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Coreoatetose paroxística cinesiogênica: relato de um caso tratado com carbamazepina

Kinesigenic paroxysmal choreoathetosis: report of a case treated with carbamazepina

Resumos

A coreatetose paroxistica (CP) é entidade rara. Até mesmo profissionais que estudam desordens do movimento não costumam vê-la com freqüência. A ocorrência paroxística de movimentos distônicos, coréicos e atetósicos é a apresentação típica da síndrome. O início costuma ser abrupto e os ataques podem durar de alguns segundos até horas. Casos esporádicos e, mais frequentemente, casos familiares têm sido relatados. A abordagem terapêutica com anticonvulsivantes, como a carbamazepina, nem sempre tem sucesso. Com esta droga, porém, geralmente há boa resposta na variante cinesiogênica da CP. É relatado o caso de um paciente jovem do sexo masculino com essa variante da CP. O início da doença se deu na puberdade. O exame neurológico era normal entre os ataques. A investigação laboratorial, EEG, TC de crânio e RNM de segmento cefálico foram normais. Carbamazepina em doses baixas (100 mg/dia) foi eficaz no manejo dos ataques.

coreoatetose; paroxistica; cinesiogênica; anticonvulsivantes; carbamazepina


Paroxysmal choreoathetosis (PC) is a rare entity, and professionals who study movement disordes do not find it easily. Paroxysmal occurence of dystonic, choreic, athetosic movements is the typical feature of this syndrome. The onset is always subtle and the attacks can last from few seconds to hours. Sporadic cases and more common familial cases have been reported. The therapeutic approach with anticonvulsivant drugs like carbamazepine is not always successful. However, there is a good response to this drug in the kinesigenic form of PC. The case of a 21 years old male patient is reported here. The onset occurred during puberty, at 14 years old. Neurologic examination was normal between attacks. Subtle and brief choreic, athetosic and dystonic limb and legs movements precipitated by sudden quick and unexpected passive movements, as by startle, were observed during the attacks. There was no familial history. Routine laboratorial investigation, EEG, CT scan and MRI did not show significant changes. Carbamazepine in low dosages (100 mg/day) brought a complete control of the attacks.

choreoathetosis; paroxysmal; kinesigenic; anticonvulsivants; carbamazepine


Coreoatetose paroxística cinesiogênica: relato de um caso tratado com carbamazepina

Kinesigenic paroxysmal choreoathetosis: report of a case treated with carbamazepina

P.E.M. Carrilho; E.R. Barbosa, M.S. Haddad; L.R. Comerlatti; J.C.P. Limongi; M. Scaff

Clínica Neurológica do Hospital das Clínicas (HC) da Faculdade de Medicina da Universidade de São Paulo (FMUSP)

RESUMO

A coreatetose paroxistica (CP) é entidade rara. Até mesmo profissionais que estudam desordens do movimento não costumam vê-la com freqüência. A ocorrência paroxística de movimentos distônicos, coréicos e atetósicos é a apresentação típica da síndrome. O início costuma ser abrupto e os ataques podem durar de alguns segundos até horas. Casos esporádicos e, mais frequentemente, casos familiares têm sido relatados. A abordagem terapêutica com anticonvulsivantes, como a carbamazepina, nem sempre tem sucesso. Com esta droga, porém, geralmente há boa resposta na variante cinesiogênica da CP. É relatado o caso de um paciente jovem do sexo masculino com essa variante da CP. O início da doença se deu na puberdade. O exame neurológico era normal entre os ataques. A investigação laboratorial, EEG, TC de crânio e RNM de segmento cefálico foram normais. Carbamazepina em doses baixas (100 mg/dia) foi eficaz no manejo dos ataques.

Palavras-chave: coreoatetose, paroxistica, cinesiogênica, anticonvulsivantes, carbamazepina.

SUMMARY

Paroxysmal choreoathetosis (PC) is a rare entity, and professionals who study movement disordes do not find it easily. Paroxysmal occurence of dystonic, choreic, athetosic movements is the typical feature of this syndrome. The onset is always subtle and the attacks can last from few seconds to hours. Sporadic cases and more common familial cases have been reported. The therapeutic approach with anticonvulsivant drugs like carbamazepine is not always successful. However, there is a good response to this drug in the kinesigenic form of PC. The case of a 21 years old male patient is reported here. The onset occurred during puberty, at 14 years old. Neurologic examination was normal between attacks. Subtle and brief choreic, athetosic and dystonic limb and legs movements precipitated by sudden quick and unexpected passive movements, as by startle, were observed during the attacks. There was no familial history. Routine laboratorial investigation, EEG, CT scan and MRI did not show significant changes. Carbamazepine in low dosages (100 mg/day) brought a complete control of the attacks.

Key words: choreoathetosis, paroxysmal, kinesigenic, anticonvulsivants, carbamazepine.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 10-dezembro-1993.

Dr. Paulo Eduardo Mestrinelli Carrilho - Clínica Neurológica, Hospital das Clínicas, FMUSP - Caixa Postal 8091 - 01065-970 São Paulo SP - Brasil.

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Datas de Publicação

  • Publicação nesta coleção
    19 Jan 2011
  • Data do Fascículo
    Set 1994
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