Pathological correlates of frontotemporal lobar degeneration in the elderly
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Atik Baborie Timothy D. Griffiths Evelyn Jaros Ian G. McKeith David J. Burn Anna ichardson Raffaele Ferrari Jorge Moreno Parastoo Momen
- چاپ و سال / کشور: 2010
Description
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 determinewhat 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 alsomet 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 themajor clinical and histological subtypes present in younger individuals can be seen in the older group.
Acta Neuropathol (2011) 121:365–371 DOI 10.1007/s00401-010-0765-z Received: 7 September 2010 / Revised: 18 October 2010 / Accepted: 19 October 2010 / Published online: 27 October 2010
