Hippocampal sclerosis dementia differs from hippocampal sclerosis in frontal lobe degeneration

Abstract

Hippocampal sclerosis (HS) is characterized by selective neuronal loss and gliosis in CA1 and the subiculum and has been associated with several disorders, including Alzheimer's disease, frontotemporal lobar degeneration with ubiquitin immunoreactive inclusions (FTLD-U), vascular dementia and some tauopathies. In some cases, HS is not associated with other degenerative pathologies. Such cases are sometimes referred to as HS dementia (HSD). DiVerences between HSD and HS in the setting of FTLD-U have not been systematically investigated. To this end, eight cases of HSD and ten cases of HS associated with FTLD-U were studied with Nissl and periodic acidSchiV stains to assess neuronal loss and corpora amylacea, respectively. Sections were immunostained with antibodies to glial Wbrillary acidic protein, HLA-DR and synaptophysin and immunoreactivity was measured with image analysis in CA1 and the subiculum of each case. Additionally, sections were immunostained with antibodies to 4-R tau to determine the presence of argyrophilic grains. HSD was diVerent from HS associated with FTLD-U. SpeciWcally, it was more common in the elderly, and it was associated with more marked neuronal and synaptic loss and with greater reactive gliosis. Corpora amylacea tended to be more frequent in HSD than in FTLD-U, but there was no diVerence in frequency of argyrophilic grains.

Hippocampal Sclerosis and Epilepsy in Elderly Population

“…Noteworthy, TDP43-pathology has been reported to be relatively common in AD [4][5][6][7][8][9][10][11][12][13][14] and in line with this, TDP43-pathology was observed in as many as 88% of our cases with severe AD related pathology (Braak stages V-VI). It should be noted that clinical studies reporting that EP is common in AD include all subjects with AD diagnosis.…”

“…In AD, the hippocampal formation displays substantial pathology including AD related hallmark lesions such as neurofibrillary tangles and neuritic plaques [3]. Furthermore, many reports have indicated that a substantial number of AD subjects, in addition to the AD related lesions, also display TAR DNA binding protein 43 (TDP43) within the hippocampus [4][5][6][7][8][9][10][11][12][13][14]. TDP43 related pathology is primarily seen in subjects with frontotemporal lobar degeneration (FTLD) [15].…”

Clinical, neuropathological and genotypic variability in SNCA A53T familial Parkinson’s disease

“…In the A53T individual with wild-type Parkin, neuronal loss was present in area CA1 and the subiculum and resembled hippocampal sclerosis, which in the elderly is often associated with ubiquitin or TDP-43 positive neuronal inclusions [1][2], but also had frontotemporal cortical atrophy and with TDP-43 positive neuritic and intranuclear inclusions. TDP-43 is a conserved heterogeneous ribonucleoprotein associated with RNA and DNA binding, transcription repression, alternative splicing and higher-order arrangement of nuclear bodies [4,7,44].…”

Understanding hippocampal sclerosis in the elderly: Epidemiology, characterization, and diagnostic issues

“…Amador-Ortiz et al [ 14 ] also defi ned and compared two subgroups of HS: 8 cases with "pure" HS and dementia (HSD) and 10 cases of HS with FTLD-U. HSD cases were older (85 vs 64 years) and had more tangles in the hippocampus (Braak stage as high as IV).…”