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Disease relevance of Chorea


Psychiatry related information on Chorea


High impact information on Chorea

  • Phenytoin and choreic movements [11].
  • Convalescent sera and sera from other streptococcal diseases in the absence of chorea did not activate the kinase [12].
  • Chorea monoclonal antibodies showed specificity for mammalian lysoganglioside and N-acetyl-beta-D-glucosamine (GlcNAc), the dominant epitope of the group A streptococcal (GAS) carbohydrate [12].
  • These results are relevant to the clinical cases of chorea associated with elevated concentrations of estrogen, which occur in pregnancy and during oral contraceptive use [13].
  • Kainic acid lesions of the striatum dissociate amphetamine and apomorphine stereotypy: similarities to Huntingdon's chorea [14].

Chemical compound and disease context of Chorea


Biological context of Chorea


Anatomical context of Chorea

  • These results are consistent with the hypothesis that chorea results from preferential loss of striatal neurons projecting to the lateral globus pallidus and that rigid-akinetic HD is a consequence of the additional loss of striatal neurons projecting to the medial segment of the pallidum [24].
  • The severe loss of D2-receptor-bearing striatal neuron, with concomitant loss of dopaminergic projections from the nigra to the posterior putamen, is consistent with both chorea and extrapyramidal rigidity being features of patients with neuroacanthocytosis [25].
  • Concentrations of gamma-aminobutyric acid, choline acetyltransferase activity and spiroperidol binding in the basal ganglia did not correlate with the generation of choreic movements [26].
  • The global atrophy of cerebral cortex and white matter observed in all degrees of HD may account for the cognitive and neuropsychiatric impairments which often precede the onset of chorea [27].
  • When HD patients were divided into more (i.e., HD with chorea; n=4) and less impaired (i.e., without chorea; n=6) groups, both showed significant decreases in left anterior cerebral artery flow velocity during visual spatial executive cognition testing compared with control subjects [28].

Gene context of Chorea

  • Increased serum concentrations of monokine induced by interferon-gamma/CXCL9 and interferon-gamma-inducible protein 10/CXCL-10 in Sydenham's chorea patients [29].
  • The frequencies of the HLA-DRB1 and HLA-DQB1 alleles in patients with pure chorea were not significantly different from those observed in controls [30].
  • We evaluated 91 patients with RF for HLA-A, HLA-B, and HLA-DR antigens; of these, 33 had pure chorea, 26 pure carditis, 16 pure arthritis, and 16 carditis plus arthritis [30].
  • Considering that chorea in this patient might be due to the disruption of a gene at either of the 4p15.32 or 4q33 breakpoints, CLCN3 was considered as a candidate gene [31].
  • MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF [32].

Analytical, diagnostic and therapeutic context of Chorea


  1. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Karpuj, M.V., Becher, M.W., Springer, J.E., Chabas, D., Youssef, S., Pedotti, R., Mitchell, D., Steinman, L. Nat. Med. (2002) [Pubmed]
  2. Letter: Asphyxiation, bulimia, and insulin levels in Huntington disease (chorea). Whittier, J.R. JAMA (1976) [Pubmed]
  3. Recent advances in understanding the pathogenesis of Huntington's disease. Reddy, P.H., Williams, M., Tagle, D.A. Trends Neurosci. (1999) [Pubmed]
  4. Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3. Kambouris, M., Bohlega, S., Al-Tahan, A., Meyer, B.F. Am. J. Hum. Genet. (2000) [Pubmed]
  5. The spectrum of levodopa-induced dyskinesias. Fahn, S. Ann. Neurol. (2000) [Pubmed]
  6. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Beal, M.F., Kowall, N.W., Ellison, D.W., Mazurek, M.F., Swartz, K.J., Martin, J.B. Nature (1986) [Pubmed]
  7. Baclofen therapy may be associated with chorea in Alzheimer's disease. Crystal, H.A. Ann. Neurol. (1990) [Pubmed]
  8. Higher sedentary energy expenditure in patients with Huntington's disease. Pratley, R.E., Salbe, A.D., Ravussin, E., Caviness, J.N. Ann. Neurol. (2000) [Pubmed]
  9. The psychiatric symptoms of rheumatic fever. Mercadante, M.T., Busatto, G.F., Lombroso, P.J., Prado, L., Rosário-Campos, M.C., do Valle, R., Marques-Dias, M.J., Kiss, M.H., Leckman, J.F., Miguel, E.C. The American journal of psychiatry. (2000) [Pubmed]
  10. Pemoline induced chorea and Gilles de la Tourette's syndrome. Bonthala, C.M., West, A. The British journal of psychiatry : the journal of mental science. (1983) [Pubmed]
  11. Phenytoin and choreic movements. Nausieda, P.A., Koller, W.C., Klawans, H.L., Weiner, W.J. N. Engl. J. Med. (1978) [Pubmed]
  12. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Kirvan, C.A., Swedo, S.E., Heuser, J.S., Cunningham, M.W. Nat. Med. (2003) [Pubmed]
  13. Increased dopamine receptor sensitivity after estrogen treatment using the rat rotation model. Hruska, R.E., Silbergeld, E.K. Science (1980) [Pubmed]
  14. Kainic acid lesions of the striatum dissociate amphetamine and apomorphine stereotypy: similarities to Huntingdon's chorea. Mason, S.T., Sanberg, P.R., Fibiger, H.C. Science (1978) [Pubmed]
  15. Letter: Amantadine in chorea. Gray, M.W., Herzberg, L., Lerman, J.A., Turnbull, M.J., Victoratos, G. Lancet (1975) [Pubmed]
  16. Reversible chorea due to ranitidine and cimetidine. Lehmann, A.B. Lancet (1988) [Pubmed]
  17. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Wheeler, V.C., Gutekunst, C.A., Vrbanac, V., Lebel, L.A., Schilling, G., Hersch, S., Friedlander, R.M., Gusella, J.F., Vonsattel, J.P., Borchelt, D.R., MacDonald, M.E. Hum. Mol. Genet. (2002) [Pubmed]
  18. Benefits of monitoring plasma levodopa in Parkinson's disease patients with drug-induced chorea. Sage, J.I., Mark, M.H., McHale, D.M., Sonsalla, P.K., Vitagliano, D. Ann. Neurol. (1991) [Pubmed]
  19. A 10-year experience with postpump chorea. Medlock, M.D., Cruse, R.S., Winek, S.J., Geiss, D.M., Horndasch, R.L., Schultz, D.L., Aldag, J.C. Ann. Neurol. (1993) [Pubmed]
  20. Dystonia-predominant adult-onset Huntington disease: association between motor phenotype and age of onset in adults. Louis, E.D., Anderson, K.E., Moskowitz, C., Thorne, D.Z., Marder, K. Arch. Neurol. (2000) [Pubmed]
  21. Movement disorders and mitochondrial dysfunction. Hanna, M.G., Bhatia, K.P. Curr. Opin. Neurol. (1997) [Pubmed]
  22. The pathogenesis of Machado Joseph Disease: a high manganese/low magnesium initiated CAG expansion mutation in susceptible genotypes? Purdey, M. Journal of the American College of Nutrition. (2004) [Pubmed]
  23. Persistent chorea following cardiac surgery for congenital heart disease. Osari, S., Muranaka, H., Kojima, T., Kimura, Y. Acta paediatrica Japonica; Overseas edition. (1995) [Pubmed]
  24. Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia. Albin, R.L., Reiner, A., Anderson, K.D., Penney, J.B., Young, A.B. Ann. Neurol. (1990) [Pubmed]
  25. Presynaptic and postsynaptic striatal dopaminergic function in neuroacanthocytosis: a positron emission tomographic study. Brooks, D.J., Ibanez, V., Playford, E.D., Sawle, G.V., Leigh, P.N., Kocen, R.S., Harding, A.E., Marsden, C.D. Ann. Neurol. (1991) [Pubmed]
  26. Choreic movements in the macaque monkey induced by kainic acid lesions of the striatum combined with L-dopa. Pharmacological, biochemical and physiological studies on neural mechanisms. Kanazawa, I., Kimura, M., Murata, M., Tanaka, Y., Cho, F. Brain (1990) [Pubmed]
  27. Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease. de la Monte, S.M., Vonsattel, J.P., Richardson, E.P. J. Neuropathol. Exp. Neurol. (1988) [Pubmed]
  28. Cerebral blood flow velocity decreases during cognitive stimulation in Huntington's disease. Deckel, A.W., Cohen, D., Duckrow, R. Neurology (1998) [Pubmed]
  29. Increased serum concentrations of monokine induced by interferon-gamma/CXCL9 and interferon-gamma-inducible protein 10/CXCL-10 in Sydenham's chorea patients. Teixeira, A.L., Cardoso, F., Souza, A.L., Teixeira, M.M. J. Neuroimmunol. (2004) [Pubmed]
  30. HLA class I and class II profiles of patients presenting with Sydenham's chorea. Donadi, E.A., Smith, A.G., Louzada-Júnior, P., Voltarelli, J.C., Nepom, G.T. J. Neurol. (2000) [Pubmed]
  31. Refined localisation of the voltage-gated chloride channel, CLCN3, to 4q33. Taine, L., Coupry, I., Boisseau, P., Saura, R., Lacombe, D., Arveiler, B. Hum. Genet. (1998) [Pubmed]
  32. Neurological mitochondrial cytopathies. Mehndiratta, M.M., Agarwal, P., Tatke, M., Krishnamurthy, M. Neurology India. (2002) [Pubmed]
  33. Serial brain SPECT images in a case of Sydenham chorea. Lee, P.H., Nam, H.S., Lee, K.Y., Lee, B.I., Lee, J.D. Arch. Neurol. (1999) [Pubmed]
  34. Steroid-responsive chorea in childhood following cardiac transplantation. Blunt, S.B., Brooks, D.J., Kennard, C. Mov. Disord. (1994) [Pubmed]
  35. Common neural mechanisms in experimental chorea and hemiballismus in the monkey. Evidence from 2-deoxyglucose autoradiography. Mitchell, I.J., Jackson, A., Sambrook, M.A., Crossman, A.R. Brain Res. (1985) [Pubmed]
  36. Deep brain stimulation of the internal pallidum did not improve chorea in a patient with neuro-acanthocytosis. Wihl, G., Volkmann, J., Allert, N., Lehrke, R., Sturm, V., Freund, H.J. Mov. Disord. (2001) [Pubmed]
  37. Randomized Double-Blind Study With Prednisone in Sydenham's Chorea. Paz, J.A., Silva, C.A., Marques-Dias, M.J. Pediatric neurology. (2006) [Pubmed]
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