Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.
Alzheimer Dis Assoc Disord. 2011 Oct-Dec;25(4):364-8. doi: 10.1097/WAD.0b013e31820f8f50.
Hippocampal sclerosis (HpScl) in the elderly is often associated with neurodegeneration.
We studied the clinical and pathologic features of HpScl in 205 consecutive patients with dementia who came to autopsy from 1997 to 2008, focusing on associations with TAR DNA-binding protein 43 (TDP-43) pathology and allelic variants in the progranulin (GRN) and apolipoprotein E (APOE).
Of the 205 dementia patients, 28 had HpScl (14%). TDP-43 pathology was more frequent in cases with HpScl compared with those without HpScl (89% vs. 24%). GRN rs5848 T-allele but not APOE ε4 was associated with HpScl. In cases of HpScl with TDP-43 pathology and age of onset after 75 years (n=11), 8 had Alzheimer disease (AD)-like amnestic syndrome, but most (6 of 8) had pathology not consistent with AD (Braak stage III or less), including 4 with frontotemporal lobar degeneration with TDP, 1 with diffuse Lewy body disease, and 1 with "pure HpScl."
HpScl is common in an elderly cohort with dementia, occurring in 14% of the cases in this series, and 89% have TDP-43 pathology, often associated with a risk variant in GRN. Patients with HpScl who present after the age of 75 years often have presentations consistent with AD, but at autopsy have non-Alzheimer pathologies. Elderly patients with HpScl may be mistaken for AD.
老年人海马硬化（HpScl）常与神经退行性变有关。
我们研究了从 1997 年至 2008 年进行尸检的 205 例连续痴呆患者的 HpScl 的临床和病理特征，重点关注与 TAR DNA 结合蛋白 43（TDP-43）病理学以及颗粒蛋白前体（GRN）和载脂蛋白 E（APOE）等位基因变异的关联。
在 205 例痴呆患者中，有 28 例患有 HpScl（14%）。与无 HpScl 相比，有 HpScl 的病例中 TDP-43 病理学更为常见（89%比 24%）。GRN rs5848 T 等位基因而非 APOE ε4 与 HpScl 相关。在 HpScl 伴 TDP-43 病理学和 75 岁以后发病的病例（n=11）中，有 8 例为阿尔茨海默病（AD）样遗忘综合征，但大多数（8 例中的 6 例）的病理学不符合 AD（Braak Ⅲ期或以下），包括 4 例伴 TDP 的额颞叶变性、1 例弥漫性路易体病和 1 例“单纯性 HpScl”。
在老年痴呆患者队列中，HpScl 很常见，在本系列中占 14%，89%有 TDP-43 病理学，常与 GRN 的风险变异相关。75 岁以后发病的 HpScl 患者的表现常与 AD 一致，但尸检时则为非阿尔茨海默病的病理学。老年 HpScl 患者可能被误诊为 AD。
