Cota Bruno Cézar Lage, Fonseca João Gabriel Marques, Rodrigues Luiz Oswaldo Carneiro, Rezende Nilton Alves de, Batista Pollyanna Barros, Riccardi Vincent Michael, Resende Luciana Macedo de
Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.
Universidade Federal de Minas Gerais, Faculdade de Medicina, Departamento de Clínica Médica, Belo Horizonte MG, Brasil.
Arq Neuropsiquiatr. 2018 May;76(5):287-295. doi: 10.1590/0004-282X20180031.
Auditory processing deficits are common in people with neurofibromatosis type 1 (NF1) and they often report difficulties in musical performance.
We investigated whether NF1 could be associated with amusia as well as with some impairment of primary auditory cortex activity.
Eighteen people with NF1 and 22 healthy volunteers, matched for age, sex and educational level, were evaluated with the Montreal Battery Evaluation of Amusia - short version. The integrity of cortical primary auditory processing areas was evaluated by evoked potential mismatch negativity.
Amusia was correlated with NF1 (p = 0.001, odds ratio = 42.0, confidence interval 4.5-39.6). Patients with NF1 exhibited a greater prevalence of amusia than healthy controls (67% vs. 4.5%) and difficulties in both melodic and temporal music perception. Worse performance on the Montreal Battery Evaluation of Amusia was correlated with a greater mismatch negativity latency in NF1 group.
Amusia is a common feature in NF1 and may result from impairment of activity in primary auditory processing areas.
听觉处理缺陷在1型神经纤维瘤病（NF1）患者中很常见，他们经常报告在音乐演奏方面存在困难。
我们研究了NF1是否与失乐感以及初级听觉皮层活动的某些损伤有关。
18名NF1患者和22名年龄、性别和教育水平相匹配的健康志愿者接受了蒙特利尔失乐感评估量表简版评估。通过诱发电位失匹配负波评估皮层初级听觉处理区域的完整性。
失乐感与NF1相关（p = 0.001，优势比 = 42.0，置信区间4.5 - 39.6）。NF1患者的失乐感患病率高于健康对照组（67% 对 4.5%），并且在旋律和节奏音乐感知方面都存在困难。在蒙特利尔失乐感评估量表上表现更差与NF1组中更长的失匹配负波潜伏期相关。
失乐感是NF1的常见特征，可能是由初级听觉处理区域的活动受损导致的。
