Cognitive decline and behavioral changes are common features in amyotrophic lateral sclerosis (ALS) and imply worse prognosis as well as increased disease burden for patients and caregivers. Currently, there is a lack of studies regarding behavioral profile in Brazilian ALS cohorts. We assessed the prevalence and profile of behavioral impairment (ALSbi) in a Brazilian non-demented C9orf72-negative ALS cohort according to broad behavioral assessment and the latest consensus. Among 76 initially recruited consecutive ALS patients, 70 were included, including seven ALS type 8 (VAPB-related ALS) individuals. Patients with Frontotemporal Dementia (FTD) diagnosis were excluded. Sixteen ALS patients (23%) were diagnosed as ALSbi. Among ALS type 8 individuals, 2 (28.6%) were diagnosed as ALSbi. Neuropsychiatric Inventory Questionnaire (NPI) total scores did positively correlate with age, but not with other demographic or clinical data. Apathy was the most prevalent finding in the ALSbi subgroup, although the prevalence (20%) was smaller than reported in previous literature. Dysphoria and anxiety were also prevalent findings in the whole ALS cohort. Future studies with larger cohorts and validated ALS-specific tools are needed in order to expand our knowledge.

中文翻译：

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巴西非痴呆C9orf72阴性ALS人群的行为表现。

认知能力下降和行为改变是肌萎缩性侧索硬化症（ALS）的常见特征，预后较差，患者和护理人员的疾病负担增加。当前，在巴西ALS人群中缺乏关于行为特征的研究。根据广泛的行为评估和最新共识，我们评估了巴西非痴呆C9orf72阴性ALS队列中行为障碍（ALSbi）的普遍性和概况。在最初招募的76位连续ALS患者中，包括70位，包括7位ALS 8型（VAPB相关ALS）患者。排除额颞痴呆（FTD）患者。16名ALS患者（占23％）被诊断为ALSbi。在ALS 8型个体中，有2名（28.6％）被诊断为ALSbi。神经精神病学问卷调查表（NPI）总分与年龄呈正相关，但与其他人口统计学或临床数据无正相关。冷漠是ALSbi亚组中最普遍的发现，尽管患病率（20％）比以前的文献报道的要小。在整个ALS队列中，烦躁不安和焦虑症也很普遍。为了扩展我们的知识，需要对更大的队列和经过验证的ALS专用工具进行进一步的研究。