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Diencephalic syndrome

Disease definition

Diencephalic syndrome (DS) is a rare condition characterized by profound emaciation and failure to thrive (with normal caloric intake and normal linear growth), hyperalertness, hyperkinesia and euphoria, in the presence of hypothalamic tumors.

ORPHA:1672

  • Synonym(s):
    • Diencephalic cachexia
    • Diencephalic syndrome of childhood
    • Diencephalic syndrome of emaciation
    • Russell diencephalic cachexia
    • Russell syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Childhood
  • ICD-10: C72.8
  • OMIM: -
  • UMLS: C0271889  C0342436
  • MeSH: -
  • GARD: 6276
  • MedDRA: -

Detailed information

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