Chorea: clinical correlates of 119 cases

Mendes, Maria Fernanda; Andrade, Luiz Augusto Franco De; Ferraz, Henrique Ballalai

doi:10.1590/S0004-282X1996000300010

Abstracts

Chorea is a clinical syndrome characterized by abnormal involuntary arrhythmic movements, randomly distributed in time, affecting mainly the distal parts of the limbs. There are many diseases associated with chorea but the distribution of the etiologies vary too much in different parts of the world. We intended to study the etiologies of chorea in a Movement Disorders Unit of a university hospital-based outpatient clinic in Brazil. We studied the records of 119 patients with chorea based in the diagnostic criteria of the World Federation of Neurology. Sydenham's chorea (SC) was the most frequent cause of chorea (51.3%) of our sample. Other common causes were Huntington's chorea (18.5%) and chorea post-stroke (9.2%). SC is not commonly seen in developed countries nowadays but is not rare in Brazil. SC patients generally have the clinical manifestation of it in the first 20 years of age and girls are more affected than boys and this feature was observed in our sample. Based on our own experience and in the review of the literature we propose an etiological classification of chorea.

chorea; Sydenham's chorea; Huntington's disease; movement disorders

Coréia é uma síndrome caracterizada por movimentos involuntários arrítmicos, rápidos, abruptos, não repetitivos no tempo e com distribuição variável, preferentemente distal. O número de entidades clínicas reconhecidamente associadas a movimentos coréicos tem se tornado cada vez maior com o passar do tempo. Propusemo-nos estudar a frequência e algumas características epidemiológicas das coréias atendidas em um ambulatório especializado em distúrbios do movimento. Foram estudados os prontuários de 119 pacientes com o diagnóstico sindrômico de coréia. O predomínio absoluto foi de coréia de Sydenham (CS) com 51,3% do total da amostra. Outras causas frequentes foram doença de Huntington (DH) presente em 18,5% e a coréia secundária a doença cerebrovascular em 9,2% dos pacientes. O sexo feminino predominou em todas as faixas etárias, mas principalmente nos pacientes mais jovens, em função da CS predominar e desta ser mais comum nas meninas. Também, a coréia secundária ao uso de anovulatórios e a gravidez contribuiu para este predomínio. Baseados na experiência do nosso serviço e na revisão da literatura, propomos uma classificação para as coréias.

coréia; coréia de Sydenham; doença de Huntington; distúrbios do movimento

Coréia: análise clínica de 119 casos

Chorea: clinical correlates of 119 cases

Maria Fernanda Mendes^I; Luiz Augusto Franco De Andrade^II; Henrique Ballalai Ferraz^III

^ISetor de Investigação em Moléstias Extrapiramidais da Disciplina de Neurologia da Escola Paulista de Medicina (EPM), Universidade Federal de São Paulo (UNIFESP). Mestre em Neurologia pela EPM

^IISetor de Investigação em Moléstias Extrapiramidais da Disciplina de Neurologia da Escola Paulista de Medicina (EPM), Universidade Federal de São Paulo (UNIFESP). Professor Livre-Docente e Chefe da Disciplina de Neurologia

^IIISetor de Investigação em Moléstias Extrapiramidais da Disciplina de Neurologia da Escola Paulista de Medicina (EPM), Universidade Federal de São Paulo (UNIFESP). Médico Assistente da Disciplina de Neurologia, Doutor em Neurologia

RESUMO

Coréia é uma síndrome caracterizada por movimentos involuntários arrítmicos, rápidos, abruptos, não repetitivos no tempo e com distribuição variável, preferentemente distal. O número de entidades clínicas reconhecidamente associadas a movimentos coréicos tem se tornado cada vez maior com o passar do tempo. Propusemo-nos estudar a frequência e algumas características epidemiológicas das coréias atendidas em um ambulatório especializado em distúrbios do movimento. Foram estudados os prontuários de 119 pacientes com o diagnóstico sindrômico de coréia. O predomínio absoluto foi de coréia de Sydenham (CS) com 51,3% do total da amostra. Outras causas frequentes foram doença de Huntington (DH) presente em 18,5% e a coréia secundária a doença cerebrovascular em 9,2% dos pacientes. O sexo feminino predominou em todas as faixas etárias, mas principalmente nos pacientes mais jovens, em função da CS predominar e desta ser mais comum nas meninas. Também, a coréia secundária ao uso de anovulatórios e a gravidez contribuiu para este predomínio. Baseados na experiência do nosso serviço e na revisão da literatura, propomos uma classificação para as coréias.

Palavras-chave: coréia, coréia de Sydenham, doença de Huntington, distúrbios do movimento.

ABSTRACT

Chorea is a clinical syndrome characterized by abnormal involuntary arrhythmic movements, randomly distributed in time, affecting mainly the distal parts of the limbs. There are many diseases associated with chorea but the distribution of the etiologies vary too much in different parts of the world. We intended to study the etiologies of chorea in a Movement Disorders Unit of a university hospital-based outpatient clinic in Brazil. We studied the records of 119 patients with chorea based in the diagnostic criteria of the World Federation of Neurology. Sydenham's chorea (SC) was the most frequent cause of chorea (51.3%) of our sample. Other common causes were Huntington's chorea (18.5%) and chorea post-stroke (9.2%). SC is not commonly seen in developed countries nowadays but is not rare in Brazil. SC patients generally have the clinical manifestation of it in the first 20 years of age and girls are more affected than boys and this feature was observed in our sample. Based on our own experience and in the review of the literature we propose an etiological classification of chorea.

Key words:chorea, Sydenham's chorea, Huntington's disease, movement disorders.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 25-março-1996.

Dr. Henrique Ballalai Ferraz - Disciplina de Neurologia, Escola Paulista de Medicina, UNIFESP - Rua Botucatu 740 - 04023-900 São Paulo SP - Brasil.

1. Adams RD, Victor M. Principles of neurology: Abnormalities of movement disorders and posture due to diseases of the extrapiramidal motor systems. Ed5. New York: McGraw Hill, 1993:56-73.
2. Bird ED. Huntington chorea: etiology and pathogenesis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:255-266.
3. Bird ED, Iversen LL. Huntington's chorea: post-mortem measurement of glutamic-acid-decarboxylase, choline, acetyltransferase and dopamine in basal ganglia. Brain 1974;97:457-472.
4. Bird ED, Ackay AP, Rayner CN, Iversen LL. Reduced glutamic-acid-decarboxilase of post-mortem brain in Huntington's chorea. Lancet 1973;1:1090-1092.
5. Bonilla E, Prasad AL, Arrieta A. Huntington's disease: studies on brain free aminoacids. Life Sci 1988;42:1153-1158.
6. Bruyn GW. Chorea-acanthocytosis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:327-334.
7. Bruyn GW, Went LN. Huntington's chorea. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:267-313.
8. Crossman AR, Mitchell IJ, Sambrook MA, Jackson A. Chorea and myoclonus in the monkey induced by gumma-aminobutyric acid antagonism in the lentiform complex. Brain 1988; 111:1211 -1233.
9. Ellison DW, Real MF, Mazurek MF, Malloy JR, Bird ED, Martin JB. Aminoacid neurotransmitter abnormalities in Huntington's disease and the quinolinic acid animal model of Huntington's disease. Brain 1987; 110:1657-1673.
10. Greenhouse AH. On chorea, lupus erythematous and cerebral vasculitis. Arch Intern Med 1966; 117:389-393.
11. Hoefer PF, Putnam TJ. Action potentials of muscles in athetosis and Sydenham's chorea. Arch Neurol Psychiatry 1940;44:517-531.
12. Huntington G. On chorea. Medical and Surgical Reporter 1872;26:317-321.
13. Kanazawa I. Clinical pathology of basal ganglia disease. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:65-86.
14. Kanazawa I, Sasaki H, Muramoto O, Matsushita M, Mizutani T, Iwabushi K, Ikeda T, Takahata N. Studies on neurotransmitter markers and striatal neuronal cell density in Huntington's disease and dentatorubropallidoluysian atrophy. J Neurol Sci. 1985;70:151-165.
15. Kish SJ, Shannah K, Hornykiewcz O. Elevated serotonin and reduced dopamine in subregionally divided Huntington's disease striatum. Ann Neurol 1987;22:386-389.
16. Klawans HL. Chorea. Can J Neurol Sci 1987;14:536-540.
17. Klawans HL, Goetz CG, Perlik S. Presymptomatic and early detection in Huntington's disease. Ann Neurol 1980,8:343-347.
18. Lakke PF. Classification of extrapyramidal disorders. J Neurol Sci 1981 ;51:311-327.
19. Lyon IW. Chronic hereditary chorea. American Medical Times 1893;7:289-290.
20. Martin JB. Huntington's disease: new approachs to an old problem. Neurology 1984,34:1059-1072.
21. Mount LA, Reback S. Familial paroxysmal choreoathetosis: preliminary report on a hitherto undescribed clinical syndrome. Arch Neurol 1940;44:841 -846.
22. Nausieda PA. Sydenham's chorea, chorea gravidarum and contraceptive induced chorea. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:359-367.
²³
Organización Panamericana de Salud. Prevención y control de la fiebre reumatica en la comunidad. OPAS: 1980, Publicación cientifica 399.
24. Padberg GW, Bruyn GW. Chorea: differential diagnosis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:549-564.
25. Perry TL, Hansen S, Kloster M. Huntington's chorea: deficiency of gumma-aminobutyric acid in brain. N Engl J Med 1973;288:337-342.
26. Shoulson I. On chorea. Clin Neuropharmacol 1986;9:585-599.
27. Spokes EG. Neurochemical alteration in Huntington's chorea: a study of post-mortem brain tissue. Brain 1980;103:179-210.
28. Sydenham T. The whole works of that excellent practical physician, Dr. Thomas Sydenham. London: R Wellington, 1712.
29. Thiebaut F, Isch F. Étude sémiologique du movement choréique. Rev Prat (Paris) 1957;8:127-130.
30. Wilson SAK. Disorders of motility and of muscle tone, with special reference to the corpus striatum. Lancet 1925;2:169-178.

Publication Dates

Publication in this collection
10 Nov 2010
Date of issue
Sept 1996

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Adams RD, Victor M. Principles of neurology: Abnormalities of movement disorders and posture due to diseases of the extrapiramidal motor systems. Ed5. New York: McGraw Hill, 1993:56-73.

[2] 2. Bird ED. Huntington chorea: etiology and pathogenesis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:255-266.

[3] 3. Bird ED, Iversen LL. Huntington's chorea: post-mortem measurement of glutamic-acid-decarboxylase, choline, acetyltransferase and dopamine in basal ganglia. Brain 1974;97:457-472.

[4] 4. Bird ED, Ackay AP, Rayner CN, Iversen LL. Reduced glutamic-acid-decarboxilase of post-mortem brain in Huntington's chorea. Lancet 1973;1:1090-1092.

[5] 5. Bonilla E, Prasad AL, Arrieta A. Huntington's disease: studies on brain free aminoacids. Life Sci 1988;42:1153-1158.

[6] 6. Bruyn GW. Chorea-acanthocytosis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:327-334.

[7] 7. Bruyn GW, Went LN. Huntington's chorea. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:267-313.

[8] 8. Crossman AR, Mitchell IJ, Sambrook MA, Jackson A. Chorea and myoclonus in the monkey induced by gumma-aminobutyric acid antagonism in the lentiform complex. Brain 1988; 111:1211 -1233.

[9] 9. Ellison DW, Real MF, Mazurek MF, Malloy JR, Bird ED, Martin JB. Aminoacid neurotransmitter abnormalities in Huntington's disease and the quinolinic acid animal model of Huntington's disease. Brain 1987; 110:1657-1673.

[10] 10. Greenhouse AH. On chorea, lupus erythematous and cerebral vasculitis. Arch Intern Med 1966; 117:389-393.

[11] 11. Hoefer PF, Putnam TJ. Action potentials of muscles in athetosis and Sydenham's chorea. Arch Neurol Psychiatry 1940;44:517-531.

[12] 12. Huntington G. On chorea. Medical and Surgical Reporter 1872;26:317-321.

[13] 13. Kanazawa I. Clinical pathology of basal ganglia disease. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:65-86.

[14] 14. Kanazawa I, Sasaki H, Muramoto O, Matsushita M, Mizutani T, Iwabushi K, Ikeda T, Takahata N. Studies on neurotransmitter markers and striatal neuronal cell density in Huntington's disease and dentatorubropallidoluysian atrophy. J Neurol Sci. 1985;70:151-165.

[15] 15. Kish SJ, Shannah K, Hornykiewcz O. Elevated serotonin and reduced dopamine in subregionally divided Huntington's disease striatum. Ann Neurol 1987;22:386-389.

[16] 16. Klawans HL. Chorea. Can J Neurol Sci 1987;14:536-540.

[17] 17. Klawans HL, Goetz CG, Perlik S. Presymptomatic and early detection in Huntington's disease. Ann Neurol 1980,8:343-347.

[18] 18. Lakke PF. Classification of extrapyramidal disorders. J Neurol Sci 1981 ;51:311-327.

[19] 19. Lyon IW. Chronic hereditary chorea. American Medical Times 1893;7:289-290.

[20] 20. Martin JB. Huntington's disease: new approachs to an old problem. Neurology 1984,34:1059-1072.

[21] 21. Mount LA, Reback S. Familial paroxysmal choreoathetosis: preliminary report on a hitherto undescribed clinical syndrome. Arch Neurol 1940;44:841 -846.

[22] 22. Nausieda PA. Sydenham's chorea, chorea gravidarum and contraceptive induced chorea. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:359-367.

[23] ²³
Organización Panamericana de Salud. Prevención y control de la fiebre reumatica en la comunidad. OPAS: 1980, Publicación cientifica 399.

[24] 24. Padberg GW, Bruyn GW. Chorea: differential diagnosis. In Vinken PJ, Bruyn GW, Klawans HL (eds). Handbook of clinical neurology: Extrapyramidal disorders. Amsterdam: Elsevier, 1986;5:549-564.

[25] 25. Perry TL, Hansen S, Kloster M. Huntington's chorea: deficiency of gumma-aminobutyric acid in brain. N Engl J Med 1973;288:337-342.

[26] 26. Shoulson I. On chorea. Clin Neuropharmacol 1986;9:585-599.

[27] 27. Spokes EG. Neurochemical alteration in Huntington's chorea: a study of post-mortem brain tissue. Brain 1980;103:179-210.

[28] 28. Sydenham T. The whole works of that excellent practical physician, Dr. Thomas Sydenham. London: R Wellington, 1712.

[29] 29. Thiebaut F, Isch F. Étude sémiologique du movement choréique. Rev Prat (Paris) 1957;8:127-130.

[30] 30. Wilson SAK. Disorders of motility and of muscle tone, with special reference to the corpus striatum. Lancet 1925;2:169-178.