TY - JOUR T1 - Wolfram syndrome associated with leukoencephalopathy JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 928 LP - 928 DO - 10.1136/jnnp.2009.185579 VL - 81 IS - 8 AU - Pierre Labauge AU - Dimitri Renard AU - Annabelle Chaussenot AU - Veronique Paquis-Flucklinger Y1 - 2010/08/01 UR - http://jnnp.bmj.com/content/81/8/928.abstract N2 - 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 … ER -