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Wolfram syndrome associated with leukoencephalopathy
  1. Pierre Labauge1,
  2. Dimitri Renard1,
  3. Annabelle Chaussenot2,
  4. Veronique Paquis-Flucklinger2
  1. 1Department of Neurology, CHU de Nîmes, 30 029 Nîmes Cedex, France
  2. 2Department of Medical Genetics and FRE 3086 CNRS/UNS, CHU de Nice and Medicine School, 06 000 Nice, France
  1. Correspondence to Pierre Labauge, Department of Neurology, CHU de Nîmes. 30 029 Nîmes Cedex, France; labauge{at}yahoo.fr

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A 29-year-old woman born from consanguineous parents developed, since the age of 5, insulin-dependent diabetes mellitus, bilateral optic atrophy, neurosensory hearing loss, ataxia and chronic depression. The patient had normal learning capacities. Clinical examination showed ataxia and reduced lower leg deep tendon reflexes. Testing of cognitive functions revealed clearly a frontal network deficit and amnesic dysfunction together with a mild to moderate depression. Brain MRI …

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