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Rheumatic Chorea

St Vitus' Dance


Presentation

  • 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]
  • Mitral annular diameter was found to be increased (p 0.001) in patients presenting with polyarthritis alone.[ncbi.nlm.nih.gov]
  • A patient with chorea presents with persistent involuntary, purposeless, and usually symmetric movements of the extremities and muscular incoordination.[moh-it.pure.elsevier.com]
  • 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]
  • Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea.[ncbi.nlm.nih.gov]
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]
  • Abstract Detailed echocardiographic analysis was performed in 10 children with first episode of acute rheumatic fever who presented with acute rheumatic polyarthritis or rheumatic chorea and had no clinically detectable evidence of active carditis.[ncbi.nlm.nih.gov]
  • Two unrelated patients with a family history of rheumatic fever had isolated, acquired chorea. Both index cases, as well as affected family members, had increased expression of the rheumatic B-cell alloantigen D8/17.[ncbi.nlm.nih.gov]
  • 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]
  • Klinge 1 demonstrated that these "rheumatic" granulomas, formerly thought to be specific for rheumatic fever, are neither specific nor constant. In rheumatic fever the predominant rôle of streptococci as allergens is undisputed.[annals.org]
Weakness
  • 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]
  • Muscular weakness leads to inability to maintain a steady grip. Ask the patient to grip your hand or wrist tightly and maintain it. The fluctuating strength of the grip is called 'milkmaid's grip'.[patient.info]
  • 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]
  • 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]
Falling
  • 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 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]
  • 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]
  • 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]
  • 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]
Hunting
  • Infection An infectious cause has been suspected and hunted for decades but has never been convincingly or reproducibly proven.[arthritisresearch.us]
Muscle Weakness
  • 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]
  • 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]
  • Other symptoms of the disorder may include diminished muscle tone, muscle weakness, and emotional and behavioural disturbances, particularly obsessive-compulsive behaviours.[brainfoundation.org.au]
  • It can also be associated with muscle weakness and emotional outbursts. Because of antibiotics, rheumatic fever is now rare in developed countries.[webmd.com]
  • Sometimes other effects appear alongside the movements, such as muscle weakness, poor muscle tone and clumsiness. The movements seem to vary in intensity, and the area they affect may change over time.[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]
  • 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]
Chorea
  • Therapy with prednisone shortened the duration of rheumatic chorea; some reported recurrences of chorea and had minor neurologic sequelae.[ncbi.nlm.nih.gov]
  • Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence.[ncbi.nlm.nih.gov]
  • In view of the significant side effects associated with the drugs currently used to treat chorea, we sought to further evaluate the efficacy of carbamazepine in children with rheumatic chorea.[ncbi.nlm.nih.gov]
  • The authors investigated the role of Vitamin E in reducing rheumatic chorea. A case series of patients of rheumatic chorea were administered Vitamin E in the dose 50 IU daily for fifteen days.[ncbi.nlm.nih.gov]
  • A statistically significant increase (P 0.01) in the prevalence of ANeurA was found for patients with active chorea (acute and chronic) compared with the prevalence in patients with past histories of RhCh (controlled chorea).[ncbi.nlm.nih.gov]
Involuntary Movements
  • 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]
  • 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]
  • 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]
  • R. 12 y.o, came to our observation for involuntary movements of limbs and trunk. She had no pathological findings on brain NMR, EEG and echocardiography. Blood tests showed high ESR and TAS value, throat swab positive for GAS.[ped-rheum.biomedcentral.com]
  • It is a quasi purposive, non repetitive involuntary movement. Sydenham’s chorea is often associated with hypotonia and emotional instability.[cardiophile.org]
Irritability
  • 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]
  • 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]
  • 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
  • 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]
  • Tremor Tremor is a regular osillatory movement of the extremities related to posture or activity.[pediatrics.emory.edu]
  • Thyrotoxicosis: Tremor, tachycardia, lidlag and goitre (may be present) 6. Drugs: Take a complete drug history[epomedicine.com]
Tic Disorder
  • Critique: tic disorder controls with OCD were from reported literature[pandasppn.org]
  • Angelman syndrome (Medical Encyclopedia) Chronic motor tic disorder (Medical Encyclopedia) Facial tics (Medical Encyclopedia) Movement - uncontrollable (Medical Encyclopedia) Movement - uncontrolled or slow (Medical Encyclopedia) Movement - uncoordinated[icdlist.com]
  • Researchers have noted an association between recurrent SC and the later development of the abrupt onset forms of obsessive-compulsive disorder, attention deficit/hyperactivity disorder, tic disorders, and autism, which they call PANDAS, for Pediatric[ninds.nih.gov]
  • PANDAS ( P ediatric A utoimmune N europsychiatric D isorders A ssociated with S treptococcal infections PANDAS, is a term used to describe a subset of children who have Obsessive Compulsive Disorder (OCD) and/or tic disorders such as Tourette's Syndrome[pediatrics.emory.edu]

Workup

Chlamydia
  • Similarly, infection with Mycoplasma, Chlamydia, and Proteus have been considered as causes of RA, but the evidence is not convincing.[arthritisresearch.us]
HLA-DR4
  • Rheumatoid arthritis has been known to be associated with HLA tissue types since the 1970s, with the risk of RA increased fourfold in individuals with the HLA-DR4 tissue type (new nomenclature HLA-DRB1 0401, 0402, and so on, for the different variants[arthritisresearch.us]

Treatment

  • We suggest that carbamazepine may serve as a first-line treatment for rheumatic chorea.[ncbi.nlm.nih.gov]
  • All patients received some medical treatment. Haloperidol was the most prescribed medication (15 patients - 75 %). Sulpiride, diazepam and valproate were also used as symptomatic treatment.[ncbi.nlm.nih.gov]
  • Prednisone treatment may lead to a shortened course of chorea (4.0 weeks in prednisone-treated [n 32] vs 9.0 weeks in untreated [n 14]; P .0001).[ncbi.nlm.nih.gov]
  • Sydenham Chorea Treatment Due to the fact that Sydenham chorea is a self-limited disease, treatment should be administrated to particular cases with severe evolution.[doctortipster.com]

Prognosis

  • Prognosis Commonly, Sydenham's chorea is self-limiting with spontaneous remission. Symptoms generally improve in a week or two and are better by 8-9 months, rarely lasting a year; however, they may occasionally wax and wane for up to 10 years.[patient.info]
  • 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]
  • ., valproic acid ) Prognosis : Usually self-limiting If treated, full remission usually occurs within 3–4 months.[amboss.com]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.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]
  • 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]
  • Aid for Diagnosis of Etiology of Chorea 1. Huntington’s disease: Assess mental status (early dementia) 2. Wilson’s disease: Kayser-Fleischer ring, Stigmata of chronic liver disease, and akinetic-rigid syndrome 3.[epomedicine.com]

Epidemiology

  • 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]
  • Department of Clinical Epidemiology Unit Government Medical College Nagpur[link.springer.com]
  • Epidemiology Typical age of onset is 5-15 years. There is a female preponderance reported in most studies. A predisposition runs in families. The incidence of Sydenham's chorea reflects that of RF. It is rarely seen in developed countries nowadays.[patient.info]
  • References: [2] [3] [4] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
Sex distribution
Age distribution

Pathophysiology

  • Get a quick and memorable overview of anatomy, pathophysiology, and clinical presentation from the precision and beauty of Netter and Netter-style plates that highlight key neuroanatomical and neurologic concepts.[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]
  • ., 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 : Streptococcal[amboss.com]

Prevention

  • Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.[ncbi.nlm.nih.gov]
  • 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]
  • Humoral immunity definitely plays a role in RhCh; thus, routine administration of corticosteroids to patients with acute RhCh is suggested to prevent neuron damage and chronicity.[ncbi.nlm.nih.gov]
  • […] clinicians to approach the diagnostic problems presented by children with ARF and RHD; through proper assessment and integration of the history, physical examination, investigations, especially the modern tool, the echocardiography and learn about the prevention[books.google.com]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]

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