Russell's syndrome II (Alexander Russell)

Alternative eponyms

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A diencephalic syndrome of infancy marked by progressive emaciation in spite of normal or increased food intake, tremor, ataxia, etc.

Description

A syndrome in children who fail to thrive, marked by progressive emaciation in spite of normal or increased food intake, tremor, ataxia and sometimes nystagmus (50%), euphoric appearance, exceptional alertness, accelerated growth with acromegaloid features, hypotension, hypoglycemia. It is usually due to a tumour in the diencephalon, most commonly a glioma. Both sexes affected; onset at 3 months to 2 years.

Bibliography

  • A. Russell:
    Diencephalic Syndrome of Hyperkinetic Emaciation. Doctoral dissertation, 1948. A diencephalic syndrome of emaciation in infancy and childhood.
    Archives of Disease in Childhood, London, 1951, 26: 274.

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