Sydenham's Chorea: A Clinical Follow-Up of 65 Patients

Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset of the symptoms was 11.7 ± 2.6 years (range, 6-17 years). Of the patients, 63% were female and 37% were male (male/female: 1.7/1). Chorea was generalized in 78.5% of the patients, right hemichorea in 12.3%, and left hemichorea 9.2%. There was a history of rheumatic fever in 30.8% of the patients. Echocardiographic study showed cardiac valve involvement in 70.5% of 61 patients. Brain magnetic resonance imaging, which was performed on only 18 patients, was evaluated as normal in all. Electroencephalography was also performed on only 18 patients and showed abnormal waves in 50% of them. Pimozide was mostly the first choice of drug therapy. Nevertheless, drug therapy was not needed in 18.5% of the patients. The recovery period of the first attack of the chorea was 1 to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenham's chorea is still an important health problem in Turkey with respect to its morbidity.

[1]  M. Jergas,et al.  Sydenham chorea: clinical, EEG, MRI and SPELT findings in the early stage of the disease , 1993, Journal of Neurology.

[2]  H. Ring,et al.  Neuropsychiatry of the basal ganglia , 2002, Journal of neurology, neurosurgery, and psychiatry.

[3]  G. Dbaibo,et al.  Rheumatic Fever in Children: A 15-Year Experience in a Developing Country , 2000, Pediatric Cardiology.

[4]  İ. Reisli,et al.  Acute rheumatic fever in Konya, Turkey , 2000, Pediatrics international : official journal of the Japan Pediatric Society.

[5]  M. Castillo,et al.  Sydenham's chorea: MRI and proton spectroscopy , 1999, Neuroradiology.

[6]  S. B. Becher,et al.  Chorée de Sydenham chez l'enfant , 1999 .

[7]  M. Shevell,et al.  Restricted Unilateral Sydenham's Chorea: Reversible Contralateral Striatal Hypermetabolism Demonstrated on Single Photon Emission Computed Tomographic Scanning , 1999, Journal of child neurology.

[8]  A. Gebremariam.  Sydenham's chorea: risk factors and the role of prophylactic benzathine penicillin G in preventing recurrence. , 1999, Annals of tropical paediatrics.

[9]  P. Lee,et al.  Serial brain SPECT images in a case of Sydenham chorea. , 1999, Archives of neurology.

[10]  S. Ben Becher,et al.  [Sydenham's chorea in children]. , 1999, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

[11]  M. Morimatsu,et al.  High-signal basal ganglia on T1-weighted images in a patient with Sydenham's chorea , 1998, Neuroradiology.

[12]  P. Lombroso,et al.  Sydenham's chorea. , 1997, The Psychiatric clinics of North America.

[13]  F. Cardoso,et al.  Chorea in fifty consecutive patients with rheumatic fever , 1997, Movement disorders : official journal of the Movement Disorder Society.

[14]  P. Vieco,et al.  Sydenham Chorea , 1997, Neurology.

[15]  J. Rapoport,et al.  Sydenham's chorea , 1995, Neurology.

[16]  T. Fuchigami,et al.  [Effectiveness of haloperidol in the treatment of chorea minor]. , 1995, No to hattatsu = Brain and development.

[17]  T. Sipahi,et al.  Acute rheumatic fever in children in the Ankara area in 1990–1992 and comparison with a previous study in 1980–1989 , 1994, Acta paediatrica.

[18]  M. Bronze,et al.  Epitopes of streptococcal M proteins that evoke antibodies that cross-react with human brain. , 1993, Journal of immunology.

[19]  L. Culpepper,et al.  Antineuronal antibodies in movement disorders. , 1993, Pediatrics.

[20]  A. Luxen,et al.  Reversible striatal hypermetabolism in a case of sydenham's chorea , 1993, Movement disorders : official journal of the Movement Disorder Society.

[21]  H. Herzog,et al.  Increased striatal glucose consumption in sydenham's chorea , 1993, Movement disorders : official journal of the Movement Disorder Society.

[22]  A. Bisno,et al.  Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. , 1992, JAMA.

[23]  R. Shakir,et al.  Effectiveness of sodium valproate in the treatment of Sydenham's chorea , 1990, Neurology.

[24]  G. Fenichel,et al.  Pimozide treatment of Sydenham's chorea , 1990, Neurology.

[25]  J. Artigas Palláres,et al.  [Carbamazepine in paroxysmal choreoathetosis in Sydenham's chorea]. , 1989, Anales espanoles de pediatria.

[26]  M. Roig,et al.  Carbamazepine: an alternative drug for the treatment of nonhereditary chorea. , 1988, Pediatrics.

[27]  S. Ganji,et al.  Sydenham's Chorea: Clinical, EEG, CT Scan, and Evoked Potential Studies , 1988, Clinical EEG.

[28]  G. Novak,et al.  Valproic acid in the treatment of Sydenham chorea. , 1985, Pediatric neurology.

[29]  J. G. Gibson,et al.  Successful treatment of refractory Sydenham's chorea with pimozide. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[30]  S. Naidu,et al.  Sydenham's chorea: A possible presynaptic dopaminergic dysfunction initially , 1980, Annals of Neurology.

[31]  Michael J. Aminoff,et al.  Electrodiagnosis in Clinical Neurology , 1980 .

[32]  L. Ch'ien,et al.  Sydenham's chorea and seizures. Clinical and electroencephalographic studies. , 1978, Archives of neurology.

[33]  L. N. Green.  Corticosteroids in the treatment of Sydenham's chorea. , 1978, Archives of neurology.

[34]  C. Hawkes,et al.  Tetrabenazine in Sydenham's chorea. , 1977, British medical journal.

[35]  P. Bray,et al.  A danger of haloperidol therapy in children. , 1976, The Journal of pediatrics.