Definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in amyotrophic lateral sclerosis (ALS): Consensus from ALS and Motor Neuron Disease Scientific Department of the Brazilian Academy of Neurology

Abstract

The spectrum of neuropsychiatric phenomena observed in amyotrophic lateral sclerosis (ALS) is wide and not fully understood. Disorders of laughter and crying stand among the most common manifestations. The aim of this study is to report the results of an educational consensus organized by the Brazilian Academy of Neurology to evaluate the definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in ALS patients. Twelve members of the Brazilian Academy of Neurology - considered to be experts in the field - were recruited to answer 12 questions about the subject. After exchanging revisions, a first draft was prepared. A face-to-face meeting was held in Fortaleza, Brazil on 9.23.22 to discuss it. The revised version was subsequently emailed to all members of the ALS Scientific Department from the Brazilian Academy of Neurology and the final revised version submitted for publication. The prevalence of pseudobulbar affect/pathological laughter and crying (PBA/PLC) in ALS patients from 15 combined studies and 3906 patients was 27.4% (N = 1070), ranging from 11.4% to 71%. Bulbar onset is a risk factor but there are limited studies evaluating the differences in prevalence among the different motor neuron diseases subtypes, including patients with and without frontotemporal dementia. Antidepressants and a combination of dextromethorphan and quinidine (not available in Brazil) are possible therapeutic options. This group of panelists acknowledge the multiple gaps in the current literature and reinforces the need for further studies.

Resumo

O espectro de fenômenos neuropsiquiátricos observados na ELA é amplo e não completamente entendido. Desordens do riso e do choro estão entre as manifestações mais comuns. O objetivo deste estudo é relatar os resultados de um Consenso organizado pela Academia Brasileira de Neurologia para avaliar definições, fenomenologia, diagnóstico, e manejo dos distúrbios do riso e do choro em pacientes com ELA. Doze membros da Academia Brasileira de Neurologia – considerados experts na área – foram recrutados para responder 12 questões na temática. Depois da verificação das revisões, um primeiro manuscrito foi preparado. Após, foi realizado um encontro presencial em Fortaleza, Brasil, em 23/09/2022, para discussão do conteúdo. A versão revisada foi posteriormente enviada por e-mail para todos os membros do Departamento Científico de DNM/ELA da Academia Brasileira de Neurologia e a versão final revisada foi submetida para publicação. A prevalência da síndrome pseudobulbar em pacientes com ELA em 15 estudos combinados com 3906 pacientes foi de 27,4% (n = 1070), variando entre 11,4% e 71%. Início bulbar é um fator de risco, mas há limitados estudos avaliando as diferenças em prevalência entre os diferentes subtipos de Doença do Neurônio Motor, incluindo pacientes com e sem Demência Frontotemporal. Antidepressivos e uma combinação de dextrometorfana e quinidina (indisponíveis no Brasil) são opções terapêuticas possíveis. Esse grupo de panelistas reconhece as múltiplas demandas não atendidas na literatura atual e reforça a necessidade de futuros estudos.

Keywords

Amyotrophic Lateral Sclerosis - Emotional Incontinence - Pathological Laughter and Crying - Pseudobulbar Affect - Affective Symptoms - Emotional Regulation - Affect

Palavras-chave

esclerose lateral amiotrófica - incontinência emocional - desordens do riso e do choro - afeto pseudobulbar - distúrbios do afeto - regulação emocional - afeto

Authors' Contributions

FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: conceptualization; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: data curation: FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: formal analysis; FAAG: funding acquisition: FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: investigation; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: methodology; FAAG, WBVRP: project administration; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: resources: FAAG, WBVRP: software; FAAG: supervision; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: validation; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: visualization; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: writing – original draft; FAAG, WBVRP, MATC, CCC, FMBC, METD, MCFJ, WMJ, ASBO, CLR, DJS, EDT: writing – review & editing.

On behalf of the Scientific Department of Motor Neuron Disease/ALS of the Brazilian Academy of Neurology.

Publikationsverlauf

Eingereicht: 19. Februar 2023

Angenommen: 28. März 2023

Artikel online veröffentlicht:
30. August 2023

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