From the Department of Internal Medicine, Rehabilitation and Geriatrics (A.M.), Geneva University Hospitals and University of Geneva, Switzerland; Department of Neurology (A.M., S.E., B.D., M.T.), National Reference Center for PPA and Rare Dementias, Institute for Memory and Alzheimer's Disease, Pitié Salpêtrière Hospital, AP-HP, Paris; Sorbonne Universités (A.B., O.C., A.R., S.E., L.F., M.C., B.D., M.T.), UPMC University Paris 06, Inserm, CNRS, Institut du Cerveau et la Moelle, Paris; Inria Paris (A.B., O.C., A.R., S.E., T.K.), Aramis Project-Team, Paris; Departments of Neuroradiology (A.B.) and Metabolic Biochemistry (F.L.), Pitié Salpêtrière Hospital, AP-HP, Paris; Department of Neurology and Laboratory of Functional Neurosciences (EA 4559) (O.G.), University Hospital of Amiens; Department of Neurology (F.E.-B.), Memory Research and Resource Center for Alzheimer's Disease, University Hospital of Angers; Department of Psychiatry, Neurology and Rehabilitation (O. Moreaud), University Hospital of Grenoble, Memory Research and Resource Center for Alzheimer's Disease; Department of Neurology (F.P.), University Hospital of Lille; Department of Neurology (P.C.), University Hospital of Limoges; Department of Neurology (K.B.), Memory Research and Resource Center for Alzheimer's Disease, University Hospital of Montpellier; Department of Neurology (M.V.), University Hospital of Nantes; Department of Neurology (O. Martinaud), University Hospital of Rouen; Normandie University (O. Martinaud, S.B.), UNICAEN, EPHE, INSERM, U1077, Neuropsychologie et Imagerie de la Mémoire Humaine; Department of Neurology (B.L.), University Hospital of Saint-Etienne; Department of Neurology (J.P., M.P.), Pierre Paul Riquet Hospital, Toulouse; INSERM/UPS (J.P.), UMR 1214-ToNIC, Toulouse NeuroImaging Center, University of Toulouse III; and Department of Neurology (S.B.), Memory Research and Resource Center for Alzheimer's Disease, University Hospital Pontchaillou, Rennes, France.
Neurology. 2018 Mar 20;90(12):e1057-e1065. doi: 10.1212/WNL.0000000000005165. Epub 2018 Feb 14.
To reveal the prevalence and localization of cerebral microbleeds (CMBs) in the 3 main variants of primary progressive aphasia (PPA) (logopenic, semantic, and nonfluent/agrammatic), to identify the relationship with underlying Alzheimer pathology, and to explore whether CMBs contribute to language breakdown.
We used a cross-sectional design in a multicenter cohort of 82 patients with PPA and 19 similarly aged healthy controls. MRI allowed for rating CMBs (2-dimensional gradient recalled echo T2*, susceptibility weighted imaging sequences) and white matter hyperintensities. CSF Alzheimer disease biomarker analyses available in 63 of the 82 patients provided the stratification of PPA into subgroups with patients who had or did not have probable underlying Alzheimer pathology.
The prevalence of CMBs was higher in patients with PPA (28%) than in controls (16%). They were more prevalent in logopenic PPA (50%) than in semantic PPA (18%) and nonfluent/agrammatic PPA (17%). The localization of CMBs was mainly lobar (81%) with no difference between the PPA variants. CMBs were more frequent in PPA patients with positive than with negative CSF Alzheimer disease biomarkers (67% vs 20%). Patients with and without lobar CMBs had similar volumes of white matter hyperintensities. Language and general cognitive impairment in PPA was unrelated to CMB rates.
CMB prevalence in PPA is higher than in healthy controls. CMBs were most prevalent in the logopenic variant, were related to underlying Alzheimer pathology, and did not affect the language/cognitive impairment. Our findings also suggest that CMB detection with MRI contributes to PPA variant diagnosis, especially of logopenic PPA, and provides an estimator of the underlying neuropathology.
揭示主要 3 种原发性进行性失语症（PPA）变异型（流利型、语义型和非流利/语法障碍型）中脑微出血（CMBs）的流行率和定位，确定与潜在阿尔茨海默病病理学的关系，并探讨 CMB 是否导致语言障碍。
我们在 82 例 PPA 患者和 19 名年龄相似的健康对照者的多中心队列中采用横断面设计。MRI 可用于评定 CMB（二维梯度回波 T2*、磁敏感加权成像序列）和脑白质高信号。在 82 例患者中的 63 例可进行脑脊液阿尔茨海默病生物标志物分析，将 PPA 分层为有或无潜在阿尔茨海默病病理学的亚组。
PPA 患者 CMB 的患病率（28%）高于对照组（16%）。与语义性 PPA（18%）和非流利/语法障碍性 PPA（17%）相比，流畅性 PPA 患者 CMB 的患病率更高（50%）。CMBs 的定位主要为脑叶（81%），在 PPA 变异型之间无差异。与 CSF 阿尔茨海默病生物标志物阴性患者相比，阳性患者 CMB 的发生率更高（67%比 20%）。有无脑叶 CMB 的 PPA 患者脑白质高信号的体积相似。PPA 患者的语言和一般认知功能障碍与 CMB 发生率无关。
PPA 中 CMB 的患病率高于健康对照组。CMBs 在流畅性变异型中最常见，与潜在的阿尔茨海默病病理学有关，且不影响语言/认知障碍。我们的研究结果还表明，MRI 检测 CMB 有助于 PPA 变异型诊断，尤其是流畅性 PPA，并提供潜在神经病理学的估计值。
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