title: Bipolar Disorder Type 1 in a 17-Year-Old Girl with Wolfram Syndrome.
abstract: Wolfram syndrome (WS, MIM 222300) is a rare autosomal, recessive neurodegenerative disorder associated with mutations in WFS1, a gene that has been associated with bipolar disorder (BD). WS, characterized by the association of juvenile-onset diabetes mellitus (DM) and bilateral progressive optic atrophy (BPOA), encompasses several other clinical features, including cognitive impairments and psychiatric disorders. Detailed data on the psychiatric phenotype are still scarce, and how WS relates to BD is still unknown.
  
  
  
  A 17-year-old girl with WS was hospitalized for early-onset BD. A multidisciplinary and developmental assessment was carried out to control mood symptoms and address how BD could be related to WS.
  
  
  
  Besides DM and BPOA, the patient had several risk factors for BD/mood disorders as follows: (1) a history of abuse and maltreatment; (2) a history of specific language disorder and borderline intelligence associated with academic failure; and (3) a comorbid hypothyroidism. Treatment encompassed all aspects of the adolescent's conditions, such as the use of mood stabilizers, addressing psychosocial and scholastic problems, and treating hypothyroid dysfunction.
  
  
  
  Given the complexity of WS, this case suggests that the possible association between WS and BD should not only be merely limited to a possible statistical association with WFS1 polymorphism but also to developmental, cognitive, and endocrine risk factors for BD.
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Jean
authors.initials: J
authors.lastname: Xavier
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Nadège
authors.initials: N
authors.lastname: Bourvis
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Antoine
authors.initials: A
authors.lastname: Tanet
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Tatiana
authors.initials: T
authors.lastname: Ramos
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Didier
authors.initials: D
authors.lastname: Perisse
authors.affiliation: 3 Department of Genetics, Centre de Référence Déficience Intellectuelle de Causes Rares , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Isabelle
authors.initials: I
authors.lastname: Marey
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .|2 Institut des Systèmes Intelligents et Robotiques (ISIR), CNRS UMR 7222, Université Pierre et Marie Curie , Paris, France .
authors.forename: David
authors.initials: D
authors.lastname: Cohen
authors.affiliation: 1 Department of Child and Adolescent Psychiatry, Université Pierre et Marie Curie , Assistance Publique-Hôpitaux de Paris, GH Pitié-Salpêtrière, Paris, France .
authors.forename: Angèle
authors.initials: A
authors.lastname: Consoli
mesh_terms: D000293:Adolescent; D001714:Bipolar Disorder / Q000175:diagnosis* / Q000209:etiology; D003922:Diabetes Mellitus, Type 1 / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics; D009154:Mutation; D012307:Risk Factors; D014929:Wolfram Syndrome / Q000235:genetics / Q000503:physiopathology*
chemical_list: D008565:Membrane Proteins; C114987:wolframin protein
keywords: Wolframsyndrome; bipolar disorder; developmental assessment; risk factors; specific language disorder; treatment