对患有8型肌缩侧面硬化症的患者进行认知和行为随访
Cássia de Alcântara1, Marcelo Maroco Cruzeiro2, Marcondes Cavalcante França3
1Programa de Pós-Graduação em Neurociências da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
概括
肌缩侧面硬化8型 (ALS8) 显示慢慢的认知衰退并行运动退化. 大多数认知功能保持稳定,只有ALS8患者的语言表现略有下降.
科学领域:
- 神经学 神经学
- 神经科学是一个神经科学.
- 遗传学 遗传学 是一个
背景情况:
- 肌缩侧面硬化8型 (ALS8) 是一种罕见的家族性运动神经元疾病,与VAPB p.P56S突变有关.
- 有限的纵向数据存在于ALS8患者的认知和行为进展.
研究的目的:
- 研究ALS8.8患者认知功能和行为症状的纵向进展.
- 了解疾病的发展轨迹,超越运动和功能衰退.
主要方法:
- 一组23名ALS8患者 (12名男性) 接受了连续的神经心理评估.
- 随访持续时间在24至48个月之间,平均为33个月.
主要成果:
- 在随访期间观察到轻微的运动和功能衰退.
- 语言流,执行功能,情节性记忆或面部情绪识别没有显著变化.
- 在阿登布鲁克认知检查修订的语言子域中注意到了下降.
- 刻板印象行为减少,而焦虑和抑郁症状保持稳定.
- 在研究期间,没有患者患上痴呆症.
结论:
- 在ALS8的认知衰退进展缓慢,反映了运动退化.
- 语言功能显示微妙的下降,而其他认知领域保持相对稳定.
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