[Russel's syndrome: diencephalic tumor in a child].

Abstract:

: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 months 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 nystagmus 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/1) 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).

journal_name

Arq Neuropsiquiatr

authors

Da Motta LA,Garcia Filho OV,Horta MP,Lima BO,Pratesi R,Farage Filho M

doi

10.1590/s0004-282x1990000100016

subject

Has Abstract

pub_date

1990-03-01 00:00:00

pages

107-12

issue

1

eissn

0004-282X

issn

1678-4227

journal_volume

48

pub_type

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