Sorbonne Université, INSERM, Institut Pierre Louis d'Épidémiologie et de Santé Publique, Paris, France.
Département de Neurologie, Centre de référence CERVCO, Hôpital Lariboisière, AP-HP, Université Paris 7 Denis Diderot, Paris, France.
J Alzheimers Dis. 2020;77(1):291-300. doi: 10.3233/JAD-200310.
For developing future clinical trials in Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), it seems crucial to study the long-term changes of cognition.
We aimed to study the global trajectory of cognition, measured by the Mini-Mental State Examination (MMSE) and the Mattis Dementia Rating Scale (MDRS), along the course of CADASIL.
Follow-up data of 185 CADASIL patients, investigated at the French National Referral center CERVCO from 2003, were considered for analysis based on strict inclusion criteria. Assuming that the MMSE and the MDRS provide imprecise measures of cognition, the trajectory of a common cognitive latent process during follow-up was delineated using a multivariate latent process mixed model. After adjustment of this model for sex and education, the sensitivities of the two scales to cognitive change were compared.
Analysis of the cognitive trajectory over a time frame of 60 years of age showed a decrease of performances with aging, especially after age of 50 years. This decline was not altered by sex or education but patients who graduated from high school had a higher mean cognitive level at baseline. The sensitivities of MMSE and MDRS scales were similar and the two scales suffered from a ceiling effect and curvilinearity.
These data support that cognitive decline is not linear and mainly occurs after the age of 50 years during the course of CADASIL. They also showed that MMSE and MDRS scales are hampered by major limitations for longitudinal studies.
为了在脑常染色体显性动脉病伴皮质下梗死和白质脑病（CADASIL）中开展未来的临床试验，研究认知的长期变化似乎至关重要。
我们旨在研究通过简易精神状态检查（MMSE）和 Mattis 痴呆评定量表（MDRS）测量的认知的总体轨迹，沿着 CADASIL 的病程进行研究。
根据严格的纳入标准，对 2003 年从法国国家转诊中心 CERVCO 接受调查的 185 例 CADASIL 患者的随访数据进行了分析。假设 MMSE 和 MDRS 提供了认知的不精确测量，使用多变量潜在过程混合模型描绘了随访期间共同认知潜在过程的轨迹。在该模型中对性别和教育进行调整后，比较了这两个量表对认知变化的敏感性。
对 60 年时间框架内认知轨迹的分析表明，随着年龄的增长认知表现下降，尤其是在 50 岁以后。这种下降不受性别或教育的影响，但高中毕业生在基线时具有更高的平均认知水平。MMSE 和 MDRS 量表的敏感性相似，这两个量表都受到天花板效应和曲线性的限制。
这些数据支持认知下降不是线性的，并且主要发生在 CADASIL 病程中的 50 岁以后。它们还表明，MMSE 和 MDRS 量表在纵向研究中受到重大限制。
