostly T4, which are capable of generating lymphokines capable of activating proinflammatory systems, which appear to be the cause of valve inflammation. (valvulitis) during the acute attack. The acute phase of the disease is characterized by exudative and proliferative inflammatory reactions that affect connective tissue and collagen. The pathological process is diffuse, but mainly attacks the heart, brain, joints, skin and subcutaneous tissues; A generalized small vessel vasculitis is usually recorded. Other streptococci like those in group C and G are capable of eliciting an immune response,
Clinical picture
The classic clinical picture is preceded, 2-3 weeks before, by strep throat , with tonsil redness, with or without exudate, petechiae on the palate, submaxillary or laterocervical lymphadenopathy, dysphagia, high or moderate fever, abdominal pain and sometimes scarlet fever rash, later appearing the own symptoms of the disease. Symptoms start with general malaise, asthenia, adynamia, myalgia, arthralgia, hyporexia and fever. Any of the following manifestations can be associated with this table:
- Arthritis. Present in 75% of cases. It is the main sign, but the least specific. It almost always affects large joints (knees, elbows, wrists and ankles) in an asymmetric and migratory way, but it can affect small joints such as those of the hands, feet and spine. When the acute outbreak disappears, the inflammation disappears without sequelae. In cases where no treatment is received, the condition may last 2 to 3 weeks.
- Carditis. It can be seen in 40-50% of cases. It is appreciated in the 3rd week. It is the most serious manifestation of rheumatic fever since it can produce from mild manifestations to the death of the patient during the acute attack or leave sequelae that will later affect the functioning of the heart. The acute heart attack affects its three layers constituting a pancarditis (pericarditis, myocarditis and endocarditis). If carditis does not appear within 2 or 3 weeks after the attack, it is rare that it occurs.
- Sydenham’s Korea. It occurs in 2% of patients. It is considered a late manifestation, usually appearing between 2 and 3 months after the onset of streptococcal infection. The manifestation of acute attack in the central nervous system is injury to the basal ganglia and the caudate nucleus (extrapyramidal system), a late phenomenon in rheumatic fever. It is characterized by involuntary movements, lack of coordination and muscle weakness as well as emotional lability. It mainly affects the muscles of the face and extremities; speech can become explosive and difficult. They disappear during sleep, but can occur at rest and interfere with voluntary activity. The emotional changes are mainly crying and restlessness, the patients despair when they cannot control the movements of their hands or face.
- Subcutaneous nodules (Meynet’s nodules). They are observed in 10% of cases. They usually appear after the first few weeks of the disease and almost always appear in patients with carditis. They are firm, painless, move easily and can measure up to 2cm; Although they rarely appear, their presence should make carditis suspicious. They usually occur on the extensor face of the joints, particularly the elbows, knees, and wrists. They last from 1 to 2 weeks.
- Marginal erythema. It is seen in less than 5% of patients. It is a non-pruritic, macular, evanescent eruption, with a pale and circular center, with serpiginous edges, fleeting, of short duration and migratory. Its location occurs in the trunk, buttocks and the proximal region of the extremities, but not in the face. It is also more common in cases with carditis. Duration of the rheumatic attack It fluctuates between 3 weeks to 6 months, as long as there is no new streptococcal infection that prolongs the picture.
Diagnosis
It is based on the Jones criteria, modified by the American Heart association in 1992 that stipulates the major and minor criteria.
Major criteria
- Sydenham’s Korea.
- Subcutaneous nodules.
- Marginal erythema.
Minor criteria
- Arthralgia
- Fever greater than 38 C.
Paraclinical.
- Elevation of acute phase reactants: erythrocyte sedimentation and elevated C-reactive protein
- Leukocytosis; alpha-2 and increased gamma globulins.
- Prolongation of the PR interval.
- Evidence of Streptococcal infection (Group A)
Increased antibodies against streptococcus: antisptreptolysins (more than 333
U Todd) and other streptococcal antigens.
- Pharyngeal culture positive for group A streptococcus
The existence of two major signs or one major and two minor signs supported by previous infection with group A streptococcus strongly indicate the probability of Rheumatic Fever. The absence casts doubt on the diagnosis, except that it is Sydenham’s Chorea, with a long period of previous infection.
Complementary exams
It should be noted that there is no specific laboratory test for the diagnosis of rheumatic fever, although they can guide us towards the diagnosis of this entity.
- Pharyngeal culture: it only shows positivity in 10% of cases.
- Antistreptolysin O titre: its elevation above 200 U only indicates that the patient contacted the streptococcus.
- Blood count: leukocytosis, neutrophilia, polynucleosis, and moderate anemia.
- Erythrosedimentation: accelerated above 50mm in almost all patients.
- C reactive protein: high.
- Sinus tachycardia.
- Long PR interval greater than 0.16 seconds and long Qt interval.
- Disorders of repolarization of the ST segment.
Differential diagnosis
- Juvenile rheumatoid arthritis.
- Reactive arthritis.
- Septic arthritis.
- Postinfectious arthritis.
- Sickle cell anemia.
- Serum sickness.
- Drop .
- Systemic lupus erythematosus.
- Still’s disease.
- From the point of view heart, you should think of the endocarditis , myocarditis, the cardiomyopathies and Kawasaki disease.
- The differential diagnosis should also be made with skin lesions that present with various nodules and eruptions, as well as with choreic syndromes of non-rheumatic cause.
Complications
- Pericarditis .
- Heart failure .
- Atrial fibrillation.
- Subacute bacterial endocarditis or slow endocarditis.
- Venous thrombosis.
Evolution and prognosis
The disease is considered cyclical, evolving due to outbreaks with spontaneous cyclical regression between 8 and 10 weeks after the outbreak. 80% of patients reach adulthood, and of these approximately 65% can lead a normal life. Recurrences are more frequent during the 5 years after the initial outbreak and decrease progressively as time passes without rheumatic activity. They are signs of active rheumatic activity. The fever, tachycardia, leukocytosis, elevated ESR, C – reactive protein positive, weight loss.
The prognosis is determined by the degree of residual cardiac involvement, which can be excellent if relapses are prevented.
