Loading…
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology
Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (
Saved in:
| Published in: | Acta neuropathologica 2009, Vol.117 (1), p.45-53 |
|---|---|
| Main Authors: | , , , , , , , , , , , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653 |
| container_end_page | 53 |
| container_issue | 1 |
| container_start_page | 45 |
| container_title | Acta neuropathologica |
| container_volume | 117 |
| creator | Nishihira, Yasushi Tan, Chun-Feng Hoshi, Yasuhiro Iwanaga, Keisuke Yamada, Megumi Kawachi, Izumi Tsujihata, Mitsuhiro Hozumi, Isao Morita, Takashi Onodera, Osamu Nishizawa, Masatoyo Kakita, Akiyoshi Takahashi, Hitoshi |
| description | Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short ( |
| doi_str_mv | 10.1007/s00401-008-0443-6 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_66723506</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>66723506</sourcerecordid><originalsourceid>FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653</originalsourceid><addsrcrecordid>eNqFkU1rGzEQhkVpSFw3P6CXInrIbZuRtNKujyUfTSCQQNyzkLWztlztaivtNvjfR8aGQKHkpJHmmXc08xLyhcF3BlBdJoASWAFQF1CWolAfyIyVghcghfhIZgA5qwTnZ-RTStt841UpT8kZqxdc1ELNyO_nIUTTOEtNtwtjDMMmx96MGI2nyXqMIblEQ0t96Ne0maIZXehpfjMpBesy2tAXN25oxFzn_qLf0c75hi6vn4pS0MGMm-DDeveZnLTGJzw_nnPy6_ZmeXVXPDz-vL_68VDYEmAspEDTcIVMNrWUK4MGuLGSSwbCSLVQyvAKpEXVWmzFigsQAiWvW4BFo6SYk4uD7hDDnwnTqDuXLHpvegxT0kpVXEhQ74IcJOQ9VRn89g-4DVPs8xCaM7YAXssyQ-wA2byyFLHVQ3SdiTvNQO_90ge_dPZL7_3S-x98PQpPqw6bt4qjQRngByDlVL_G-Nb5_6qvFMqf7w</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>211902854</pqid></control><display><type>article</type><title>Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology</title><source>Springer Nature</source><creator>Nishihira, Yasushi ; Tan, Chun-Feng ; Hoshi, Yasuhiro ; Iwanaga, Keisuke ; Yamada, Megumi ; Kawachi, Izumi ; Tsujihata, Mitsuhiro ; Hozumi, Isao ; Morita, Takashi ; Onodera, Osamu ; Nishizawa, Masatoyo ; Kakita, Akiyoshi ; Takahashi, Hitoshi</creator><creatorcontrib>Nishihira, Yasushi ; Tan, Chun-Feng ; Hoshi, Yasuhiro ; Iwanaga, Keisuke ; Yamada, Megumi ; Kawachi, Izumi ; Tsujihata, Mitsuhiro ; Hozumi, Isao ; Morita, Takashi ; Onodera, Osamu ; Nishizawa, Masatoyo ; Kakita, Akiyoshi ; Takahashi, Hitoshi</creatorcontrib><description>Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (<5 years). However, it is also known that a few patients may survive for 10 years or more, even without artificial respiratory support (ARS). In the present study using TDP-43 immunohistochemistry, we examined various regions of the nervous system in six patients with SALS of long duration (10–20 years) without ARS, in whom lower motor-predominant disease with Bunina bodies and ubiquitinated inclusions (UIs) in the affected lower motor neurons was confirmed. One case also showed UIs in the hippocampal dentate granule cells (UDG). In all cases, except one with UDG, the occurrence of TDP-43-immunoreactive (ir) neuronal cytoplasmic inclusions (NCIs) was confined to a few regions in the spinal cord and brainstem, including the anterior horns. In one case with UDG, TDP-43-ir NCIs were also detected in the substantia nigra, and some regions of the cerebrum, including the hippocampal dentate gyrus (granule cells). The number of neurons displaying NCIs in each region was very small (1–3 per region, except the dentate gyrus). On the other hand, the occurrence of TDP-43-ir glial cytoplasmic inclusions (GCIs) was more widespread in the central nervous system, including the cerebral white matter. Again, however, the number of glial cells displaying GCIs in each region was very small (1–3 per region). In conclusion, compared to the usual form of SALS, TDP-43 pathology shown in SALS of long duration was apparently mild in degree and limited in distribution, corresponding to the relatively benign clinical courses observed. It is now apparent that SALS of long duration is actually part of a TDP-43 proteinopathy spectrum.</description><identifier>ISSN: 0001-6322</identifier><identifier>EISSN: 1432-0533</identifier><identifier>DOI: 10.1007/s00401-008-0443-6</identifier><identifier>PMID: 18923836</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer-Verlag</publisher><subject>Aged ; Alzheimer's disease ; Amyotrophic lateral sclerosis ; Amyotrophic Lateral Sclerosis - metabolism ; Amyotrophic Lateral Sclerosis - pathology ; Anterior Horn Cells - metabolism ; Anterior Horn Cells - pathology ; Anterior Horn Cells - ultrastructure ; Autopsies ; Autopsy ; Brain - metabolism ; Brain - pathology ; Brain research ; Brain Stem - metabolism ; Brain Stem - pathology ; Cerebrum - metabolism ; Cerebrum - pathology ; Dementia ; Dentate Gyrus - metabolism ; Dentate Gyrus - pathology ; Disease ; DNA-Binding Proteins - metabolism ; Dysphagia ; Dyspnea ; Female ; Hippocampus - metabolism ; Hippocampus - pathology ; Humans ; Immunohistochemistry ; Inclusion Bodies - metabolism ; Inclusion Bodies - pathology ; Male ; Medicine ; Medicine & Public Health ; Microscopy, Immunoelectron ; Middle Aged ; Nervous system ; Neuroglia - metabolism ; Neuroglia - pathology ; Neurology ; Neurons ; Neurons - metabolism ; Neurons - pathology ; Neurosciences ; Original Paper ; Pathology ; Research centers ; Spinal Cord - metabolism ; Spinal Cord - pathology ; Substantia Nigra - metabolism ; Substantia Nigra - pathology ; Time Factors ; Ubiquitin - metabolism</subject><ispartof>Acta neuropathologica, 2009, Vol.117 (1), p.45-53</ispartof><rights>Springer-Verlag 2008</rights><rights>Springer-Verlag 2009</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653</citedby><cites>FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18923836$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Nishihira, Yasushi</creatorcontrib><creatorcontrib>Tan, Chun-Feng</creatorcontrib><creatorcontrib>Hoshi, Yasuhiro</creatorcontrib><creatorcontrib>Iwanaga, Keisuke</creatorcontrib><creatorcontrib>Yamada, Megumi</creatorcontrib><creatorcontrib>Kawachi, Izumi</creatorcontrib><creatorcontrib>Tsujihata, Mitsuhiro</creatorcontrib><creatorcontrib>Hozumi, Isao</creatorcontrib><creatorcontrib>Morita, Takashi</creatorcontrib><creatorcontrib>Onodera, Osamu</creatorcontrib><creatorcontrib>Nishizawa, Masatoyo</creatorcontrib><creatorcontrib>Kakita, Akiyoshi</creatorcontrib><creatorcontrib>Takahashi, Hitoshi</creatorcontrib><title>Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology</title><title>Acta neuropathologica</title><addtitle>Acta Neuropathol</addtitle><addtitle>Acta Neuropathol</addtitle><description>Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (<5 years). However, it is also known that a few patients may survive for 10 years or more, even without artificial respiratory support (ARS). In the present study using TDP-43 immunohistochemistry, we examined various regions of the nervous system in six patients with SALS of long duration (10–20 years) without ARS, in whom lower motor-predominant disease with Bunina bodies and ubiquitinated inclusions (UIs) in the affected lower motor neurons was confirmed. One case also showed UIs in the hippocampal dentate granule cells (UDG). In all cases, except one with UDG, the occurrence of TDP-43-immunoreactive (ir) neuronal cytoplasmic inclusions (NCIs) was confined to a few regions in the spinal cord and brainstem, including the anterior horns. In one case with UDG, TDP-43-ir NCIs were also detected in the substantia nigra, and some regions of the cerebrum, including the hippocampal dentate gyrus (granule cells). The number of neurons displaying NCIs in each region was very small (1–3 per region, except the dentate gyrus). On the other hand, the occurrence of TDP-43-ir glial cytoplasmic inclusions (GCIs) was more widespread in the central nervous system, including the cerebral white matter. Again, however, the number of glial cells displaying GCIs in each region was very small (1–3 per region). In conclusion, compared to the usual form of SALS, TDP-43 pathology shown in SALS of long duration was apparently mild in degree and limited in distribution, corresponding to the relatively benign clinical courses observed. It is now apparent that SALS of long duration is actually part of a TDP-43 proteinopathy spectrum.</description><subject>Aged</subject><subject>Alzheimer's disease</subject><subject>Amyotrophic lateral sclerosis</subject><subject>Amyotrophic Lateral Sclerosis - metabolism</subject><subject>Amyotrophic Lateral Sclerosis - pathology</subject><subject>Anterior Horn Cells - metabolism</subject><subject>Anterior Horn Cells - pathology</subject><subject>Anterior Horn Cells - ultrastructure</subject><subject>Autopsies</subject><subject>Autopsy</subject><subject>Brain - metabolism</subject><subject>Brain - pathology</subject><subject>Brain research</subject><subject>Brain Stem - metabolism</subject><subject>Brain Stem - pathology</subject><subject>Cerebrum - metabolism</subject><subject>Cerebrum - pathology</subject><subject>Dementia</subject><subject>Dentate Gyrus - metabolism</subject><subject>Dentate Gyrus - pathology</subject><subject>Disease</subject><subject>DNA-Binding Proteins - metabolism</subject><subject>Dysphagia</subject><subject>Dyspnea</subject><subject>Female</subject><subject>Hippocampus - metabolism</subject><subject>Hippocampus - pathology</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Inclusion Bodies - metabolism</subject><subject>Inclusion Bodies - pathology</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Microscopy, Immunoelectron</subject><subject>Middle Aged</subject><subject>Nervous system</subject><subject>Neuroglia - metabolism</subject><subject>Neuroglia - pathology</subject><subject>Neurology</subject><subject>Neurons</subject><subject>Neurons - metabolism</subject><subject>Neurons - pathology</subject><subject>Neurosciences</subject><subject>Original Paper</subject><subject>Pathology</subject><subject>Research centers</subject><subject>Spinal Cord - metabolism</subject><subject>Spinal Cord - pathology</subject><subject>Substantia Nigra - metabolism</subject><subject>Substantia Nigra - pathology</subject><subject>Time Factors</subject><subject>Ubiquitin - metabolism</subject><issn>0001-6322</issn><issn>1432-0533</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><recordid>eNqFkU1rGzEQhkVpSFw3P6CXInrIbZuRtNKujyUfTSCQQNyzkLWztlztaivtNvjfR8aGQKHkpJHmmXc08xLyhcF3BlBdJoASWAFQF1CWolAfyIyVghcghfhIZgA5qwTnZ-RTStt841UpT8kZqxdc1ELNyO_nIUTTOEtNtwtjDMMmx96MGI2nyXqMIblEQ0t96Ne0maIZXehpfjMpBesy2tAXN25oxFzn_qLf0c75hi6vn4pS0MGMm-DDeveZnLTGJzw_nnPy6_ZmeXVXPDz-vL_68VDYEmAspEDTcIVMNrWUK4MGuLGSSwbCSLVQyvAKpEXVWmzFigsQAiWvW4BFo6SYk4uD7hDDnwnTqDuXLHpvegxT0kpVXEhQ74IcJOQ9VRn89g-4DVPs8xCaM7YAXssyQ-wA2byyFLHVQ3SdiTvNQO_90ge_dPZL7_3S-x98PQpPqw6bt4qjQRngByDlVL_G-Nb5_6qvFMqf7w</recordid><startdate>2009</startdate><enddate>2009</enddate><creator>Nishihira, Yasushi</creator><creator>Tan, Chun-Feng</creator><creator>Hoshi, Yasuhiro</creator><creator>Iwanaga, Keisuke</creator><creator>Yamada, Megumi</creator><creator>Kawachi, Izumi</creator><creator>Tsujihata, Mitsuhiro</creator><creator>Hozumi, Isao</creator><creator>Morita, Takashi</creator><creator>Onodera, Osamu</creator><creator>Nishizawa, Masatoyo</creator><creator>Kakita, Akiyoshi</creator><creator>Takahashi, Hitoshi</creator><general>Springer-Verlag</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TK</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7X8</scope></search><sort><creationdate>2009</creationdate><title>Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology</title><author>Nishihira, Yasushi ; Tan, Chun-Feng ; Hoshi, Yasuhiro ; Iwanaga, Keisuke ; Yamada, Megumi ; Kawachi, Izumi ; Tsujihata, Mitsuhiro ; Hozumi, Isao ; Morita, Takashi ; Onodera, Osamu ; Nishizawa, Masatoyo ; Kakita, Akiyoshi ; Takahashi, Hitoshi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Aged</topic><topic>Alzheimer's disease</topic><topic>Amyotrophic lateral sclerosis</topic><topic>Amyotrophic Lateral Sclerosis - metabolism</topic><topic>Amyotrophic Lateral Sclerosis - pathology</topic><topic>Anterior Horn Cells - metabolism</topic><topic>Anterior Horn Cells - pathology</topic><topic>Anterior Horn Cells - ultrastructure</topic><topic>Autopsies</topic><topic>Autopsy</topic><topic>Brain - metabolism</topic><topic>Brain - pathology</topic><topic>Brain research</topic><topic>Brain Stem - metabolism</topic><topic>Brain Stem - pathology</topic><topic>Cerebrum - metabolism</topic><topic>Cerebrum - pathology</topic><topic>Dementia</topic><topic>Dentate Gyrus - metabolism</topic><topic>Dentate Gyrus - pathology</topic><topic>Disease</topic><topic>DNA-Binding Proteins - metabolism</topic><topic>Dysphagia</topic><topic>Dyspnea</topic><topic>Female</topic><topic>Hippocampus - metabolism</topic><topic>Hippocampus - pathology</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Inclusion Bodies - metabolism</topic><topic>Inclusion Bodies - pathology</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Microscopy, Immunoelectron</topic><topic>Middle Aged</topic><topic>Nervous system</topic><topic>Neuroglia - metabolism</topic><topic>Neuroglia - pathology</topic><topic>Neurology</topic><topic>Neurons</topic><topic>Neurons - metabolism</topic><topic>Neurons - pathology</topic><topic>Neurosciences</topic><topic>Original Paper</topic><topic>Pathology</topic><topic>Research centers</topic><topic>Spinal Cord - metabolism</topic><topic>Spinal Cord - pathology</topic><topic>Substantia Nigra - metabolism</topic><topic>Substantia Nigra - pathology</topic><topic>Time Factors</topic><topic>Ubiquitin - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Nishihira, Yasushi</creatorcontrib><creatorcontrib>Tan, Chun-Feng</creatorcontrib><creatorcontrib>Hoshi, Yasuhiro</creatorcontrib><creatorcontrib>Iwanaga, Keisuke</creatorcontrib><creatorcontrib>Yamada, Megumi</creatorcontrib><creatorcontrib>Kawachi, Izumi</creatorcontrib><creatorcontrib>Tsujihata, Mitsuhiro</creatorcontrib><creatorcontrib>Hozumi, Isao</creatorcontrib><creatorcontrib>Morita, Takashi</creatorcontrib><creatorcontrib>Onodera, Osamu</creatorcontrib><creatorcontrib>Nishizawa, Masatoyo</creatorcontrib><creatorcontrib>Kakita, Akiyoshi</creatorcontrib><creatorcontrib>Takahashi, Hitoshi</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Proquest Health & Medical Complete</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Psychology Database (Alumni)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>ProQuest Central Essentials</collection><collection>AUTh Library subscriptions: ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>PML(ProQuest Medical Library)</collection><collection>Psychology Database (ProQuest)</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><jtitle>Acta neuropathologica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Nishihira, Yasushi</au><au>Tan, Chun-Feng</au><au>Hoshi, Yasuhiro</au><au>Iwanaga, Keisuke</au><au>Yamada, Megumi</au><au>Kawachi, Izumi</au><au>Tsujihata, Mitsuhiro</au><au>Hozumi, Isao</au><au>Morita, Takashi</au><au>Onodera, Osamu</au><au>Nishizawa, Masatoyo</au><au>Kakita, Akiyoshi</au><au>Takahashi, Hitoshi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology</atitle><jtitle>Acta neuropathologica</jtitle><stitle>Acta Neuropathol</stitle><addtitle>Acta Neuropathol</addtitle><date>2009</date><risdate>2009</risdate><volume>117</volume><issue>1</issue><spage>45</spage><epage>53</epage><pages>45-53</pages><issn>0001-6322</issn><eissn>1432-0533</eissn><abstract>Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (<5 years). However, it is also known that a few patients may survive for 10 years or more, even without artificial respiratory support (ARS). In the present study using TDP-43 immunohistochemistry, we examined various regions of the nervous system in six patients with SALS of long duration (10–20 years) without ARS, in whom lower motor-predominant disease with Bunina bodies and ubiquitinated inclusions (UIs) in the affected lower motor neurons was confirmed. One case also showed UIs in the hippocampal dentate granule cells (UDG). In all cases, except one with UDG, the occurrence of TDP-43-immunoreactive (ir) neuronal cytoplasmic inclusions (NCIs) was confined to a few regions in the spinal cord and brainstem, including the anterior horns. In one case with UDG, TDP-43-ir NCIs were also detected in the substantia nigra, and some regions of the cerebrum, including the hippocampal dentate gyrus (granule cells). The number of neurons displaying NCIs in each region was very small (1–3 per region, except the dentate gyrus). On the other hand, the occurrence of TDP-43-ir glial cytoplasmic inclusions (GCIs) was more widespread in the central nervous system, including the cerebral white matter. Again, however, the number of glial cells displaying GCIs in each region was very small (1–3 per region). In conclusion, compared to the usual form of SALS, TDP-43 pathology shown in SALS of long duration was apparently mild in degree and limited in distribution, corresponding to the relatively benign clinical courses observed. It is now apparent that SALS of long duration is actually part of a TDP-43 proteinopathy spectrum.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer-Verlag</pub><pmid>18923836</pmid><doi>10.1007/s00401-008-0443-6</doi><tpages>9</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0001-6322 |
| ispartof | Acta neuropathologica, 2009, Vol.117 (1), p.45-53 |
| issn | 0001-6322 1432-0533 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_66723506 |
| source | Springer Nature |
| subjects | Aged Alzheimer's disease Amyotrophic lateral sclerosis Amyotrophic Lateral Sclerosis - metabolism Amyotrophic Lateral Sclerosis - pathology Anterior Horn Cells - metabolism Anterior Horn Cells - pathology Anterior Horn Cells - ultrastructure Autopsies Autopsy Brain - metabolism Brain - pathology Brain research Brain Stem - metabolism Brain Stem - pathology Cerebrum - metabolism Cerebrum - pathology Dementia Dentate Gyrus - metabolism Dentate Gyrus - pathology Disease DNA-Binding Proteins - metabolism Dysphagia Dyspnea Female Hippocampus - metabolism Hippocampus - pathology Humans Immunohistochemistry Inclusion Bodies - metabolism Inclusion Bodies - pathology Male Medicine Medicine & Public Health Microscopy, Immunoelectron Middle Aged Nervous system Neuroglia - metabolism Neuroglia - pathology Neurology Neurons Neurons - metabolism Neurons - pathology Neurosciences Original Paper Pathology Research centers Spinal Cord - metabolism Spinal Cord - pathology Substantia Nigra - metabolism Substantia Nigra - pathology Time Factors Ubiquitin - metabolism |
| title | Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-06T06%3A55%3A42IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Sporadic%20amyotrophic%20lateral%20sclerosis%20of%20long%20duration%20is%20associated%20with%20relatively%20mild%20TDP-43%20pathology&rft.jtitle=Acta%20neuropathologica&rft.au=Nishihira,%20Yasushi&rft.date=2009&rft.volume=117&rft.issue=1&rft.spage=45&rft.epage=53&rft.pages=45-53&rft.issn=0001-6322&rft.eissn=1432-0533&rft_id=info:doi/10.1007/s00401-008-0443-6&rft_dat=%3Cproquest_cross%3E66723506%3C/proquest_cross%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c400t-53ead26e15d855baea02ac525103a56966a2705ce6fcef3b23033e528f009d653%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=211902854&rft_id=info:pmid/18923836&rfr_iscdi=true |