Orphanet: Ogden syndrome

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Ogden syndrome

Disease definition

Ogden syndrome is a rare, genetic progeroid syndrome characterized by a variable phenotype including postnatal growth delay, severe global developmental delay, hypotonia, non-specific dysmorphic facies with aged appearance and cryptorchidism, as well as cardiac arrthymias and skeletal anomalies. Patients typically present with widely opened fontanels, mainly truncal hypotonia, a waddling gait with hypertonia of the extremities, small hands and feet, broad great toes, scoliosis and redundant skin with lack of subcutaneous fat.


Classification level: Disorder
  • Synonym(s):
    • Premature aging appearance-developmental delay-cardiac arrhythmia syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: X-linked recessive or X-linked dominant 
  • Age of onset: Infancy, Neonatal
  • ICD-10: E34.8
  • OMIM: 300855
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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