Universidade Federal do Ceará, Departamento de Clínica Médica, Núcleo de Desenvolvimento e Pesquisa de Medicamentos, Fortaleza CE, Brazil.
Universidade Federal de São Paulo, Departamento de Neurologia e Neurocirurgia/Ebserh, Setor de Investigações nas Doenças Neuromusculares, São Paulo SP, Brazil.
Arq Neuropsiquiatr. 2023 Aug;81(8):764-775. doi: 10.1055/s-0043-1771176. Epub 2023 Aug 30.
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% ( = 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.
在肌萎缩侧索硬化症（ALS）中观察到的神经精神现象谱很广泛，尚未完全了解。笑和哭的障碍是最常见的表现之一。本研究的目的是报告巴西神经病学学会组织的一项教育共识的结果，该共识旨在评估 ALS 患者笑和哭障碍的定义、现象学、诊断和管理。招募了 12 名巴西神经病学学会的成员 - 被认为是该领域的专家 - 来回答 12 个关于该主题的问题。在交换修订版后，编写了第一份草稿。2022 年 9 月 23 日在巴西福塔莱萨举行了一次面对面会议来讨论该草案。随后将修订后的版本通过电子邮件发送给巴西神经病学学会 ALS 科学部门的所有成员，并提交最终修订版进行发表。在 15 项联合研究和 3906 名患者中，ALS 患者的假性延髓情绪/病理性大笑和哭泣（PBA/PLC）的患病率为 27.4%（=1070），范围为 11.4%至 71%。延髓起病是一个危险因素，但目前评估包括额颞叶痴呆患者和非额颞叶痴呆患者在内的不同运动神经元疾病亚型之间患病率差异的研究有限。抗抑郁药和右美沙芬与奎尼丁的联合用药（巴西不可用）可能是治疗选择。该专家组承认当前文献中的多个空白，并强调需要进一步研究。
