An Interesting Case of Thyrotoxicosis with Choreoathetosis - A Rare Association.

Thyrotoxicosis may present with spectrum of movement disorders. Though tremor is most frequently associated, chorea has also been reported rarely. A rare case of thyrotoxicosis in a young female presenting with choreoathetotic movement is reported here. The choreoathetotic movement in this case was attributed to thyrotoxicosis based on clinical and biochemical criteria after exclusion of other causes.

Huntington disease [Chorea] in the Middle East

Huntington disease [HD] has been reported in Arab families in several Middle East countries including Saudi Arabia, Oman, Syria, Lebanon, and Egypt and in non-Arab populations in other countries in the region. It is probably under-reported, and until now, has not been recorded in Yemen, the United Arab Emirates, Jordan or in Iraq. The Middle East has always been on the crossroads of trade and travel, and HD was probably introduced to some of these countries in previous times. The prevalence rate in Middle Eastern Arabs is estimated to vary from 3 to 4 per 100,000. Although the HD gene which codes for the protein huntingtin has been identified, the function of this protein is not known. At present, no treatment has been found to delay the onset of HD or to treat it effectively. Although relatively rare, HD has increasingly become a focus of international gene research, with the support and collaboration of the International Huntington Association [IHA]. The IHA has been represented in Saudi Arabia and Oman

Sydenham's chorea: clinical and evolutive characteristics

CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992). We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks) and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35 percent of the patients, and 25 (29 percent) presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71 percent of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea recurrence

Sydenham chorea: clinical and laboratory findings: analysis of 187 cases

Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called pure chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil

Aspectos clinicos e laboratoriais de pacientes hospitalizados por coreia reumatica / Clinical and laboratory aspects of patients hospitalized for rheumatic chorea

O trabalho apresenta uma revisao clinica retrospectiva dos prontuarios de 49 pacientes hospitalizados por coreia reumatica. A idade dos pacientes na epoca do dignostico foi, em media, de 12,3 anos. Exluindo-se os movimentos coreicos, a hipotonia constitutiu o sinal neurologico mais frequente. A poliartrite foi o sinal maior mais vezes associado a coreia. Dos exames laboratoriais, o titulo de antiestreptolisina O e a proteina serica estavam alterados em mais da metade dos pacientes. A coreia reumatica, que representa uma das formas de manifestacao da febre reumatica, vinha apresentando reducao da sua incidencia: nos ultimos anos, entretanto, parece ter havido aumento significativo do numero de casos, a semelhanca de relatos da literatura