ABSTRACT
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.
ABSTRACT
Pseudobulbar affect (PBA) is a neurological symptom of inappropriate and uncontrollable laughter or crying that occurs secondary to a variety of neurological conditions, including parkinsonian disorders. PBA is a socially and emotionally debilitating symptom that has been estimated to affect 3.6% to 42.5% of the population with Parkinson’s disease. While indexing measures and treatment options for PBA have been extensively studied in neurological conditions such as amyotrophic lateral sclerosis and multiple sclerosis, there has been considerably less attention given in the literature to PBA in parkinsonian disorders. The purpose of this review is to discuss the pathophysiology of PBA, its prevalence and impact on quality of life in parkinsonian disorders, and the treatment options currently available. Areas requiring further study, including the development of standardized, cross-culturally validated methods of symptom assessment, and evidence-based studies exploring the efficacy of current treatment options in parkinsonian disorders, are also highlighted.
Subject(s)
Abstracting and Indexing , Amyotrophic Lateral Sclerosis , Crying , Laughter , Multiple Sclerosis , Parkinson Disease , Parkinsonian Disorders , Prevalence , Quality of Life , Symptom AssessmentABSTRACT
Objective To evaluate the clinical effect of dextromethorphan and its effect on daily living of patients with poststroke pseudobulbar affect. Methods Sixty patients with poststroke pseudobulbar affect admitted in our hospital from May 2013 to October 2016 were enrolled. Then they were randomly divided into the control group and the treatment group,with 30 patients in each group.Patients in the control group were treated with fluox-etine therapy and patients in the treatment group were treated with dextromethorphan therapy.The center for neuro-logic study lability scale(CNS-LS)and activity of daily living(Barthel index,BI)before and 30 days after the treat-ments in the two groups had been accessed. Results Thirty days after the treatment,CNS-LS of the treatment group had obvious improvement compared with that before treatment(P < 0.01),but CNS-LS of the control group had no obvious improvement compared with that before treatment(P > 0.05). And significant improvement has been found 30 days after the treatment between the two groups(P<0.01).Furthermore,significant difference was found on BI between these two groups(P<0.05).Conclusions Dextromethorphan is effective in treatment of pa-tients with poststroke pseudobulbar affect and it can improve the activity of daily living of these patients.
ABSTRACT
Pathological laughter, pseudobulbar affect or a myriad of its other synonyms, is a condition common secondary to neurological injury, presenting with uncontrolled laughing or crying, appearing to be mood-incongruent and significantly debilitating. The objective of this case report is to highlight a rare case of pathological laughter associated with Cerebral Lupus. Methods: We report a case of a 27-year-old lady presenting with signs and symptoms of stroke with dysarthria progressing to aphasia and then pathological laughter, with an underlying poorly controlled Systemic Lupus Erythematosus, manifesting as Cerebral Lupus. Results: An improvement in affect congruency followed by decreased frequency of outbreaks among bouts of pathological laughter. Conclusion: The combination of Escitalopram and Topiramate is effective in the symptomatic treatment of Pathological Laughter. ASEAN Journal of Psychiatry, Vol. 18 (1): January – June 2017: XX XX.