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Pharmacotherapy for behavioural manifestations in frontotemporal dementia: An expert consensus from the European Reference Network for Rare Neurological Diseases (ERN-RND)

. 2024 Dec ; 31 (12) : e16446. [epub] 20241024

Language English Country England, Great Britain Media print-electronic

Document type Journal Article

BACKGROUND AND PURPOSE: Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by pervasive personality and behavioural disturbances with severe impact on patients and caregivers. In current clinical practice, treatment is based on nonpharmacological and pharmacological approaches. Unfortunately, trial-based evidence supporting symptomatic pharmacological treatment for the behavioural disturbances in FTD is scarce despite the significant burden this poses on the patients and caregivers. METHOD: The study examined drug management decisions for several behavioural disturbances in patients with FTD by 21 experts across European expert centres affiliated with the European Reference Network for Rare Neurological Diseases (ERN-RND). RESULTS: The study revealed the highest consensus on drug treatments for physical and verbal aggression, impulsivity and obsessive delusions. Antipsychotics (primarily quetiapine) were recommended for behaviours posing safety risks to both patients and caregivers (aggression, self-injury and self-harm) and nightly unrest. Selective serotonin reuptake inhibitors were recommended for perseverative somatic complaints, rigidity of thought, hyperphagia, loss of empathy and for impulsivity. Trazodone was specifically recommended for motor unrest, mirtazapine for nightly unrest, and bupropion and methylphenidate for apathy. Additionally, bupropion was strongly advised against in 10 out of the 14 behavioural symptoms, emphasizing a clear recommendation against its use in the majority of cases. CONCLUSIONS: The survey data can provide expert guidance that is helpful for healthcare professionals involved in the treatment of behavioural symptoms. Additionally, they offer insights that may inform prioritization and design of therapeutic studies, particularly for existing drugs targeting behavioural disturbances in FTD.

1st Department of Neurology St Anne´s University Hospital and Faculty of Medicine Masaryk University Brno Czech Republic

Centre for Rare Diseases and Institute of Medical Genetics and Applied Genomics University Hospital Tübingen Tübingen Germany

Centre for Rare Diseases University Hospital Tübingen Tübingen Germany

Centre of Excellence of Neurodegenerative Disease AP HP Pitié Salpêtrière Hospital Paris France

CIBERNED Network Centre for Biomedical Research in Neurodegenerative Diseases National Institute of Health Carlos 3 Madrid Spain

Clinical Investigation Centre for Neurosciences Institut du Cerveau Pitié Salpêtrière Hospital Paris France

Department of Biomedical and Neuromotor Science University of Bologna Bologna Italy

Department of Cognitive Neurology Referral Centre for Cognitive Neurology and Neurophysiology University Hospital Centre Zagreb Zagreb Croatia

Department of Medicine Universidad de Oviedo Oviedo Spain

Department of Neuorology Neurogenetics Clinic and Clinical Trial Unit Danish Dementia Research Centre Copenhagen University Hospital Rigshospitalet Copenhagen Denmark

Department of Neurodegenerative Disease Dementia Research Centre UCL Institute of Neurology London UK

Department of Neurology 3rd Faculty of Medicine Charles University and Thomayer University Hospital Prague Czech Republic

Department of Neurology and Alzheimer Centre Erasmus MC Erasmus MC University Medical Centre Rotterdam The Netherlands

Department of Neurology Cognitive Centre Ghent University Hospital Ghent Belgium

Department of Neurology Hospital Universitario Central de Asturias Oviedo Spain

Department of Neurology Institute of Memory and Alzheimer's Disease AP HP Pitié Salpêtrière Hospital Paris France

Department of Neurology LMU University Hospital LMU Munich Munich Germany

Department of Neurology Medical University Innsbruck Innsbruck Tyrol Austria

Department of Neurology Neurocentre Oulu University Hospital Oulu Finland

Department of Neurology RWTH Aachen University Aachen Germany

Department of Neurology University Hospital Leuven Leuven Belgium

Department of Neuroscience and Padua Neuroscience Centre University of Padua Padua Italy

German Centre for Neurodegenerative Diseases Munich Germany

Institute for Medical Genetics and Applied Genomics University of Tübingen Tübingen Germany

Instituto de Investigación Sanitaria del Principado de Asturias Oviedo Spain

IRCCS Istituto delle Scienze Neurologiche di Bologna Bologna Italy

Medicine and Psychiatry Department University of Cantabria Santander Spain

MRC Oulu University Hospital Oulu Finland

Munich Cluster for Systems Neurology Munich Germany

Neurology Service Marqués de Valdecilla University Hospital Institute for Research Marqués de Valdecilla Santander Cantabria Spain

Paris Brain Institute Institut du Cerveau ICM Inserm U1127 CNRS UMR 7225 AP HP Hôpital Pitié Salpêtrière Sorbonne Université Paris France

Reference Centre for Rare or Early Dementias IM2A Département de Neurologie AP HP Hôpital Pitié Salpêtrière Paris France

Research Unit of Clinical Medicine University of Oulu Oulu Finland

School of Medicine University of Zagreb Zagreb Croatia

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