A patient with chorea presents with persistent involuntary, purposeless, and usually symmetric movements of the extremities and muscular incoordination. [moh-it.pure.elsevier.com]

ANeurA were present in the sera of 100, 93, and 44% of the patients with acute, chronic, and past histories of RhCh, respectively. A definition of chronic chorea is presented for the first time. None of the control subjects had ANeurA in their sera. [ncbi.nlm.nih.gov]

It is usually late onset, occurring upto 6 months after acute infection but may occasionally be present as presenting symptom of rheumatic fever. It is a self-limiting condition with spontaneous remission lasting from 1 week to 6 months. [msjonline.org]

[…] of "pure" chorea, the present report calls attention to the rare group of patients. .. [nejm.org]

• Fever

  Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence. [ncbi.nlm.nih.gov]

  What Causes Rheumatic Fever? Rheumatic fever results from an inflammatory reaction to certain group A streptococcusbacteria. [webmd.com]

  Why: sydenham's chorea is seen in rheumatic fever. The chorea may be associated with other rheumatic manifestations or it may present as the sole expression of rheumatic fever. [icd10data.com]

  Hemichorea as a presentation of acute rheumatic fever. Chang Gung Med J. 2006;29:612-6. Woo CLF, Liu KT, Young BWY. Acute rheumatic fever presenting with Sydenham’s chorea. HK J Paediatr (new series). 2003;8:198-202. Klawans HL, Brandabur MM. [msjonline.org]

• Weakness

  In some extremely rare cases (less than 2 percent), severe muscle weakness, irritability, or confusion may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea. [rarediseases.org]

  Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability. [ninds.nih.gov]

  A variable intensity of muscle weakness may be present, leading to inability to sustain a contraction. In clinical forms with predominance of hypotonia (mild chorea) hyperkinesia is attenuated. [doctortipster.com]

  Other symptoms of the disorder may include diminished muscle tone, muscle weakness, and emotional and behavioural disturbances, particularly obsessive-compulsive behaviours. [brainfoundation.org.au]

  Chorea (sydenham's): a neurological disorder characterized by purposeless, rapid, involuntary movements, emotional lability, and muscular weakness. Why: sydenham's chorea is seen in rheumatic fever. [icd10data.com]

• Falling

  Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability. [ninds.nih.gov]

  As symptoms progress, the person may appear clumsy and drop things or fall frequently. Eventually, the uncontrollable, jerky, and irregular movements develop. Varying degrees of speech impairment are also seen as well as emotional instability. [medicineonline.com]

  The global burden of disease caused by rheumatic fever currently falls disproportionately on children living in the developing world, especially where poverty is widespread. [pedclerk.uchicago.edu]

  The antibody titre falls as the disease improves and rises again in relapse [ 12 ]. MRI studies are often normal but MRI spectroscopy may reveal autoimmune damage to the basal nuclei [ 13 ]. [patient.info]

  Epidemiology• Ages 5-15 yrs are most susceptible• Rare boys• Common in 3rd world countries• Environmental factors-- over crowding, poor sanitation, poverty,• Incidence more during fall ,winter & early spring10/27/2012 5 6. [slideshare.net]

• Muscle Weakness

  In addition to choreic movements, individuals with Sydenham chorea may develop muscle weakness, slurred speech (dysarthria), diminished muscle tone (hypotonia), tics, obsessions, compulsions, inattention, anxiety, labile mood, and decreased verbal output [rarediseases.org]

  A variable intensity of muscle weakness may be present, leading to inability to sustain a contraction. In clinical forms with predominance of hypotonia (mild chorea) hyperkinesia is attenuated. [doctortipster.com]

  Other symptoms of the disorder may include diminished muscle tone, muscle weakness, and emotional and behavioural disturbances, particularly obsessive-compulsive behaviours. [brainfoundation.org.au]

  More severe cases can cause the muscles of the arms, legs, or face to twitch uncontrollably. It can also be associated with muscle weakness and emotional outbursts. Because of antibiotics, rheumatic fever is now rare in developed countries. [webmd.com]

  These movements can affect the muscles in the limbs, face and trunk. Sometimes other effects appear alongside the movements, such as muscle weakness, poor muscle tone and clumsiness. [gosh.nhs.uk]

• Facial Grimacing

  The symptoms vary in severity, from mild cases in which there is facial grimacing, restlessness and slight incoordination to severe cases where involuntary movements make it impossible for the child to function normally. [brainfoundation.org.au]

  Other neurologic symptoms include behavior change, dysarthria, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache, slowed cognition, facial grimacing, fidgetiness and hypotonia. [3] [4] Also, there [en.wikipedia.org]

  In milder cases of Sydenham's, the patient may have only facial grimacing and some difficulty putting on clothes or doing other tasks that require fine coordination. [encyclopedia.com]

  Characteristic findings include fluctuating grip strength (milkmaid’s grip), tongue fasciculations or tongue darting (the tongue cannot protrude without darting in and out), facial grimacing, and explosive speech with or without tongue clucking. [merckmanuals.com]

• Chorea

  chorea; Sydenham, chorea; minor, chorea; rheumatic, Sydenham; chorea, minor; chorea, rheumatic; chorea, rheumatic chorea, Sydenhams chorea Dutch reumatische chorea, Sydenham; chorea, chorea; Sydenham, chorea; minor, chorea; reumatisch, minor; chorea [fpnotebook.com]

  Therapy with prednisone shortened the duration of rheumatic chorea; some reported recurrences of chorea and had minor neurologic sequelae. [ncbi.nlm.nih.gov]

  Codes I02 Rheumatic chorea I02.0 Rheumatic chorea with heart involvement I02.9 Rheumatic chorea without heart involvement [icd10data.com]

• Involuntary Movements

  Chorea (sydenham's): a neurological disorder characterized by purposeless, rapid, involuntary movements, emotional lability, and muscular weakness. Why: sydenham's chorea is seen in rheumatic fever. [icd10data.com]

  The symptoms of SC can vary from a halting gait and slight grimacing to involuntary movements that are frequent and severe enough to be incapacitating. [ninds.nih.gov]

  The various clinical signs of rheumatic chorea were scored with MAIMS score (Modified Abnormal Involuntary Movement Scale score) which is used for tardive dyskinesia. No other drug for abnormal movements was used. [ncbi.nlm.nih.gov]

  A year later movements relapsed with extension to left emisoma. She began haloperidol with remission 6 months after. R. 12 y.o, came to our observation for involuntary movements of limbs and trunk. [ped-rheum.biomedcentral.com]

  Nerve cells in the brain deteriorate, producing sudden, involuntary movements that are jerky and purposeless. These movements gradually become more severe and affect all movement. [medicineonline.com]

• Irritability

  Irritability, anxiety, and emotional instability, chiefly episodes of crying initiated by trivial incidents, are also common symptoms. [britannica.com]

  In some extremely rare cases (less than 2 percent), severe muscle weakness, irritability, or confusion may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea. [rarediseases.org]

  How: typically, the onset of chorea is gradual, with irritability, uncooperativeness, fits of anger, crying, and inappropriate behavior present before the choreiform movements are noted. [icd10data.com]

  This may make some children feel irritable or angry. They may also have mood swings. How is Sydenham's chorea diagnosed? Sydenham's chorea is often diagnosed by asking questions, examining the child and hearing how the condition happened. [gosh.nhs.uk]

  Ataxia Ataxia is unsteady movements or inability to control movements and typically is caused by cerebellar dysfunction from irritation or disruption of cerebellar tracts. Loss of peripheral sensory input can also cause ataxia. [pediatrics.emory.edu]

• Tremor

  Tremor Tremor is a regular osillatory movement of the extremities related to posture or activity. [pediatrics.emory.edu]

  Among 33 patients seen at a median of 10.3 years (range 6.3-14.9 years) after their initial bout of chorea, 20% reported residual tremor or mood swings. Ten of the 33 (30%) had one or more recurrences of chorea. [ncbi.nlm.nih.gov]

  Tremors are a type of dyskinesia. Nerve diseases cause many movement disorders, such as Parkinson's disease. Other causes include injuries, autoimmune diseases, infections and certain medicines. [icdlist.com]

  Thyrotoxicosis: Tremor, tachycardia, lidlag and goitre (may be present) 6. Drugs: Take a complete drug history [epomedicine.com]

  Familial benign chorea with intention tremor: a clinical entity. J Pediatr. 1967 May. 70(5):724-9. [Medline]. Poewe WH, Kleedorfer B, Willeit J, Gerstenbrand F. [emedicine.medscape.com]

• Dysarthria

  In addition to choreic movements, individuals with Sydenham chorea may develop muscle weakness, slurred speech (dysarthria), diminished muscle tone (hypotonia), tics, obsessions, compulsions, inattention, anxiety, labile mood, and decreased verbal output [rarediseases.org]

  Dysarthria, dyskinesias, and milk maid grasp. Chorea will suppress with haloperidol. [pediatrics.emory.edu]

  Dysarthria is frequent, and patients may present with a reduction of verbal frequency (“disinclination to speak”). [clinicaladvisor.com]

  Other neurologic symptoms include behavior change, dysarthria, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache, slowed cognition, facial grimacing, fidgetiness and hypotonia. [3] [4] Also, there [en.wikipedia.org]

  Associated features may include delayed motor development, dysarthria, intention tremor, athetosis, and hypotonia. Severity is highly variable but choreic movements are typically continuous and not episodic. [emedicine.medscape.com]

We suggest that carbamazepine may serve as a first-line treatment for rheumatic chorea. [ncbi.nlm.nih.gov]

Treatment There is no specific treatment for SC. For people with the mildest form, bed rest during the period of active movements is sufficient. [ninds.nih.gov]

Treatment of neuropsychiatric signs: Psychiatric signs are usually short-lived. [clinicaladvisor.com]

Prognosis Generally the prognosis for children with Sydenham’s chorea is good. Complete recovery usually occurs, often within weeks of the onset. [brainfoundation.org.au]

Prognosis Most children recover completely from SC, although a small number will continue to have disabling, persistent chorea despite treatment. [ninds.nih.gov]

Prognosis• Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines• Good prognosis for older age group & if no carditis during the initial attack• Bad prognosis for younger children [slideshare.net]

References: [4] [17] [18] Prognosis Prevention Primary prevention : prompt antibiotic treatment (e.g., penicillin V ) of GAS tonsillopharyngitis diagnosed by throat culture or rapid strep test Secondary prevention Antibiotic prophylaxis to prevent recurrence [amboss.com]

Outlook (Prognosis) Sydenham chorea usually clears up in a few months. In rare cases, an unusual form of Sydenham chorea may begin later in life. Possible Complications No complications are expected. [leehealth.org]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

This book describes in detail the latest clinical and etiological information regarding chorea. Management strategies, pathophysiology, and associated medical and psychiatric problems associated with chorea are also addressed. [books.google.com]

Severe personality disorder and rheumatic fever are two such etiologic agents which may be operative in Sydenham's chorea. [pediatrics.aappublications.org]

Selected tests should be performed to identify evidence for a streptococcal infection and to identify possible alternative etiologies. [clinicaladvisor.com]

The endogenous toxin hypothesis of the etiology of Parkinson’s disease and a pilot trial of high discharge antioxidant in an attempt to show the progression of illness. Ann N Y Acad Sci 1989; 570: 186–196. PubMed CrossRef Google Scholar 5. [link.springer.com]

Abstract To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years [ncbi.nlm.nih.gov]

The influence of this is likely to be truly spectacular and special, as this will help in bringing many patients, into the net of penicillin prophylaxis and may assist in changing the epidemiological face of the disease. [books.google.com]

Epidemiologic implications: It is reasonable to consider individuals at low risk of ARF if they come from a setting or population with known low rates of ARF and RHD (Class IIa, Level of Evidence C). [acc.org]

References: [2] [3] [4] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Kaplan Patogenesis of acute rheumatic fever and rheumatic heart disease: evasive after half a century of clinical, epidemiological and laboratory investigation Heart., 91 (2005), pp. 3-4 [51] National Heart Foundation of Australia and the Cardiac Society [apcontinuada.com]

Management strategies, pathophysiology, and associated medical and psychiatric problems associated with chorea are also addressed. [books.google.com]

A discussion of the pathophysiology, clinical presentation, and management of SC in the emergency department follows. Department of Emergency Medicine, Tufts-New England Medical Center, Boston, MA. [journals.lww.com]

Most common form of adulthood chorea: Huntington’s chorea and Drug-induced chorea Pathophysiology of Chorea Site of lesion: Striatum (Putamen/Caudate nucleus) For details about neurophysiology and neuropathology of basal ganglia, read here. [epomedicine.com]

Neuropsychiatric symptoms (e.g., inappropriate laughing/crying, agitation, anxiety, apathy, obsessive-compulsive behavior) Epidemiology ♀ < ♂ ( ∼ 2:1 ) Longer latent period than other rheumatic manifestations (presents several months following GAS infection ) Pathophysiology [amboss.com]

Prevention & Expectations What can be done to prevent the condition? Most cases of Sydenham chorea can be prevented by early diagnosis and prompt treatment of streptococcal infections, such as strep throat. [medicineonline.com]

Prevention Pay careful attention to children's complaints of sore throats and get early treatment to prevent acute RF. [leehealth.org]

The provider may also prescribe antibiotics to prevent future RF infections. This is called preventive antibiotics, or antibiotic prophylaxis. Severe movement or emotional symptoms may need to be treated with medicines. [medlineplus.gov]

Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence. [ncbi.nlm.nih.gov]

• To prevent streptococcal infections which precipitate recurrences of rheumatic fever• Prevent development of chronic rheumatic heart disease• If recurrences are prevented, 70% of patients with carditis in the initial attack will eventually have normal [slideshare.net]