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Wolfram Syndrome: Case Report and Review of the Literature

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Comprehensive Therapy

Wolfram syndrome (WFS) is a rare diffuse neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, deafness, and a wide variety of central nervous system abnormalities. Insulin-dependent diabetes mellitus with optic nerve atrophy is a sufficient criterion for the diagnosis. WFS is a devastating disease for the patients and their families. This study emphasizes the need for careful evaluation of cases having insulin-dependent diabetes mellitus and optic atrophy.

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References

  • Barret TG, Bundey SE. Wolfram (DIDMOAD) syndrome. J Med Genet 1997;34:838–41.

    Google Scholar 

  • Von Graeffe A. Uber die mit diabetes vorkommenden shstorunger. Arch Ophthalmol 1858;4:230–44.

    Google Scholar 

  • Wolfram DJ. Diabetes mellitus and simple optic atrophy among siblings. A report of four cases. Proc Mayo Clin 1938;13:715–8.

    Google Scholar 

  • Shaw DA, Ducan JP. Optic atrophy and nerve deafness in diabetes insipidus. J Neurol Neurosurg Psychiatry 1958;2:47.

    Google Scholar 

  • Ikkos DG, Fraser GR, Matsouki-Gaurae, Petrochilos M. The association of juvenile diabetes mellitus, primary optic atrophy and perceptive hearing loss in three siblings with additional diabetes mellitus, diabetes insipidus in one case. Acta Endocrinol 1970;65:95–105.

    PubMed  CAS  Google Scholar 

  • Najjar SS, Saikaly MG, Zaytoun GM, Abdelnoor A. Association of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. The Wolfram or DIDMOAD syndrome. Arch Dis Child 1985;60:823–8.

    Article  PubMed  CAS  Google Scholar 

  • Zen PR, Pinto LL, Schwartz IV, Barrett TG, Paskulin G. Report of a Brazilian patient with Wolfram Syndrome. J Pediatr (Rio J) 2002;78:529–32.

    Article  Google Scholar 

  • Blasi C, Pierelli F, Rispoli E, Sapanora M, Vingob E, Andreani D. Wolfram’s syndrome: a clinical, diagnostic, and interpretative contribution. Diabetes Care 1986;9:521–8.

    Article  PubMed  CAS  Google Scholar 

  • Fishman L, Ehrlich RM. Wolfram syndrome: report of four cases and review of literature. Diabetes Care 1986;9:405–8.

    Article  PubMed  CAS  Google Scholar 

  • Kinsley BT, Firth RGF. The Wolfram syndrome: a primary neurodegenerative disorder with lethal potential. Ir Med J 1992;85:34–6.

    PubMed  CAS  Google Scholar 

  • Cremers CWR, Wijdeveld PGAB, Pinckers AJLG. Juvenile diabetes mellitus, optic atrophy, hearing loss, diabetes insipidus, atonia of the urinary tract and bladder and other abnormalities (Wolfram syndrome). Acta Paediatr Scand Suppl 1977;264:1–16.

    Article  PubMed  Google Scholar 

  • Barret TG, Bundey SE, Macleod AF. Neurodegeneration and diabetes: UK nationwide study of Wolfram (DIDMOAD) syndrome. Lancet 1995;346:1458–63.

    Article  Google Scholar 

  • Inoue H, Tanizawa Y, Wasson J, Behn P, Kalidas K, Bernal-Mizrachi E, et al. A gene encoding a transmembrane protein is mutated in patients with diabetes mellitus and optic atrophy (Wolfram syndrome). Nat Genet 1998;20:143–8.

    Article  PubMed  CAS  Google Scholar 

  • Simsek E, Simsek T, Tekgul S, Hosal S, Seyrantepe V, Aktan G. Wolfram (DIDMOAD) syndrome: a multidisciplinary clinical study in nine Turkish patients and review of the literature. Acta Paediatr 2003;92:55–61.

    Article  PubMed  CAS  Google Scholar 

  • Galluzzi P, Filosomi G, Vallone IM, Bardelli AM, Venturi C. MRI of Wolfram syndrome (DIDMOAD). Neuroradiology 1999;41:729–31.

    Article  PubMed  CAS  Google Scholar 

  • Swift RG, Sadler DB, Swift M. Psychiatric findings in Wolfram syndrome homozygotes. Lancet 1990;336:667–9.

    Article  PubMed  CAS  Google Scholar 

  • Kehl O, Keller U. DIDMOAD-syndrome (diabetes insipidus, diabetes mellitus, optic atrophy, deafness) with cerebello-pontine atrophy. Schweiz Med Wochenschr 1982;112:348–52 (in German).

    PubMed  CAS  Google Scholar 

  • Pakdemirli E, Karabulut N, Bir LS, Sermez Y. Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome. Australas Radiol 2005;49:189–91.

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Şeyhmus Ari.

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The authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or provider of services discussed in this article. The authors also do not discuss the use of off-label products, which includes unlabeled, unapproved, or investigative products or devices.

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Ari, Ş., Keklíkçí, U., Çaça, İ. et al. Wolfram Syndrome: Case Report and Review of the Literature. Compr Ther 33, 18–20 (2007). https://doi.org/10.1007/s12019-007-0007-z

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  • DOI: https://doi.org/10.1007/s12019-007-0007-z

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