Juvenile diabetes and optic atrophy

  • S. M. M. Sheriff
  • J. G. Tetley
  • C. R. Maddock


A familial condition characterized by juvenile diabetes mellitus (DM), optic atrophy (OA), nerve deafness (D), diabetes insipidus (DI) and central nervous system defects is appearing in the medical literature with increasing frequency. These clinical features are found in variable combinations. The syndrome is either referred to as Wolfram-Tyrer syndrome1 after two independent early observers2,3, or as DIDMOAD syndrome4, an acronym depicting some of its most commonly expressed features. Other features have been reported including heredo-familial ataxias, dilatation of the urinary tract and sideroblastic anaemia. Despite the diversity of expression of the syndrome, there is a similarity in the pattern of clinical presentation among affected individuals of the same family.


Hearing Loss Juvenile Diabetes Diabetes Insipidus Optic Atrophy Affected Sibling 
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Copyright information

© The Society for the Study of Inborn Errors of Metabolism 1980

Authors and Affiliations

  • S. M. M. Sheriff
  • J. G. Tetley
  • C. R. Maddock

There are no affiliations available

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