Decrease of neurons in the medullary arcuate nucleus in myotonic dystrophy
Respiratory insufficiency has been reported frequently in patients with myotonic dystrophy (MyD). Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the central nervous system. The medullary arcuate nucleus (ARC) has been shown to be involved in th...
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| Veröffentlicht in: | Acta neuropathologica 2001-07, Vol.102 (1), p.89-93 |
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| creator | ONO, Seiitsu TAKAHASHI, Keiichi KANDA, Fumio JINNAI, Kenji FUKUOKA, Yoshihiro MITAKE, Shigehisa INAGAKI, Toshiaki KURISAKI, Hiroshi NAGAO, Koichi SHIMIZU, Natsue |
| description | Respiratory insufficiency has been reported frequently in patients with myotonic dystrophy (MyD). Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the central nervous system. The medullary arcuate nucleus (ARC) has been shown to be involved in the regulation of respiration. We performed a quantitative study of neurons in the ARC in eight MyD patients, ten control subjects with other neurological diseases (control group A) and eight control subjects without neurological diseases (control group B). Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The density of neurons in the ARC in MyD patients with hypoventilation was significantly lower than in MyD patients without hypoventilation and control groups A and B. There was no significant difference in the neuronal density of the ARC between MyD patients without hypoventilation and control groups A and B. These data suggest that the neuronal loss of the ARC is associated with the presence of hypoventilation in MyD. |
| doi_str_mv | 10.1007/s004010000359 |
| format | Article |
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Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the central nervous system. The medullary arcuate nucleus (ARC) has been shown to be involved in the regulation of respiration. We performed a quantitative study of neurons in the ARC in eight MyD patients, ten control subjects with other neurological diseases (control group A) and eight control subjects without neurological diseases (control group B). Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The density of neurons in the ARC in MyD patients with hypoventilation was significantly lower than in MyD patients without hypoventilation and control groups A and B. There was no significant difference in the neuronal density of the ARC between MyD patients without hypoventilation and control groups A and B. These data suggest that the neuronal loss of the ARC is associated with the presence of hypoventilation in MyD.</description><identifier>ISSN: 0001-6322</identifier><identifier>EISSN: 1432-0533</identifier><identifier>DOI: 10.1007/s004010000359</identifier><identifier>PMID: 11547956</identifier><identifier>CODEN: ANPTAL</identifier><language>eng</language><publisher>Berlin: Springer</publisher><subject>Aged ; Alveoli ; Arcuate nucleus ; Arcuate Nucleus of Hypothalamus - pathology ; Biological and medical sciences ; Cell Count ; Central nervous system ; Diseases of striated muscles. Neuromuscular diseases ; Female ; Humans ; Hypoventilation ; Hypoventilation - etiology ; Male ; Medical sciences ; Medulla Oblongata ; Middle Aged ; Myotonic dystrophy ; Myotonic Dystrophy - complications ; Myotonic Dystrophy - pathology ; Neurological diseases ; Neurology ; Neurons ; Neurons - pathology ; Reference Values ; Respiration</subject><ispartof>Acta neuropathologica, 2001-07, Vol.102 (1), p.89-93</ispartof><rights>2001 INIST-CNRS</rights><rights>Springer-Verlag 2001.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c412t-f484dea6a3767137962c8aa84084fc423cdd937b2d90978984b7dab6d23fdf4a3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,781,785,27929,27930</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1086045$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11547956$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>ONO, Seiitsu</creatorcontrib><creatorcontrib>TAKAHASHI, Keiichi</creatorcontrib><creatorcontrib>KANDA, Fumio</creatorcontrib><creatorcontrib>JINNAI, Kenji</creatorcontrib><creatorcontrib>FUKUOKA, Yoshihiro</creatorcontrib><creatorcontrib>MITAKE, Shigehisa</creatorcontrib><creatorcontrib>INAGAKI, Toshiaki</creatorcontrib><creatorcontrib>KURISAKI, Hiroshi</creatorcontrib><creatorcontrib>NAGAO, Koichi</creatorcontrib><creatorcontrib>SHIMIZU, Natsue</creatorcontrib><title>Decrease of neurons in the medullary arcuate nucleus in myotonic dystrophy</title><title>Acta neuropathologica</title><addtitle>Acta Neuropathol</addtitle><description>Respiratory insufficiency has been reported frequently in patients with myotonic dystrophy (MyD). Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the central nervous system. The medullary arcuate nucleus (ARC) has been shown to be involved in the regulation of respiration. We performed a quantitative study of neurons in the ARC in eight MyD patients, ten control subjects with other neurological diseases (control group A) and eight control subjects without neurological diseases (control group B). Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The density of neurons in the ARC in MyD patients with hypoventilation was significantly lower than in MyD patients without hypoventilation and control groups A and B. There was no significant difference in the neuronal density of the ARC between MyD patients without hypoventilation and control groups A and B. These data suggest that the neuronal loss of the ARC is associated with the presence of hypoventilation in MyD.</description><subject>Aged</subject><subject>Alveoli</subject><subject>Arcuate nucleus</subject><subject>Arcuate Nucleus of Hypothalamus - pathology</subject><subject>Biological and medical sciences</subject><subject>Cell Count</subject><subject>Central nervous system</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Hypoventilation</subject><subject>Hypoventilation - etiology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Medulla Oblongata</subject><subject>Middle Aged</subject><subject>Myotonic dystrophy</subject><subject>Myotonic Dystrophy - complications</subject><subject>Myotonic Dystrophy - pathology</subject><subject>Neurological diseases</subject><subject>Neurology</subject><subject>Neurons</subject><subject>Neurons - pathology</subject><subject>Reference Values</subject><subject>Respiration</subject><issn>0001-6322</issn><issn>1432-0533</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNpd0ElLxDAYBuAgio6jR68SULxVs6c9yrgz4EXPJc3CdOg0Y9Ic-u-NTsHllIWHl-97ATjD6BojJG8iQgzlG0KUV3tghhklBeKU7oNZ_sSFoIQcgeMY1_lFJOOH4AhjzmTFxQy83FkdrIoWegd7m4LvI2x7OKws3FiTuk6FEaqgkxos7JPubPoGm9EPvm81NGMcgt-uxhNw4FQX7el0zsH7w_3b4qlYvj4-L26XhWaYDIVjJTNWCUWlkJjKShBdKlUyVDKnGaHamIrKhpgKVbKsStZIoxphCHXGMUXn4GqXuw3-I9k41Js2apsn7a1PsZZ5O8IxzvDiH1z7FPo8W00Y5hJXkousip3SwccYrKu3od3krWuM6q-K6z8VZ38-paYmN_Sjp04zuJyAilp1Lqhet_FXaikQ4_QTakaCAA</recordid><startdate>20010701</startdate><enddate>20010701</enddate><creator>ONO, Seiitsu</creator><creator>TAKAHASHI, Keiichi</creator><creator>KANDA, Fumio</creator><creator>JINNAI, Kenji</creator><creator>FUKUOKA, Yoshihiro</creator><creator>MITAKE, Shigehisa</creator><creator>INAGAKI, Toshiaki</creator><creator>KURISAKI, Hiroshi</creator><creator>NAGAO, Koichi</creator><creator>SHIMIZU, Natsue</creator><general>Springer</general><general>Springer Nature B.V</general><scope>IQODW</scope><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>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7X8</scope></search><sort><creationdate>20010701</creationdate><title>Decrease of neurons in the medullary arcuate nucleus in myotonic dystrophy</title><author>ONO, Seiitsu ; TAKAHASHI, Keiichi ; KANDA, Fumio ; JINNAI, Kenji ; FUKUOKA, Yoshihiro ; MITAKE, Shigehisa ; INAGAKI, Toshiaki ; KURISAKI, Hiroshi ; NAGAO, Koichi ; SHIMIZU, Natsue</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c412t-f484dea6a3767137962c8aa84084fc423cdd937b2d90978984b7dab6d23fdf4a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Aged</topic><topic>Alveoli</topic><topic>Arcuate nucleus</topic><topic>Arcuate Nucleus of Hypothalamus - pathology</topic><topic>Biological and medical sciences</topic><topic>Cell Count</topic><topic>Central nervous system</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Hypoventilation</topic><topic>Hypoventilation - etiology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Medulla Oblongata</topic><topic>Middle Aged</topic><topic>Myotonic dystrophy</topic><topic>Myotonic Dystrophy - complications</topic><topic>Myotonic Dystrophy - pathology</topic><topic>Neurological diseases</topic><topic>Neurology</topic><topic>Neurons</topic><topic>Neurons - pathology</topic><topic>Reference Values</topic><topic>Respiration</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>ONO, Seiitsu</creatorcontrib><creatorcontrib>TAKAHASHI, Keiichi</creatorcontrib><creatorcontrib>KANDA, Fumio</creatorcontrib><creatorcontrib>JINNAI, Kenji</creatorcontrib><creatorcontrib>FUKUOKA, Yoshihiro</creatorcontrib><creatorcontrib>MITAKE, Shigehisa</creatorcontrib><creatorcontrib>INAGAKI, Toshiaki</creatorcontrib><creatorcontrib>KURISAKI, Hiroshi</creatorcontrib><creatorcontrib>NAGAO, Koichi</creatorcontrib><creatorcontrib>SHIMIZU, Natsue</creatorcontrib><collection>Pascal-Francis</collection><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>Health & Medical Collection</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 Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</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>Medical Database</collection><collection>Psychology Database</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 Central China</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>ONO, Seiitsu</au><au>TAKAHASHI, Keiichi</au><au>KANDA, Fumio</au><au>JINNAI, Kenji</au><au>FUKUOKA, Yoshihiro</au><au>MITAKE, Shigehisa</au><au>INAGAKI, Toshiaki</au><au>KURISAKI, Hiroshi</au><au>NAGAO, Koichi</au><au>SHIMIZU, Natsue</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Decrease of neurons in the medullary arcuate nucleus in myotonic dystrophy</atitle><jtitle>Acta neuropathologica</jtitle><addtitle>Acta Neuropathol</addtitle><date>2001-07-01</date><risdate>2001</risdate><volume>102</volume><issue>1</issue><spage>89</spage><epage>93</epage><pages>89-93</pages><issn>0001-6322</issn><eissn>1432-0533</eissn><coden>ANPTAL</coden><abstract>Respiratory insufficiency has been reported frequently in patients with myotonic dystrophy (MyD). Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the central nervous system. The medullary arcuate nucleus (ARC) has been shown to be involved in the regulation of respiration. We performed a quantitative study of neurons in the ARC in eight MyD patients, ten control subjects with other neurological diseases (control group A) and eight control subjects without neurological diseases (control group B). Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The density of neurons in the ARC in MyD patients with hypoventilation was significantly lower than in MyD patients without hypoventilation and control groups A and B. There was no significant difference in the neuronal density of the ARC between MyD patients without hypoventilation and control groups A and B. These data suggest that the neuronal loss of the ARC is associated with the presence of hypoventilation in MyD.</abstract><cop>Berlin</cop><pub>Springer</pub><pmid>11547956</pmid><doi>10.1007/s004010000359</doi><tpages>5</tpages></addata></record> |
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| subjects | Aged Alveoli Arcuate nucleus Arcuate Nucleus of Hypothalamus - pathology Biological and medical sciences Cell Count Central nervous system Diseases of striated muscles. Neuromuscular diseases Female Humans Hypoventilation Hypoventilation - etiology Male Medical sciences Medulla Oblongata Middle Aged Myotonic dystrophy Myotonic Dystrophy - complications Myotonic Dystrophy - pathology Neurological diseases Neurology Neurons Neurons - pathology Reference Values Respiration |
| title | Decrease of neurons in the medullary arcuate nucleus in myotonic dystrophy |
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