Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach

Introduction. Cognitive and behavioural problems associated with Neurofibromatosis type 1 (NF1) are common sources of distress and the reasons behind seeking help. Here we describe patients with NF1 or NF1-like phenotypes referred to a Tier 3 Child and Adolescent Psychiatry Department and highlight...

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Main Authors: Ana Moscoso, Aurélie Julien, Antoine Tanet, Angèle Consoli, Martine Pagnard, France Trevisan, Isabelle Kemlin, Diana Rodriguez, David Cohen
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Psychiatry
Online Access:http://dx.doi.org/10.1155/2019/4764031
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author Ana Moscoso
Aurélie Julien
Antoine Tanet
Angèle Consoli
Martine Pagnard
France Trevisan
Isabelle Kemlin
Diana Rodriguez
David Cohen
author_facet Ana Moscoso
Aurélie Julien
Antoine Tanet
Angèle Consoli
Martine Pagnard
France Trevisan
Isabelle Kemlin
Diana Rodriguez
David Cohen
author_sort Ana Moscoso
collection DOAJ
description Introduction. Cognitive and behavioural problems associated with Neurofibromatosis type 1 (NF1) are common sources of distress and the reasons behind seeking help. Here we describe patients with NF1 or NF1-like phenotypes referred to a Tier 3 Child and Adolescent Psychiatry Department and highlight the benefits of a multidisciplinary assessment. Methods. Prospective data were gathered from NF1 patients aged 7–15 years, referred by the NF1 Referral Centre due to additional difficulties either in management or diagnosis. For the selected cases, we performed a psychiatric assessment, a tailored neuropsychological evaluation based on clinical demands and history, broad speech and motor skills evaluations if there were concerns regarding language, motor abilities and/or learning difficulties and autism specific evaluations, if clinically relevant. No exclusion criteria were applied. Results. Complex NF1 cases represented only 5% of the patients (11/224). Assessments revealed the complexity of NF1 phenotype and a variety of problems including learning difficulties, emotional problems and autism spectrum disorders. Specific evaluations of language, motor, attentional and neurovisual domains were essential to guide tailored intervention strategies. Conclusions. In terms of clinical implications, the heterogeneity of NF1 phenotypical manifestations needs to be considered when developing assessment and remediation approaches for children with complex NF1.
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spelling doaj-art-3d26c86855e744f38e50bc7031caf33d2025-02-03T05:59:56ZengWileyCase Reports in Psychiatry2090-682X2090-68382019-01-01201910.1155/2019/47640314764031Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional ApproachAna Moscoso0Aurélie Julien1Antoine Tanet2Angèle Consoli3Martine Pagnard4France Trevisan5Isabelle Kemlin6Diana Rodriguez7David Cohen8Department of Child and Adolescent Psychiatry, Robert Debré Hospital, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceCentre de Référence des Neurofibromatoses et Service de Neurologie Pédiatrique, AP-HP, Hôpital Armand Trousseau, GHUEP, Paris, FranceCentre de Référence des Neurofibromatoses et Service de Neurologie Pédiatrique, AP-HP, Hôpital Armand Trousseau, GHUEP, Paris, FranceDepartment of Child and Adolescent Psychiatry, Reference Center for Rare Psychiatric Diseases, APHP, Groupe Hospitalier Pitié-Salpêtrière, Université Sorbonne, Paris, FranceIntroduction. Cognitive and behavioural problems associated with Neurofibromatosis type 1 (NF1) are common sources of distress and the reasons behind seeking help. Here we describe patients with NF1 or NF1-like phenotypes referred to a Tier 3 Child and Adolescent Psychiatry Department and highlight the benefits of a multidisciplinary assessment. Methods. Prospective data were gathered from NF1 patients aged 7–15 years, referred by the NF1 Referral Centre due to additional difficulties either in management or diagnosis. For the selected cases, we performed a psychiatric assessment, a tailored neuropsychological evaluation based on clinical demands and history, broad speech and motor skills evaluations if there were concerns regarding language, motor abilities and/or learning difficulties and autism specific evaluations, if clinically relevant. No exclusion criteria were applied. Results. Complex NF1 cases represented only 5% of the patients (11/224). Assessments revealed the complexity of NF1 phenotype and a variety of problems including learning difficulties, emotional problems and autism spectrum disorders. Specific evaluations of language, motor, attentional and neurovisual domains were essential to guide tailored intervention strategies. Conclusions. In terms of clinical implications, the heterogeneity of NF1 phenotypical manifestations needs to be considered when developing assessment and remediation approaches for children with complex NF1.http://dx.doi.org/10.1155/2019/4764031
spellingShingle Ana Moscoso
Aurélie Julien
Antoine Tanet
Angèle Consoli
Martine Pagnard
France Trevisan
Isabelle Kemlin
Diana Rodriguez
David Cohen
Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
Case Reports in Psychiatry
title Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
title_full Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
title_fullStr Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
title_full_unstemmed Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
title_short Clinical Management of Children and Adolescents with Neurofibromatosis Type 1 Like Phenotypes and Complex Behavioural Manifestations: A Multidisciplinary and Dimensional Approach
title_sort clinical management of children and adolescents with neurofibromatosis type 1 like phenotypes and complex behavioural manifestations a multidisciplinary and dimensional approach
url http://dx.doi.org/10.1155/2019/4764031
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