Purpose

To determine the risk for dementia in neurofibromatosis type 1 (NF1) using a Finnish nationwide cohort of individuals with NF1, and data from national registries.

Methods

A Finnish cohort of 1,349 individuals with confirmed NF1 according to the US National Institutes of Health (NIH) diagnostic criteria was compared with a control cohort of 13,870 individuals matched for age, sex, and area of residence. Dementia-related hospital visits were retrieved from the Finnish Care Register for Health Care using International Classification of Diseases, 10th revision (ICD-10) diagnosis codes G30 and F00–F03. Purchases of antidementia drugs were queried with Anatomical Therapeutic Chemical (ATC) classification code N06D from the drug reimbursement register maintained by the Social Insurance Institution of Finland. The follow-up spanned 1998–2014.

Results

Totals of 16 and 165 individuals with at least two dementia-related diagnoses or drug purchases were identified in the NF1 and control cohorts, respectively. The hazard ratio for dementia in NF1 was 1.67 (95% confidence interval [CI] 1.00–2.80, P = 0.050). In an analysis stratified by the type of dementia, the risk for Alzheimer disease was increased in NF1 compared to controls with a hazard ratio of 2.88 (95% CI 1.47–5.66, P = 0.002).

Conclusion

Dementia and especially Alzheimer disease are previously unrecognized neurological complications of NF1.

中文翻译：

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1型神经纤维瘤病患痴呆症的风险增加

目的

使用芬兰全国 NF1 患者队列和来自国家登记处的数据确定 1 型神经纤维瘤病 (NF1) 痴呆的风险。

方法

根据美国国立卫生研究院 (NIH) 的诊断标准，将 1,349 名确诊为 NF1 的芬兰队列与年龄、性别和居住区域匹配的 13,870 名对照队列进行了比较。使用国际疾病分类第 10 版 (ICD-10) 诊断代码 G30 和 F00-F03 从芬兰医疗保健登记册中检索与痴呆症相关的医院就诊。使用芬兰社会保险机构维护的药物报销登记册中的解剖治疗化学 (ATC) 分类代码 N06D 查询购买的抗痴呆药物。随访时间跨度为 1998 年至 2014 年。

结果

在 NF1 和对照组中，分别确定了 16 和 165 名至少有两次痴呆相关诊断或购买药物的人。NF1 痴呆的风险比为 1.67（95% 置信区间 [CI] 1.00–2.80，P  = 0.050）。在按痴呆类型分层的分析中，与对照组相比，NF1 患阿尔茨海默病的风险增加，风险比为 2.88（95% CI 1.47-5.66，P  = 0.002）。

结论

痴呆症，尤其是阿尔茨海默病是以前未被认识的 NF1 神经系统并发症。