Pharmacopsychiatry 2015; 48(03): 104-110
DOI: 10.1055/s-0034-1398612
Original Paper
© Georg Thieme Verlag KG Stuttgart · New York

Atypical Antipsychotics and Relapsing Psychoses in 22q11.2 Deletion Syndrome: A Long-term Evaluation of 28 Patients

W. M. A. Verhoeven
1   Vincent van Gogh Institute for Psychiatry, Centre of Excellence for Neuropsychiatry, Venray, The Netherlands
2   Department of Psychiatry, Erasmus University Medical Centre, Rotterdam, The Netherlands
,
J. I. M. Egger
1   Vincent van Gogh Institute for Psychiatry, Centre of Excellence for Neuropsychiatry, Venray, The Netherlands
3   Behavioural Science Institute, Radboud University Nijmegen, Nijmegen, The Netherlands
4   Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands
› Author Affiliations
Further Information

Publication History

received 07 July 2014
revised 15 November 2014

accepted 07 January 2015

Publication Date:
05 February 2015 (online)

Abstract

Introduction: This study includes 28 patients with genetically proven 22q11.2 deletion syndrome referred for treatment-resistant psychoses and aims at the identification of a suitable pharmacological treatment strategy.

Methods: Based on standardized diagnostic procedures, key psychiatric symptoms and cognitive status were assessed. Also, data about previous diagnostic vignettes as well as the history of psychotropic medication and medical conditions were collected. Finally, the effect of the subsequent treatment regimen was periodically re-assessed.

Results: Since psychotic symptoms had been shown to be non-responsive to conventional antipsychotics including risperidone, treatment with either clozapine or quetiapine was started. In 21 patients, a substantial reduction of psychotic symptoms was achieved with either one, and in 3-quarters of this group remission was attained over a longer follow-up period. In a significant number of patients, valproic acid was added either for mood stabilizing purposes or to avoid epileptic side effects of clozapine.

Discussion: Treatment of psychotic symptoms in patients with 22q11DS with the atypical antipsychotic quetiapine or clozapine in combination with the mood-stabilizing anticonvulsant valproic acid, appears likely to be more effective than with other psychotropic compounds.

 
  • References

  • 1 Shprintzen R. Velo-cardio-facial syndrome: a distinctive behavioural phenotype. Ment Retard Dev Disabil Res Rev 2000; 6: 142-147
  • 2 Baker K, Vorstman JAS. Is there a core neuropsychiatric phenotype in 22q11.2 deletion syndrome?. Curr Opin Neurol 2012; 25: 131-137
  • 3 Jolin EM, Weller RA, Weller EB. Occurrence of affective disorders compared to other psychiatric disorders in children and adolescents with 22q11.2 deletion syndrome. J Affect Disord 2012; 136: 222-228
  • 4 Squarcione C, Torti MC, Di Fabio F et al. 22q11 Deletion syndrome: a review of the neuropsychiatric features and their neurobiological basis. Neuropsychiatr Dis Treat 2013; 9: 1873-1884
  • 5 Tang SX, Yi JJ, Calkins ME et al. Psychiatric disorders in 22q11.2 deletion syndrome are prevalent but undertreated. Psychol Med 2014; 44: 1267-1277
  • 6 Schneider M, Debbané M, Bassett AS et al. Psychiatric disorders from childhood to adulthood in 22q11.2 deletion syndrome: results from the international consortium on brain and behaviour in 22q11 deletion syndrome. Am J Psychiatry 2014; 171: 627-639
  • 7 Verhoeven WMA, Egger JIM, Tuinier S. Thoughts on the behavioural phenotypes in Prader-Willi syndrome and velo-cardio-facial syndrome: a novel approach. Acta Neuropsychiatr 2007; 19: 244-250
  • 8 Gothelf D, Schneider M, Green T et al. Risk factors and the evolution of psychosis in 22q11.2 deletion syndrome: a longitudinal 2-site study. J Am Acad Child Adolesc Psychiatry 2013; 52: 1192-1203
  • 9 Henry JC, van Amelsvoort T, Morris RG et al. An investigation of the neuropsychological profile in adults with velo-cardio-facial syndrome (VCFS). Neuropsychologia 2002; 40: 471-478
  • 10 Zinkstok J, van Amelsvoort T. Neuropsychological profile and neuroimaging in patients with 22q11.2 deletion syndrome: a review keywords. Child Neuropsychol 2005; 11: 21-37
  • 11 Niklasson L, Gillberg C. The neuropsychology of 22q11 deletion syndrome. A neuropsychiatric study of 100 individuals. Res Dev Disabil 2010; 31: 185-194
  • 12 Zarchi O, Diamond A, Weinberger R et al. A comparative study of the neuropsychiatric and neurocognitive phenotype in two microdeletion syndromes: velocardiofacial (22q11.2 deletion) and Williams (7q11.23 deletion) syndromes. Eur Psychiatry 2014; 29: 203-210
  • 13 Lajiness-ONeill R, Beaulieu I, Asamoah A et al. The neuropsychological phenotype of velocardiofacial syndrome (VCFS): relationship tot psychopathology. Arch Clin Neuropsychol 2006; 23: 175-184
  • 14 Schneider M, Van der Linden M, Menghetti S et al. Predominant negative symptoms in 22q11 deletion syndrome and their associations with cognitive functioning and functional outcome. J Psychiatry Res 2014; 48: 85-93
  • 15 Duijff SN, Klaassen PWJ, Swanenburg de Veye HFN et al. Cognitive development in children with 22q11.2 deletion syndrome. Br J Psychiatry 2012; 200: 462-468
  • 16 Zaleski C, Bassett AS, Tam K et al. The co-occurrence of early onset Parkinson disease and 22q11.2 deletion syndrome. Am J Med Genet 2009; 149A: 525-528
  • 17 Butcher NJ, Kiehl TR, Hazrati LN et al. Association between early-onset Parkinson disease and 22q11.2 deletion syndrome. Identification of a novel genetic form of Parkinson disease and its clinical implications. JAMA Neurol 2013; 70: 1359-1366
  • 18 McHugh L, Barnes-Holmes Y, Barnes-Holmes D. Persepective-taking as relational responding a developmental profile. Psychol Rec 2004; 54: 115-144
  • 19 Vilardaga R, Estévez A, Levin ME et al. Deictic relational responding, empathy, and experiential avoidance as predictors of social anhedonia: further contributions from relational frame theory. Psychol Rec 2012; 62: 409-432
  • 20 Åsberg M, Perris C, Schallig D et al. The CPRS. Development and applications of a psychiatric rating scale. Acta Psychiatr Scand 1978; ((suppl): 271)
  • 21 Goekoop JG, Knoppert-van der Klein EAM, Hoeksema T et al. Onderzoek met de CPRS in Nederlandse vertaling. Betrouwbaarheid, factorstructuur en intensiteits-beoordeling. Tijschr Psychiatrie 1994; 36: 520-526
  • 22 Royal College of Psychiatrists . DC-LD. Diagnostic criteria for psychiatric disorders for use with adults with learning disabilities/mental retardation. London: Gaskell; 2001
  • 23 Mailman RB, Murthy V. Third generation antipsychotic drugs: partial agonism or receptor functional selectivity?. Curr Pharm Des 2010; 16: 488-501
  • 24 Meltzer HY. Update on typical and atypical antipsychotic drugs. Annu Rev Med 2013; 64: 393-406
  • 25 Gladston S, Clarke DJ. Clozapine treatment of psychosis associated with velo-cardio-facial syndrome: benefits and risks. J Intellect Disabil Res 2005; 49: 567-570
  • 26 Briegel W. Deletion 22q11.2 und schizophrene Störungen im Kindes- und Jugendalter. Z Kinder-Jugendpsychiatr Psychother 2007; 35: 353-358
  • 27 Krahn LE, Maraganore DM, Michels VV. Childhood-onset schizophrenia associated with parkinsonism in a patients with a microdeletion of chromosome 22. Mayo Clin Proc 1998; 73: 956-959
  • 28 Yacoub A, Aybar M. Response to clozapine in psychosis associated with velo-cardio-facial syndrome. Psychiatry 2007; 4: 14
  • 29 Gagliano A, Masi G. Clozapine-aripiprazole Association in a 7-year-old girl with schizophrenia: clinical efficacy and successful management of neutropenia with lithium. J Child Adolesc Psychiatry 2009; 19: 595-598
  • 30 Sporn A, Addington A, Reiss AL et al. 22q11 Deletion syndrome in childhood onset schizophrenia: an update. Mol Psychiatry 2004; 9: 225-226
  • 31 Müller UJ, Fellgiebel A. Succesful treatment of long-lasting psychosis in a case of 22q11.2 deletion syndrome. Pharmacopsychiatry 2008; 41: 158-159
  • 32 Lin CE, Hwang KS, Hsieh PH et al. Treatment of schizophreniform disorder by aripiprazole in female adolescent with 22q11.2 deletion syndrome. Prog Neuropsychopharmacol Biol Psychiatry 2010; 34: 1141-1143
  • 33 Carandang CG, Scholten MC. Metyrosine in psychosis associated with 22q11.2 deletion syndrome: case report. J Child Adolesc Psychiatry 2007; 17: 115-120