Department of Public Health and Environmental Medicine, The Jikei University School of Medicine.
The Jikei University School of Medicine.
Environ Health Prev Med. 2023;28:46. doi: 10.1265/ehpm.22-00222.
For patients with neurofibromatosis type 2 (NF2), maintaining an independent state of living is important. The present study aimed to examine the loss of social independence (i.e., a status that patients can work and go to school) and its contributing factors in patients with NF2 using data from a national registry in Japan.
This longitudinal study used a registry database containing information on patients with NF2 who had submitted initial claims to receive medical expense subsidies between 2004 and 2010. Patients with "employed," "studying," and "housekeeping" categories were classified as "socially independent." Patients who were socially independent at baseline were followed-up for up to nine years. The primary outcome of the present study was the loss of social independence during the follow-up period, which was defined as the change in status from being socially independent to socially dependent. First, we examined longitudinal associations between demographic variables and neurological symptoms at baseline and the loss of social independence. Second, we examined whether the occurrence of neurological symptoms is associated with a loss of social independence in patients.
A total of 156 patients were included in the present study. During the follow-up period, 37 (23.7%) patients experienced a loss of social independence. In the first analysis, the multivariate logistic regression model showed that the loss of social independence was significantly more frequent among patients with spinal dysfunction than among patients without. In the second analysis, logistic regression analyses showed that neurological symptoms, including bilateral hearing loss, facial nerve palsy, cerebellar dysfunction, decreased facial sensation, speech dysfunction (dysphagia/dysarthria and aphasia), double vision, blindness, hemiparesis, and seizures, were significantly associated with loss of social independence.
The occurrence of various neurological symptoms of NF2 can hinder social independence in the long term. Medical service providers need to observe patients while considering the risks, and provide appropriate support to address neurological symptoms that can restrict social independence, as this will lead to maintaining social engagement.
对于神经纤维瘤病 2 型（NF2）患者而言，保持独立的生活状态非常重要。本研究旨在利用日本全国登记处的数据，对 NF2 患者丧失社会独立性（即患者能够工作和上学的状态）及其相关因素进行调查。
本纵向研究使用了一个登记处数据库，其中包含了 2004 年至 2010 年间提交初始医疗费用补贴申请的 NF2 患者的信息。将处于“就业”、“学习”和“操持家务”类别的患者归类为“社会独立”。在基线时处于社会独立状态的患者随访了长达九年的时间。本研究的主要结局是在随访期间丧失社会独立性，定义为从社会独立状态变为社会依赖状态的变化。首先，我们检查了基线时人口统计学变量和神经学症状与丧失社会独立性之间的纵向关联。其次，我们检查了神经学症状的发生是否与患者社会独立性的丧失有关。
本研究共纳入 156 例患者。在随访期间，37 例（23.7%）患者丧失了社会独立性。在第一项分析中，多变量逻辑回归模型显示，脊柱功能障碍患者丧失社会独立性的频率明显高于无脊柱功能障碍患者。在第二项分析中，逻辑回归分析显示，双侧听力损失、面神经瘫痪、小脑功能障碍、面部感觉减退、言语功能障碍（吞咽困难/构音障碍和失语症）、复视、失明、偏瘫和癫痫等神经学症状与丧失社会独立性显著相关。
NF2 的各种神经学症状的发生可能会长期阻碍社会独立性。医疗服务提供者在考虑风险的同时需要观察患者，并提供适当的支持以解决限制社会独立性的神经学症状，从而维持社会参与度。
