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Comprehensive Therapy
Published in: Comprehensive Therapy 1/2007

01-09-2007 | Original Article

Wolfram Syndrome: Case Report and Review of the Literature

Authors: Şeyhmus Ari, Uğur Keklíkçí, İhsan Çaça, Kaan Ünlü, Hasan Kayabaşi

Published in: Comprehensive Therapy | Issue 1/2007

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Abstract

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.
Literature
Barret TG, Bundey SE. Wolfram (DIDMOAD) syndrome. J Med Genet 1997;34:838–41.
Von Graeffe A. Uber die mit diabetes vorkommenden shstorunger. Arch Ophthalmol 1858;4:230–44.
Wolfram DJ. Diabetes mellitus and simple optic atrophy among siblings. A report of four cases. Proc Mayo Clin 1938;13:715–8.
Shaw DA, Ducan JP. Optic atrophy and nerve deafness in diabetes insipidus. J Neurol Neurosurg Psychiatry 1958;2:47.
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
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.PubMedCrossRef
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.CrossRef
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.PubMedCrossRef
Fishman L, Ehrlich RM. Wolfram syndrome: report of four cases and review of literature. Diabetes Care 1986;9:405–8.PubMedCrossRef
Kinsley BT, Firth RGF. The Wolfram syndrome: a primary neurodegenerative disorder with lethal potential. Ir Med J 1992;85:34–6.PubMed
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.PubMedCrossRef
Barret TG, Bundey SE, Macleod AF. Neurodegeneration and diabetes: UK nationwide study of Wolfram (DIDMOAD) syndrome. Lancet 1995;346:1458–63.CrossRef
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.PubMedCrossRef
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.PubMedCrossRef
Galluzzi P, Filosomi G, Vallone IM, Bardelli AM, Venturi C. MRI of Wolfram syndrome (DIDMOAD). Neuroradiology 1999;41:729–31.PubMedCrossRef
Swift RG, Sadler DB, Swift M. Psychiatric findings in Wolfram syndrome homozygotes. Lancet 1990;336:667–9.PubMedCrossRef
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
Pakdemirli E, Karabulut N, Bir LS, Sermez Y. Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome. Australas Radiol 2005;49:189–91.PubMedCrossRef
Metadata
Title
Wolfram Syndrome: Case Report and Review of the Literature
Authors
Şeyhmus Ari
Uğur Keklíkçí
İhsan Çaça
Kaan Ünlü
Hasan Kayabaşi
Publication date
01-09-2007
Publisher
Humana Press Inc
Published in
Comprehensive Therapy / Issue 1/2007
Print ISSN: 0098-8243
Electronic ISSN: 1559-1190
DOI
https://doi.org/10.1007/s12019-007-0007-z

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