Expanding the phenotype of NEDAMSS with a psychiatric perspective: analysis of a new case, and a systematic review of the literature.

Pathogenic variants in the IRF2BPL gene are associated with neurodevelopmental disorders with varying degrees of regression, loss of speech and epilepsy. The phenotype is also known as Neurodevelopmental Disorder with regression, Abnormal Movements, loss of Speech, and Seizures (NEDAMSS). The motor...

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Publicado en:European Child & Adolescent Psychiatry Vol. 34; no. 3; pp. 835 - 853
Autores principales: Kristiansen, Kimmie, Vernal, Ditte Lammers, Hulgaard, Ditte Roth
Formato: case study research systematic review tables/charts Journal Article
Publicado: Springer Nature Mar2025
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Mar2025
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      pub: Springer Nature
      place: New York, New York
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        10.1007/s00787-024-02522-7
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        atl: Expanding the phenotype of NEDAMSS with a psychiatric perspective: analysis of a new case, and a systematic review of the literature.
      aug:
        au:
          Kristiansen, Kimmie
          Vernal, Ditte Lammers
          Hulgaard, Ditte Roth
        affil: Mental Health Services in the Region of Southern Denmark, Child- and Adolescent Psychiatry, Esbjerg, Denmark
      sug:
        subj:
          Phenotype
          Catatonia
          Mental Disorders
          Dyskinesias
          Speech Disorders
          Seizures
          Health Knowledge
          Human
          Systematic Review
          Descriptive Statistics
          Data Analysis Software
          PubMed
          Male
          Adolescence
          Magnetic Resonance Imaging
          Adolescent: 13-18 years
          Male
      ab: Pathogenic variants in the IRF2BPL gene are associated with neurodevelopmental disorders with varying degrees of regression, loss of speech and epilepsy. The phenotype is also known as Neurodevelopmental Disorder with regression, Abnormal Movements, loss of Speech, and Seizures (NEDAMSS). The motor symptoms of this disorder share significant phenotypical characteristics with catatonia, a severe neuropsychiatric psychomotor syndrome. The objective of this article is to expand the knowledge on the presentation of NEDAMSS with a focus on psychiatric symptoms including catatonia. A systematic review of 32 case presentations of NEDAMSS, and a novel case report of a patient with NEDAMSS, exhibiting multiple psychiatric symptoms, including catatonia are presented. Psychiatric symptoms and disorders including affective disorders, psychotic symptoms, catatonia, and developmental disorders are reported in one third of the reviewed cases. Reported effects of pharmacological treatment on motor symptoms of NEDAMSS are very limited. Our case presents improvement in motor symptoms originally attributed to NEDAMSS, after treatment with Lorazepam following diagnosis with catatonia. Patients with NEDAMSS may present with both neurological and psychiatric symptoms. The clinical presentation of NEDAMSS motor symptoms and catatonia have similarities and thus poses significant challenges to the diagnostic process, with risk of incorrect or delayed treatment. The limited experience and the complex phenotype of NEDAMSS complicates pharmacological treatment and encourages caution, especially with the use of antipsychotic drugs in the presence of possible catatonic symptoms.
      pubtype: Academic Journal
      doctype:
        case study
        research
        systematic review
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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