title: Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia.
abstract: We report two patients with motor neuron disease-inclusion dementia, with special reference to the pathology of the motor neuron system and hippocampal formation. The ages of the patients at death were 55 and 62 years, and the disease durations were 8 and 3 years, respectively. The two patients exhibited progressive frontotemporal dementia in the absence of motor neuron signs. At autopsy, both cases exhibited frontotemporal lobar atrophy with ubiquitin-positive, and tau- and alpha-synuclein-negative neuronal inclusions. As expected from the clinical signs, in both cases, the upper and lower motor neuron systems were well preserved: no Bunina bodies or ubiquitinated inclusions were detected in the motor neurons. However, of great importance was that when visualized immunohistochemically, the Golgi apparatus and trans-Golgi network often exhibited fragmentation in the lower motor neurons (the spinal anterior horn cells). In one of the cases, a decrease in the amount of Golgi apparatus was also a frequent feature in the upper motor neurons (Betz cells in the motor cortex). Moreover, in both cases, circumscribed degeneration affecting the CA1-subiculum border zone was evident in the hippocampal formation. These findings further strengthen the idea that, pathologically, motor neuron disease-inclusion dementia is a rare phenotype of amyotrophic lateral sclerosis.
authors.affiliation: Department of Pathology, Brain Research Institute, Niigata University, 1-757 Asahimachi, 951-8585 Niigata, Japan. yasuko@bri.niigata-u.ac.jp
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Toyoshima
authors.forename: Yue-Shan
authors.initials: YS
authors.lastname: Piao
authors.forename: Chun-Feng
authors.initials: CF
authors.lastname: Tan
authors.forename: Masahiro
authors.initials: M
authors.lastname: Morita
authors.forename: Masaharu
authors.initials: M
authors.lastname: Tanaka
authors.forename: Kiyomitsu
authors.initials: K
authors.lastname: Oyanagi
authors.forename: Koichi
authors.initials: K
authors.lastname: Okamoto
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
mesh_terms: D003459:Crystallins / Q000378:metabolism; D055316:Cystatin C; D015891:Cystatins / Q000378:metabolism; D003704:Dementia / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D005625:Frontal Lobe / Q000473:pathology; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006023:Glycoproteins; D006624:Hippocampus / Q000473:pathology*; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D008565:Membrane Proteins; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D009419:Nerve Tissue Proteins / Q000378:metabolism; D016900:Neurofilament Proteins / Q000378:metabolism; D013116:Spinal Cord / Q000473:pathology; D051843:Synucleins; D025801:Ubiquitin / Q000378:metabolism; D051844:alpha-Synuclein; D016875:tau Proteins / Q000378:metabolism
chemical_list: C525030:CST3 protein, human; D003459:Crystallins; D055316:Cystatin C; D015891:Cystatins; D005904:Glial Fibrillary Acidic Protein; D006023:Glycoproteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D016900:Neurofilament Proteins; C497604:SNCA protein, human; D051843:Synucleins; C072237:TGOLN2 protein, human; D025801:Ubiquitin; D051844:alpha-Synuclein; D016875:tau Proteins