Coreoatetose paroxistica familiar: registo de dois casos em uma familia

Zukerman, E.; Vilanova, L.C.P.; Serafico, J.

doi:10.1590/S0004-282X1983000400007

Resumos

Os autores relatam duas observações clínicas sendo mãe e filha portadoras de crises de coreoatetose, iniciadas aos 6 e 2 anos de idade com o diagnóstico de entidade rara: a coreoatetose paroxística familiar. São descritas as características clínicas e terapêuticas e são referidos casos na literatura.

The authors present two cases of a very rare condition known as familial paroxysmal choreoathetosis. The patients, mother and daughter, were 28 and 8 years old and the disease started when they were 6 and 2 year-old, respectively. The paroxystic involuntary movements were generalized, producing speech and gait disturbances, without any impairment of consciousness. The duration of the episodes lasted 30 to 180 minutes. The paroxysmals occasionaly could be triggered by physical stress or alcohol intake. The neurological examination and the electroencephalogram during free intervals were normal. Searching the available literature these twq cases could be classified as belonging to the same form described by Mount and Reback, in 1940.

Coreoatetose paroxistica familiar: registo de dois casos em uma familia

Familial paroxysmal choreoathetosis: report of two cases from the same kindship

E. Zukerman^I; L.C.P. Vilanova^II; J. Serafico^III

^IProfessor Adjunto e Chefe do Departamento. Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina

^IIProfessor Auxiliar de Ensino. Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina

^IIIMédico Estagiário. Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina

RESUMO

Os autores relatam duas observações clínicas sendo mãe e filha portadoras de crises de coreoatetose, iniciadas aos 6 e 2 anos de idade com o diagnóstico de entidade rara: a coreoatetose paroxística familiar. São descritas as características clínicas e terapêuticas e são referidos casos na literatura.

SUMMARY

The authors present two cases of a very rare condition known as familial paroxysmal choreoathetosis. The patients, mother and daughter, were 28 and 8 years old and the disease started when they were 6 and 2 year-old, respectively. The paroxystic involuntary movements were generalized, producing speech and gait disturbances, without any impairment of consciousness. The duration of the episodes lasted 30 to 180 minutes. The paroxysmals occasionaly could be triggered by physical stress or alcohol intake. The neurological examination and the electroencephalogram during free intervals were normal. Searching the available literature these twq cases could be classified as belonging to the same form described by Mount and Reback, in 1940.

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Trabalho da Disciplina de Neurologia do Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina. Apresentado no 5º Congresso Brasileiro de Neuro-psiquiatria Infantil, Salvador, outubro de 1979. Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina.

Clínica Neurologica - Escola Paulista de Medicina - Rua Botucatu 720 - 04023São Paulo, SP - Brasil.

1. HUDGINS, R.L. & CORBIN, K.B. - An uncommon seizure disorder-familial paroxysmal choreoathetosis. Brain 89:199, 1966.
2. KERTESZ, A. - Paroxysmal kinesigenic choreoathetosis. Neurology (Minneapolis) 17:680, 1967.
3. KISHIMOTO, K. - A novel case of conditionally responsive extrapiramidal syndrome. Ann. Rpt. Res. Inst. Environn. Med. (Nagoya Univ.) 6:91, 1957.
4. LISHIMAN, W.A.; WHITTY, C. W. M. & GIBBON, J. P. F. - Seizures induced by movement: a form of reflex epilepsy. Lancet (June): 1403, 1964.
5. MOUNT, L.A. & REBACK, S. - Familial paroxysmal choreoathetosis. Arch. Neurol. Psychiat. (Chicago) 44:841, 1940.
6. PEREZ-BORJA, C.; TASSINARI, A.C. «& SWANSON, A.G. - Paroxysmal choreoathetosis and seizures induced by movement. Epilepsia 8:260, 1967.
7. PRYLES, C.V.; LIVINSTONS, S. & FORD, F.R. - Familial paroxysmal choreoathetosis of Mount and Reback. Pediatrics 9:44, 1952.
8. RICHARDS, R.N. & BARNETT, H.J.M. - Paroxysmal dystonic choreoathetosis. Neurology (Minneapolis) 18:461, 1968.
9. STEVENS, H. - Paroxysmal choreo-atetosis-a form of reflex epilepsy. Arch. Neurol. (Chicago) 14:415, 1966.
10. WALLER, D.A. - Paroxysmal kinesigenic choreoathetosis or hysteria? Am. J. Psych. 134:1439, 1977.

Datas de Publicação

Publicação nesta coleção
14 Ago 2012
Data do Fascículo
Dez 1983

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[1] 1. HUDGINS, R.L. & CORBIN, K.B. - An uncommon seizure disorder-familial paroxysmal choreoathetosis. Brain 89:199, 1966.

[2] 2. KERTESZ, A. - Paroxysmal kinesigenic choreoathetosis. Neurology (Minneapolis) 17:680, 1967.

[3] 3. KISHIMOTO, K. - A novel case of conditionally responsive extrapiramidal syndrome. Ann. Rpt. Res. Inst. Environn. Med. (Nagoya Univ.) 6:91, 1957.

[4] 4. LISHIMAN, W.A.; WHITTY, C. W. M. & GIBBON, J. P. F. - Seizures induced by movement: a form of reflex epilepsy. Lancet (June): 1403, 1964.

[5] 5. MOUNT, L.A. & REBACK, S. - Familial paroxysmal choreoathetosis. Arch. Neurol. Psychiat. (Chicago) 44:841, 1940.

[6] 6. PEREZ-BORJA, C.; TASSINARI, A.C. «& SWANSON, A.G. - Paroxysmal choreoathetosis and seizures induced by movement. Epilepsia 8:260, 1967.

[7] 7. PRYLES, C.V.; LIVINSTONS, S. & FORD, F.R. - Familial paroxysmal choreoathetosis of Mount and Reback. Pediatrics 9:44, 1952.

[8] 8. RICHARDS, R.N. & BARNETT, H.J.M. - Paroxysmal dystonic choreoathetosis. Neurology (Minneapolis) 18:461, 1968.

[9] 9. STEVENS, H. - Paroxysmal choreo-atetosis-a form of reflex epilepsy. Arch. Neurol. (Chicago) 14:415, 1966.

[10] 10. WALLER, D.A. - Paroxysmal kinesigenic choreoathetosis or hysteria? Am. J. Psych. 134:1439, 1977.