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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.48 no.1 São Paulo Mar. 1990

http://dx.doi.org/10.1590/S0004-282X1990000100016 

Sindrome de russel tumor diencefálico em uma criança

 

Russel's syndrome: diencephalic tumor in a child

 

 

Luiz A. C. Roxo da MottaI; Osvaldo V. Garcia FilhoII; Marcio P. HortaIII; Benicio O. LimaIII; Riccardo PratesiIV; Miguel Farage FilhoV

IMédico do Núcleo de Neuroendocrinologia da Unidade de Neurocirurgia do HBDF e Serviço de Endocrinologia da Unb - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)
IIResidente da Unidade de Neurocirurgia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)
IIICirurgião do Setor de Neurocirurgia Pediátrica do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)
IVProfessor de Neurologia da UnB e médico da Unidade de Neurologia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)
VChefe da Unidade de Neurocirurgia do HBDF - Trabalho realizado na Unidade de Neurocirurgia do Hospital de Base do Distrito Federal (HBDF) e Serviço de Endocrinologia da Universidade de Brasília (UnB)

 

 


RESUMO

Apresenta-se o caso de criança do sexo masculino com síndrome de Russel devida a astrocitoma pilocítico localizado na região diencefálica. O diagnóstico foi realizado aos 16 meses de idade, mas sua sintomatologia se iniciou aos 4 meses, com emagrecimento progressivo. À internação apresentava-se com peso de 6150g e 74cm de estatura, emaciada, sem panículo adiposo, irritadica e com sinais de hipertensão intracraniana. Existia estrabismo convergente, nistagmo vertical do olho esquerdo e papiledema bilateral. Os reflexos tendinosos eram exaltados e tinha tetraparesia espástica. A avaliação endócrina demonstrou aumento basal do GH (23 ng/ml), do TSH (6,2 mUI/l) e da prolactina (26 ng/ml). Os dois primeiros hormônios não responderam ao teste agudo com o TRH, enquanto a prolactina teve resposta pobre. Foi submetida a radioterapia com acelerador linear (dose total de 4000 rads) e a cirurgia, na qual não se conseguiu extirpar todo o tumor, devido a seu tamanho. Nove meses após, a criança encontra-se bem, com apreciável ganho ponderai (2500g).


SUMMARY

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th month of life, when he started losing weight By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nistagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/l) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).


 

 

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Dr. Luiz A. C. Roxo da Motta - Núcleo de Neuroendocrinologia, Unidade de Neurocirurgia, Hospital de Base do Distrito Federal - SMHS Q 101 - 70333 Brasília DF - Brasil

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