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Wolfram Syndrome

Am J Dis Child. 1978;132(8):818-819. doi:10.1001/archpedi.1978.02120330090023.
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Sir.—We read with great interest the report by Carson et al on simultaneous occurrence of diabetes mellitus, diabetes insipidus, and optic atrophy in a brother and sister (Am J Dis Child 131:1382-1385, 1977) and would like to add the following data that seemed to have escaped the attention of the authors. A review of 91 cases with this syndrome called Wolfram syndrome has been published by Cremers et al.1 These authors also report a postmortem on one patient that disclosed noticeable dilation of the renal pelvis, ureters, and bladder. The hypophysis, hypothalamus, thalamus, substantia nigra, and red nucleus were normal. The optic nerve, chiasma, and both optic tracts showed slight gliosis and a loss of myelin structure, but no signs of active myelination.

From this clinic, we have reported in 1975,2 two brothers with juvenile diabetes mellitus and optic atrophy with hypothalamic disease as judged by clinical


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