Bertrand Anne, Stroër Sebastian, Le Ber Isabelle, Teichmann Marc, Dormont Didier
Service de neuroradiologie, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France, Sorbonne Universités, UPMC Université Paris 06, Inserm, CNRS, Institut du cerveau et la moelle (ICM), Hôpital Pitié-Salpêtrière, AP-HP, Paris, France, Inria Paris, Aramis project-team, Paris, France.
Sorbonne Universités, UPMC Université Paris 06, Inserm, CNRS, Institut du cerveau et la moelle (ICM), Hôpital Pitié-Salpêtrière, AP-HP, Paris, France, Département de neurologie, Institut de la mémoire et de la maladie d'Alzheimer, Centre national de référence « Démences rares », Hôpital Pitié-Salpêtrière, AP-HP, Paris, France, Département de génétique et cytogénétique, Unité fonctionnelle de génétique clinique, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France.
Geriatr Psychol Neuropsychiatr Vieil. 2017 Sep 1;15(3):285-294. doi: 10.1684/pnv.2017.0686.
Frontotemporal lobar dementia (FTLD) is a heterogeneous group of neurodegenerative diseases. FTLD encompass: 1) behavioral forms, sometimes associated with amyotrophic lateral sclerosis; 2) linguistic forms (semantic and non-fluent primary progressive aphasia); 3) atypical parkinsonian syndromes (progressive supranuclear palsy and corticobasal syndrome). Standard brain MRI allows for strengthening the clinical suspicion of FTLD, by showing a pattern of atrophy in relation with the patient's clinical symptoms: frontotemporal anterior atrophy in behavioral forms; temporopolar or inferior left frontal atrophy in the linguistic forms; mesencephalic or corticosubcortical hemispheric atrophy in forms with atypical pakinsonism. MRI is now part of the diagnostic criteria of some FTLD (behavioral FTLD, primary progressive aphasia). Genetic forms are common in FTLD, especially in behavioral FTLD. The three main mutations (C9ORF72, GRN and MAPT) are associated with different imaging patterns, which can thus orient the clinician towards a particular mutation in a patient with a familial form of FTLD.
额颞叶痴呆（FTLD）是一组异质性神经退行性疾病。FTLD包括：1）行为型，有时与肌萎缩侧索硬化相关；2）语言型（语义性和非流利性原发性进行性失语）；3）非典型帕金森综合征（进行性核上性麻痹和皮质基底节综合征）。标准脑部MRI通过显示与患者临床症状相关的萎缩模式，有助于增强对FTLD的临床怀疑：行为型表现为额颞叶前部萎缩；语言型表现为颞极或左侧额叶下部萎缩；非典型帕金森型表现为中脑或皮质下皮质半球萎缩。MRI现在是一些FTLD（行为性FTLD、原发性进行性失语）诊断标准的一部分。遗传形式在FTLD中很常见，尤其是在行为性FTLD中。三个主要突变（C9ORF72、GRN和MAPT）与不同的影像学模式相关，因此可以指导临床医生对家族性FTLD患者的特定突变进行判断。
