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Diencephalic syndrome of infancy in Child
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Diencephalic syndrome of infancy in Child

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Contributors: Benjamin L. Mazer MD, MBA, Jamie Adams MD
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Synopsis

Diencephalic syndrome of infancy, or Russell's syndrome, is a rare cause of failure to thrive. Infants present with severe emaciation despite adequate feeding and linear growth. Hydrocephalus, skin pallor, hyperactivity, euphoria, nystagmus, and vomiting are also sometimes observed. Symptoms typically begin during the first year of life.

Diencephalic syndrome is secondary to a neoplasm growing near the hypothalamus or optic chiasm. The physiologic cause of the failure to thrive is not fully understood, but is hypothesized to be associated with abnormal growth hormone secretion.

Diencephalic syndrome will resolve with treatment of the underlying tumor.

Codes

ICD10CM:
E23.6 – Other disorders of pituitary gland

SNOMEDCT:
45740001 – Diencephalic Syndrome of Infancy

Differential Diagnosis & Pitfalls

Best Tests

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Last Updated: 10/13/2017
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Diencephalic syndrome of infancy in Child
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Diencephalic syndrome of infancy : Failure to thrive, Hyperphagia, Cachexia
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