Pathological correlates of frontotemporal lobar degeneration in the elderly
Pathological correlates of frontotemporal lobar degeneration in the elderly
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DOI：
10.1007/s00401-010-0765-z
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发表时间：
2011-03-01
影响因子：
12.7
通讯作者：
Mann, David M. A.
中科院分区：
文献类型：
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作者：
Baborie, Atik;Griffiths, Timothy D.;Mann, David M. A.
额颞叶变性（FTLD）通常被认为是一种早老性发病（即65岁之前）的疾病，只有偶尔的病例出现在65岁之后。我们着手确定FTLD病例中迟发性疾病的比例，以及与早老性发病病例相比，FTLD病例是否具有独特的临床和神经病理学特征。在曼彻斯特和纽卡斯尔联合尸检系列的117例病理证实的FTLD（109/117例也符合隆德曼彻斯特FTLD临床标准）中，我们确定了30例（发病年龄范围65-86岁），占这两个中心25年内确定的所有FTLD病例的25%。30例老年患者的神经病理表现为几个FTLD组织学亚组的特征[FTLD-TDP（1、2和3型，19例（63%））、FLTD-tau [MAPT、PiD和CBD，10例（33%）]和FTLD-UPS（1例），表型范围与早老组相似，尽管患者有MAPT，但无PGRN、突变或FUS病理，在老年组中明显缺失或较少。海马硬化（HS）存在于13/30的老年FTLD病例（43%）与14/79（18%）的老年前期FTLD患者（P = 0.012）。大多数年轻患者存在的肺叶萎缩仅在25%的老年受试者中突出。前瞻性和回顾性精神病学和医学病例记录分析表明，大多数老年FTLD患者，像他们年轻的同行，有行为特征符合额颞叶痴呆。FTLD在老年人中很常见，在年轻人中存在的所有或大部分主要临床和组织学亚型都可以在老年人中看到。
Frontotemporal lobar degeneration (FTLD) is generally recognised as a disorder with presenile onset (that is before 65 years of age) with only occasional cases presenting later than this. We set out to determine what proportion of cases of FTLD had late onset of disease and whether such cases of FTLD had distinctive clinical and neuropathological features as compared to cases with presenile onset. Within a combined Manchester and Newcastle autopsy series of 117 cases with pathologically confirmed FTLD (109/117 cases also met Lund Manchester clinical criteria for FTLD), we identified 30 cases (onset age range 65-86 years), comprising 25% of all FTLD cases ascertained in these two centres over a 25-year period. Neuropathologically, the 30 elderly cases presented features of several FTLD histological subgroups [FTLD-TDP (types 1, 2 and 3, 19 cases (63%)], FLTD-tau [MAPT, PiD and CBD, 10 cases (33%)] and FTLD-UPS (1 case), similar in range of phenotypes to that seen in the presenile group, though patients with MAPT, but not PGRN, mutation, or FUS pathology, were notably absent or fewer in the elderly group. Hippocampal sclerosis (HS) was present in 13/30 of the elderly FTLD cases (43%) compared with 14/79 (18%) (P = 0.012) in the presenile FTLD patients. Lobar atrophy present in most of the younger patients was prominent in only 25% of the elderly subjects. Prospective and retrospective psychiatric and medical case note analysis showed that the majority of the elderly FTLD patients, like their younger counterparts, had behavioural features consistent with frontotemporal dementia. FTLD is common amongst elderly persons and all or most of the major clinical and histological subtypes present in younger individuals can be seen in the older group.