chorea

Summary

Summary: Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as CHOREATIC DISORDERS. Chorea is also a frequent manifestation of BASAL GANGLIA DISEASES.

Top Publications

  1. Ferrara A, De Michele G, Salvatore E, Di Maio L, Zampella E, Capuano S, et al. A novel NKX2.1 mutation in a family with hypothyroidism and benign hereditary chorea. Thyroid. 2008;18:1005-9 pubmed publisher
    We studied a boy with congenital hypothyroidism, benign hereditary chorea, and respiratory distress. His mother and his grandfather were affected by hypothyroidism with a late onset and benign hereditary chorea...
  2. Baizabal Carvallo J, Alonso Juarez M, Koslowski M. Chorea in systemic lupus erythematosus. J Clin Rheumatol. 2011;17:69-72 pubmed publisher
    b>Chorea is recognized as one of the neurologic manifestations of systemic lupus erythematosus (SLE). Most reports show an association between chorea and antiphospholipid (aPL) antibodies in SLE patients.
  3. Cardoso F. Sydenham's chorea. Handb Clin Neurol. 2011;100:221-9 pubmed publisher
    Sydenham's chorea (SC) is the neurologic expression of acute rheumatic fever (ARF). Despite the declining incidence of ARF worldwide, it remains the most common cause of acute chorea in children...
  4. Reiner P, Galanaud D, Leroux G, Vidailhet M, Haroche J, Huong D, et al. Long-term outcome of 32 patients with chorea and systemic lupus erythematosus or antiphospholipid antibodies. Mov Disord. 2011;26:2422-7 pubmed publisher
    The aim of this work was to describe chorea during systemic lupus erythematosus or antiphospholipid antibodies and its long-term outcome.
  5. Kirvan C, Cox C, Swedo S, Cunningham M. Tubulin is a neuronal target of autoantibodies in Sydenham's chorea. J Immunol. 2007;178:7412-21 pubmed
    Sydenham's chorea is a CNS disorder and sequela of group A streptococcal infection where deposition of Abs in brain may result in movement and neuropsychiatric abnormalities. We studied human mAbs 24.3.1, 31.1.1, and 37.2...
  6. Armstrong M, Miyasaki J. Evidence-based guideline: pharmacologic treatment of chorea in Huntington disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology. 2012;79:597-603 pubmed publisher
    To develop an evidence-based guideline assessing pharmacologic options for treating Huntington disease (HD) chorea.
  7. Li J, Zhu X, Wang X, Sun W, Feng B, Du T, et al. Targeted genomic sequencing identifies PRRT2 mutations as a cause of paroxysmal kinesigenic choreoathetosis. J Med Genet. 2012;49:76-8 pubmed publisher
    ..A PKC locus has been previously mapped to the pericentromeric region of chromosome 16 (16p11.2-q12.1), but the causative gene remains unidentified...
  8. Wang X, Sun W, Zhu X, Li L, Du T, Mao W, et al. Paroxysmal kinesigenic choreoathetosis: evidence of linkage to the pericentromeric region of chromosome 16 in four Chinese families. Eur J Neurol. 2010;17:800-7 pubmed publisher
    ..The pericentromeric region of chromosome 16 has been linked to PKC by several reports. This study was to localize and identify PKC gene in four Chinese PKC families...
  9. de Teixeira A, Cardoso F, Maia D, Sacramento D, Mota C, Meira Z, et al. Frequency and significance of vocalizations in Sydenham's chorea. Parkinsonism Relat Disord. 2009;15:62-3 pubmed publisher
    Sydenham's chorea (SC) is a complication of Streptococcus infection characterized by a combination of motor and non-motor features...

Scientific Experts

More Information

Publications62

  1. Wang J, Cao L, Li X, Hu Z, Li J, Zhang J, et al. Identification of PRRT2 as the causative gene of paroxysmal kinesigenic dyskinesias. Brain. 2011;134:3493-3501 pubmed publisher
    ..Thus, we have identified PRRT2 as the first causative gene of paroxysmal kinesigenic dyskinesias, warranting further investigations to understand the pathogenesis of this disorder...
  2. Asmus F, Devlin A, Munz M, Zimprich A, Gasser T, Chinnery P. Clinical differentiation of genetically proven benign hereditary chorea and myoclonus-dystonia. Mov Disord. 2007;22:2104-9 pubmed
    Because of clinical similarities, benign hereditary chorea and myoclonus-dystonia (DYT11) might be confused...
  3. Lazurova I, Macejova Z, Benhatchi K, Oetterova M, Antolová E, Asherson R, et al. Efficacy of intravenous immunoglobulin treatment in lupus erythematosus chorea. Clin Rheumatol. 2007;26:2145-2147 pubmed publisher
    b>Chorea is a rare complication of systemic lupus erythematosus (SLE) and is strongly related to the presence of antiphospholipid antibodies. Various infections may also be triggering factors in the development of choreiform movements...
  4. Ben Pazi H, Stoner J, Cunningham M. Dopamine receptor autoantibodies correlate with symptoms in Sydenham's chorea. PLoS ONE. 2013;8:e73516 pubmed publisher
    Sydenham chorea (SC), a neuropsychiatric sequela of group-A streptococcal infection, is associated with basal ganglia autoantibodies...
  5. Ford J, Albertson T, Owen K, Sutter M, McKinney W. Acute, sustained chorea in children after supratherapeutic dosing of amphetamine-derived medications. Pediatr Neurol. 2012;47:216-8 pubmed publisher
    ..We describe two children who developed chorea after ingesting amphetamine-derived medications prescribed to treat attention deficit hyperactivity disorder...
  6. Bonelli R, Hofmann P. A systematic review of the treatment studies in Huntington's disease since 1990. Expert Opin Pharmacother. 2007;8:141-53 pubmed
    ..The authors conducted a systematic review of pharmacological therapy in HD using the available papers that were published between 1990 and 2006...
  7. Demiroren K, Yavuz H, Cam L, Oran B, Karaaslan S, Demiroren S. Sydenham's chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22:550-4 pubmed
    Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood...
  8. Bruno M, Lee H, Auburger G, Friedman A, Nielsen J, Lang A, et al. Genotype-phenotype correlation of paroxysmal nonkinesigenic dyskinesia. Neurology. 2007;68:1782-9 pubmed
    ..Paroxysmal nonkinesigenic dyskinesia (PNKD) is a rare disorder characterized by episodic hyperkinetic movement attacks. We have recently identified mutations in the MR-1 gene causing familial PNKD...
  9. Walker A, Tani L, Thompson J, Firth S, Veasy L, Bale J. Rheumatic chorea: relationship to systemic manifestations and response to corticosteroids. J Pediatr. 2007;151:679-83 pubmed
    To describe Sydenham chorea among children in a cohort of patients with rheumatic fever (RF).
  10. Kiryluk K, Khan F, Valeri A. Acute chorea and bilateral basal ganglia lesions in a hemodialysis patient. Kidney Int. 2008;73:1087-91 pubmed publisher
  11. Chang C, Felicio A, Godeiro C, Matsubara L, Duarte D, Ferraz H, et al. Chorea-ballism as a manifestation of decompensated type 2 diabetes mellitus. Am J Med Sci. 2007;333:175-7 pubmed
    b>Chorea and ballism are movement disorders that result from a variety of conditions. Hyperglycemia is an unusual recognized cause of these movement disorders...
  12. Du T, Feng B, Wang X, Mao W, Zhu X, Li L, et al. Localization and mutation detection for paroxysmal kinesigenic choreoathetosis. J Mol Neurosci. 2008;34:101-7 pubmed
    ..Paroxysmal kinesigenic choreoathetosis (PKC) is an autosomal-dominant movement disorder characterized by attacks of paroxysmal involuntary movements. To date, the causative gene has not been discovered...
  13. Walker R, Jung H, Tison F, Lee S, Danek A. Phenotypic variation among brothers with the McLeod neuroacanthocytosis syndrome. Mov Disord. 2007;22:244-8 pubmed
    ..This phenotypic variation, despite shared mutations, suggests the action of disease-modifying factors that may explain some of the difficulties with genotype-phenotype correlation in McLeod syndrome...
  14. Mahajnah M, Inbar D, Steinmetz A, Heutink P, Breedveld G, Straussberg R. Benign hereditary chorea: clinical, neuroimaging, and genetic findings. J Child Neurol. 2007;22:1231-4 pubmed
    Benign hereditary chorea is an autosomal dominant disease with an early onset of symptoms. In some families, symptoms tend to decrease in adulthood, suggesting that the disorder results from a developmental disturbance in the brain...
  15. Siniscalchi A, Gallelli L, Davoli A, De Sarro G. Efficacy and tolerability of topiramate in vascular generalized chorea. Ann Pharmacother. 2007;41:1915 pubmed
  16. Orzechowski N, Wolanskyj A, Ahlskog J, Kumar N, Moder K. Antiphospholipid antibody-associated chorea. J Rheumatol. 2008;35:2165-70 pubmed
    To describe the clinical features, treatment, and outcomes of patients with antiphospholipid antibody (aPL)-associated chorea.
  17. Rochette J, Roll P, Fu Y, Lemoing A, Royer B, Roubertie A, et al. Novel familial cases of ICCA (infantile convulsions with paroxysmal choreoathetosis) syndrome. Epileptic Disord. 2010;12:199-204 pubmed publisher
    ..The identification of novel ICCA families should contribute to better knowledge regarding the clinical manifestations of ICCA syndrome as well as the search for the underlying genetic defect(s)...
  18. Reiner P, Piette J, Leroux G, Vidailhet M, Costedoat Chalumeau N. [Chorea, lupus and antiphospholipid antibodies]. Rev Med Interne. 2012;33:206-8 pubmed publisher
    b>Chorea may occur in patients with SLE with a frequency estimated at 1 to 3% in adults and up to 9% in paediatric lupus. Chorea is frequently a presenting feature, and is strongly related to the presence of antiphospholipid antibodies...
  19. Zacharia T. Giant tumefactive perivascular spaces manifesting as chorea bilaterally. J Neuroimaging. 2011;21:205-7 pubmed publisher
    ..We report the first MR imaging description of a case of giant tumefactive (PVSs) manifesting as chorea bilaterally.
  20. Beato R, Maia D, Teixeira A, Cardoso F. Executive functioning in adult patients with Sydenham's chorea. Mov Disord. 2010;25:853-7 pubmed publisher
    Sydenham's chorea (SC) is characterized by a combination of motor and behavioral findings. Cognitive function has been scarcely studied in this condition...
  21. Maccabelli G, Pichiecchio A, Guala A, Ponzio M, Palesi F, Maranzana Rt D, et al. Advanced magnetic resonance imaging in benign hereditary chorea: study of two familial cases. Mov Disord. 2010;25:2670-4 pubmed publisher
    No brain abnormalities are usually detected on conventional magnetic resonance imaging (MRI) in benign hereditary chorea (BHC); there are currently no studies with advanced techniques in literature...
  22. Tunks R, Rojas M, Edwards K, Liske M. Do rates of arthritis and chorea predict the incidence of acute rheumatic fever?. Pediatr Int. 2011;53:742-746 pubmed publisher
    ..We hypothesize that focused attention on non-cardiac features of ARF, namely joint manifestations and chorea, might enhance its detection in settings with limited resources.
  23. Gras D, Jonard L, Roze E, Chantot Bastaraud S, Koht J, Motte J, et al. Benign hereditary chorea: phenotype, prognosis, therapeutic outcome and long term follow-up in a large series with new mutations in the TITF1/NKX2-1 gene. J Neurol Neurosurg Psychiatry. 2012;83:956-62 pubmed publisher
    Benign hereditary chorea (BHC) is a rare autosomal dominant disorder characterised by childhood onset that tends to improve in adulthood...
  24. Jankovic J, Clarence Smith K. Tetrabenazine for the treatment of chorea and other hyperkinetic movement disorders. Expert Rev Neurother. 2011;11:1509-23 pubmed publisher
  25. Mink J. Paroxysmal dyskinesias. Curr Opin Pediatr. 2007;19:652-6 pubmed
    ..Substantial progress has been made recently in understanding characteristic features of the paroxysmal dyskinesias and underlying genetic causes. This review summarizes the most important findings and discusses their implications...
  26. Gordon N. Sydenham's chorea, and its complications affecting the nervous system. Brain Dev. 2009;31:11-4 pubmed publisher
    The well-known symptoms of rheumatic fever and Sydenham's chorea are briefly discussed...
  27. Nozaki I, Inao G, Yamada M. Donepezil-induced chorea in Alzheimer's disease. J Neurol. 2007;254:1752-3 pubmed
  28. Bhatia K. Paroxysmal dyskinesias. Mov Disord. 2011;26:1157-65 pubmed publisher
    ..Potassium and calcium channel mutations underlie the 2 main forms of episodic ataxia (EA1 and EA2), whereas benign torticollis of infancy may also be a calcium channel disorder...
  29. Provenzano C, Veneziano L, Appleton R, Frontali M, Civitareale D. Functional characterization of a novel mutation in TITF-1 in a patient with benign hereditary chorea. J Neurol Sci. 2008;264:56-62 pubmed
    Benign hereditary chorea (BHC) is an autosomal dominant disorder of early onset characterised by non progressive choreic movements with normal cognitive function occasionally associated with hypothyroidism and respiratory problems...
  30. Walker R. Differential diagnosis of chorea. Curr Neurol Neurosci Rep. 2011;11:385-95 pubmed publisher
    b>Chorea is a common movement disorder that can be caused by a large variety of structural, neurochemical (including pharmacologic), or metabolic disturbances to basal ganglia function, indicating the vulnerability of this brain region...
  31. Doyle F, Cardoso F, Lopes L, Mendes M, Dias F, Cruz L, et al. Infusion of Sydenham's chorea antibodies in striatum with up-regulated dopaminergic receptors: a pilot study to investigate the potential of SC antibodies to increase dopaminergic activity. Neurosci Lett. 2012;523:186-9 pubmed publisher
    Sydenham's chorea (SC) is a neurological manifestation of rheumatic fever...
  32. Suls A, Dedeken P, Goffin K, Van Esch H, Dupont P, Cassiman D, et al. Paroxysmal exercise-induced dyskinesia and epilepsy is due to mutations in SLC2A1, encoding the glucose transporter GLUT1. Brain. 2008;131:1831-44 pubmed publisher
  33. Chambers R, Gooden M, Gilbert T, Jackson S. Childhood chorea-encephalopathy associated with recent parvovirus B19 infection in two Jamaican children. Ann Trop Paediatr. 2010;30:339-44 pubmed publisher
    ..The cases are compared with a previously described case of childhood chorea encephalopathy associated with human parvovirus B19 infection.
  34. Rochette J, Roll P, Szepetowski P. Genetics of infantile seizures with paroxysmal dyskinesia: the infantile convulsions and choreoathetosis (ICCA) and ICCA-related syndromes. J Med Genet. 2008;45:773-9 pubmed publisher
    ..The aim of this review is to update genetic aspects of infantile epileptic seizures and PD and their association in the context of ICCA and ICCA related syndromes...
  35. Weiner S, Normandin P. Sydenham chorea: a case report and review of the literature. Pediatr Emerg Care. 2007;23:20-4 pubmed
    Sydenham chorea (SC) is the neurological manifestation of rheumatic fever and is the most common acquired chorea in children. The disease presents as choreiform movements and behavioral changes after a streptoccocal throat infection...
  36. Oosterveer D, Overweg Plandsoen W, Roos R. Sydenham's chorea: a practical overview of the current literature. Pediatr Neurol. 2010;43:1-6 pubmed publisher
    Sydenham's chorea is characterized by uncoordinated movements, emotional instability, and hypotonia. It can occur up to several months after group A beta-hemolytic Streptococcus infection...
  37. Wu S, Graham B, Gelfand M, Gruppo R, Dinopolous A, Gilbert D. Clinical and positron emission tomography findings of chorea associated with primary antiphospholipid antibody syndrome. Mov Disord. 2007;22:1813-5 pubmed
    ..His symptoms resolved on prednisone; however, his antiphospholipid antibody profile remained markedly abnormal despite being symptom-free for 26 months...
  38. Bronfeld M, Belelovsky K, Erez Y, Bugaysen J, Korngreen A, Bar Gad I. Bicuculline-induced chorea manifests in focal rather than globalized abnormalities in the activation of the external and internal globus pallidus. J Neurophysiol. 2010;104:3261-75 pubmed publisher
    b>Chorea is a basal-ganglia (BG) related hyperkinetic movement disorder characterized by irregular continuous involuntary movements...
  39. Kikuchi T, Nomura M, Tomita H, Harada N, Kanai K, Konishi T, et al. Paroxysmal kinesigenic choreoathetosis (PKC): confirmation of linkage to 16p11-q21, but unsuccessful detection of mutations among 157 genes at the PKC-critical region in seven PKC families. J Hum Genet. 2007;52:334-41 pubmed
    ..Thus, one of the two genes, SCNN1G and ITGAL, could be causative for PKC, but we were not able to find any other mutations that explain the PKC phenotype...
  40. Yoshida Y, Nunomura J, Shimohata T, Nanjo H, Miyata H. Benign hereditary chorea 2: pathological findings in an autopsy case. Neuropathology. 2012;32:557-65 pubmed publisher
    Two Japanese families with benign hereditary chorea (BHC) 2 have recently been reported. BHC 2 is characterized by adult-onset non-progressive chorea, and by genetic abnormality in the locus of chromosome 8q21.3-q23.3...
  41. Chen W, Lin Y, Xiong Z, Wei W, Ni W, Tan G, et al. Exome sequencing identifies truncating mutations in PRRT2 that cause paroxysmal kinesigenic dyskinesia. Nat Genet. 2011;43:1252-5 pubmed publisher
    ..The function of PRRT2 and its role in paroxysmal kinesigenic dyskinesia should be further investigated...
  42. Marschitz I, Rodl S, Gruber Sedlmayr U, Church A, Giovannoni G, Zobel G, et al. Severe chorea with positive anti-basal ganglia antibodies after herpesencephalitis. J Neurol Neurosurg Psychiatry. 2007;78:105-7 pubmed
  43. Edwards T, Zrinzo L, Limousin P, Foltynie T. Deep brain stimulation in the treatment of chorea. Mov Disord. 2012;27:357-63 pubmed publisher
    ..Recently, owing to the clinical similarities between L-dopa induced dyskinesia and chorea, deep brain stimulation has now been implemented as a novel treatment method in both Huntington's disease and ..
  44. Frank S, Ondo W, Fahn S, Hunter C, Oakes D, Plumb S, et al. A study of chorea after tetrabenazine withdrawal in patients with Huntington disease. Clin Neuropharmacol. 2008;31:127-33 pubmed publisher
    To assess tetrabenazine (TBZ) efficacy by evaluating the change in Huntington disease-associated chorea resulting from TBZ treatment withdrawal.
  45. Brilot F, Merheb V, Ding A, Murphy T, Dale R. Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome. Neurology. 2011;76:1508-13 pubmed publisher
    To test the hypothesis that Sydenham chorea (SC) immunoglobulin G (IgG) autoantibodies bind to specific neuronal surface proteins, whereas IgG from patients with pediatric autoimmune neuropsychiatric disorders associated with ..
  46. Roos R. Huntington's disease: a clinical review. Orphanet J Rare Dis. 2010;5:40 pubmed publisher
    ..The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded...
  47. Roll P, Sanlaville D, Cillario J, Labalme A, Bruneau N, Massacrier A, et al. Infantile convulsions with paroxysmal dyskinesia (ICCA syndrome) and copy number variation at human chromosome 16p11. PLoS ONE. 2010;5:e13750 pubmed publisher
    ..The possibility that the ICCA syndrome is related to the existence of large-scale genomic alterations was addressed in the present study...
  48. Bayreuther C, Borg M, Ferrero Vacher C, Chaussenot A, Lebrun C. [Chorea-acanthocytosis without acanthocytes]. Rev Neurol (Paris). 2010;166:100-3 pubmed publisher
    b>Chorea-acanthocytosis (ChAc) is one of the neuroacanthocytosis syndromes which form a group of disorders characterized by the association of neurological abnormalities and spiculated red blood cells called acanthocytes...
  49. Kovacs G, Murrell J, Horvath S, Haraszti L, Majtenyi K, Molnar M, et al. TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea. Mov Disord. 2009;24:1843-7 pubmed publisher
    ..in a carrier of the novel K263E TARDBP variation, who developed frontotemporal dementia, supranuclear palsy, and chorea, but no signs of motor neuron disease...
  50. Wild E, Tabrizi S. The differential diagnosis of chorea. Pract Neurol. 2007;7:360-73 pubmed
    b>Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt...
  51. Appenzeller S, Yeh S, Maruyama M, Barros S, de Carvalho J. Chorea in primary antiphospholipid syndrome is associated with rheumatic fever. Rheumatol Int. 2012;32:2857-61 pubmed publisher
    The aim of the study is to evaluate the frequency of chorea in a cohort of primary antiphospholipid syndrome (PAPS) patients and their possible clinical and laboratory associations...
  52. Weber Y, Storch A, Wuttke T, Brockmann K, Kempfle J, Maljevic S, et al. GLUT1 mutations are a cause of paroxysmal exertion-induced dyskinesias and induce hemolytic anemia by a cation leak. J Clin Invest. 2008;118:2157-68 pubmed publisher
  53. Heron S, Grinton B, Kivity S, Afawi Z, Zuberi S, Hughes J, et al. PRRT2 mutations cause benign familial infantile epilepsy and infantile convulsions with choreoathetosis syndrome. Am J Hum Genet. 2012;90:152-60 pubmed publisher
    ..Furthermore, PRRT2 mutations elicit pleiotropy in terms of both age of expression (infancy versus later childhood) and anatomical substrate (cortex versus basal ganglia)...