极早发型散发性阿尔茨海默病诊断前初始症状及护理路径的特征分析

Characterization of the initial complaint and care pathways prior to diagnosis in very young sporadic Alzheimer's disease.

作者信息

Olivieri Pauline, Hamelin Lorraine, Lagarde Julien, Hahn Valérie, Guichart-Gomez Elodie, Roué-Jagot Carole, Sarazin Marie

机构信息

Department of Neurology of Memory and Language, GHU Paris Psychiatry and Neurosciences, Hôpital Sainte Anne, 1 rue Cabanis, F-75014, Paris, France.

Université de Paris, F-75006, Paris, France.

出版信息

Alzheimers Res Ther. 2021 Apr 29;13(1):90. doi: 10.1186/s13195-021-00829-0.

DOI:10.1186/s13195-021-00829-0

PMID:33926533

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8086269/

Abstract

BACKGROUND

Very-early-onset Alzheimer's disease (young-AD) differentiates from late-onset AD (old-AD) by a predominant involvement of the parietal neocortex leading to atypical presentations. The diagnosis of AD is often not the first to be mentioned in such young patients.

METHODS

We retrospectively reviewed the initial complaint and care pathways of 66 sporadic young-AD (age < 62) and 30 old-AD patients (age > 65) and compared their neuropsychological profiles at the time of diagnosis (based on clinical-biological criteria) with 44 amyloid-negative controls.

RESULTS

The initial complaint of young-AD was non-cognitive and mimicked a burnout in 32% of cases. Their main cognitive complaints were memory (38% vs 87% in old-AD) and language (17% vs 13%) impairment. The referral to a psychiatrist prior to AD diagnosis was more frequent in young-AD than in old-AD (26% vs 0%). At the time of diagnosis, young-AD were at a more severe stage of dementia than old-AD (24% vs 10% with CDR ≥ 1) but had less anosognosia.

CONCLUSIONS

Better identifying the initial signs of very-early-onset AD is crucial to improve the early diagnosis and develop new treatments.

摘要

背景

极早发型阿尔茨海默病（早发型AD）与晚发型AD（晚发型AD）不同，其主要累及顶叶新皮质，导致非典型表现。在这类年轻患者中，AD的诊断往往并非首先被提及。

方法

我们回顾性分析了66例散发性早发型AD（年龄<62岁）和30例晚发型AD患者（年龄>65岁）的初始症状和诊疗路径，并将他们诊断时（基于临床生物学标准）的神经心理学特征与44例淀粉样蛋白阴性对照进行比较。

结果

早发型AD的初始症状为非认知性，32%的病例类似职业倦怠。他们主要的认知症状是记忆障碍（38%，晚发型AD为87%）和语言障碍（17%，晚发型AD为13%）。早发型AD在AD诊断前转诊至精神科医生的频率高于晚发型AD（26%比0%）。诊断时，早发型AD的痴呆程度比晚发型AD更严重（CDR≥1者分别为24%和10%），但失认症较少。