DIDMOAD SYNDROME (DIABETES-INSIPIDUS, DIABETES-MELLITUS, OPTIC ATROPHY, DEAFNESS) WITH CEREBELLOPONTINE ATROPHY

  • 1 January 1982
    • journal article
    • research article
    • Vol. 112  (10) , 348-352
Abstract
The case is reported of a 32 yr old woman of Dutch origin who presented with diabetes insipidus, diabetes mellitus, atrophy of the optic nerve and dilatation of the urinary tract, the combination known as DIDMOAD syndrome. Unusual features of this case were regional atrophy of the cerebellum and the pons, and hydrocephalus intermus which was associated with alteration of personality and mental function. The clinical symptoms of the syndrome simulated essential type 1 diabetes with advanced complications such as retinopathy and autonomous and peripheral neuropathy. The presence of diabetes insipidus with polyuria and polydipsia was not recognized for years because the symptoms were ascribed to diabetes mellitus. The neurologic and psychological symptoms in this patient suggest a more generalized defect of the CNS in this syndrome than has been observed previously. Recognition of the condition is of importance because it requires specific therapeutic measures (e.g. for diabetes insipidus), and because of the genetic and prognostic implications.

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