Duration of illness in Huntington's disease is not related to age at onset
The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast t...
Gespeichert in:
| Veröffentlicht in: | Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1993-01, Vol.56 (1), p.98-100 |
|---|---|
| Hauptverfasser: | , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 100 |
|---|---|
| container_issue | 1 |
| container_start_page | 98 |
| container_title | Journal of neurology, neurosurgery and psychiatry |
| container_volume | 56 |
| creator | Roos, R A Hermans, J Vegter-van der Vlis, M van Ommen, G J Bruyn, G W |
| description | The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father. |
| doi_str_mv | 10.1136/jnnp.56.1.98 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1014774</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>75564367</sourcerecordid><originalsourceid>FETCH-LOGICAL-b506t-a47b87e0ac2f00680ea40ca387506e018a8b9de217f5e6a0759d6482f061d0863</originalsourceid><addsrcrecordid>eNp90c2L1DAYBvAgyjq7evMqBBT3YsekaZP0IsisusqiLH7ewtv27Zixk4xJuuh_b8oMg3qwlxyeHw9PeQl5wNmScyGfbZzbLWu55MtG3yILXkldCMG-3iYLxsqyEKxmd8lpjBs2f7o5ISe6KptsFuTtxRQgWe-oH6gdR4cxUuvo5eSSdevk3XmkvY0IEamN1PlEA46QsKfJU1gjhUS9i5jukTsDjBHvH94z8unVy4-ry-Lq_es3qxdXRVszmQqoVKsVMujKgTGpGULFOhBa5RgZ16DbpseSq6FGCUzVTS8rnbHkPdNSnJHn-97d1G6x79ClAKPZBbuF8Mt4sObvxNlvZu1vDGe8UqrKBU8OBcH_mDAms7Wxw3EEh36KRtW1rIRUGT76B278FFz-OcOVEkw0rJnrnu5VF3yMAYfjFM7MfCEzX8jU0nDT6Mwf_jn_iA8nyfnjQw6xg3EI4Dobj6ySqpSaZ1bsmY0Jfx5jCN9NXq5q8-7zylxcf_gitLw2sz_f-3a7-f_A35zZtLE</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1773039094</pqid></control><display><type>article</type><title>Duration of illness in Huntington's disease is not related to age at onset</title><source>MEDLINE</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>PubMed Central</source><source>Alma/SFX Local Collection</source><creator>Roos, R A ; Hermans, J ; Vegter-van der Vlis, M ; van Ommen, G J ; Bruyn, G W</creator><creatorcontrib>Roos, R A ; Hermans, J ; Vegter-van der Vlis, M ; van Ommen, G J ; Bruyn, G W</creatorcontrib><description>The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.</description><identifier>ISSN: 0022-3050</identifier><identifier>EISSN: 1468-330X</identifier><identifier>DOI: 10.1136/jnnp.56.1.98</identifier><identifier>PMID: 8429330</identifier><identifier>CODEN: JNNPAU</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd</publisher><subject>Adolescent ; Adult ; Aged ; Biological and medical sciences ; Chromosome Aberrations - genetics ; Chromosome Disorders ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Family Health ; Female ; Humans ; Huntington Disease - genetics ; Male ; Medical sciences ; Middle Aged ; Neurology ; Pedigree ; Sex Factors</subject><ispartof>Journal of neurology, neurosurgery and psychiatry, 1993-01, Vol.56 (1), p.98-100</ispartof><rights>1993 INIST-CNRS</rights><rights>Copyright BMJ Publishing Group LTD Jan 1993</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b506t-a47b87e0ac2f00680ea40ca387506e018a8b9de217f5e6a0759d6482f061d0863</citedby><cites>FETCH-LOGICAL-b506t-a47b87e0ac2f00680ea40ca387506e018a8b9de217f5e6a0759d6482f061d0863</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1014774/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1014774/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,4010,27900,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=4672681$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/8429330$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Roos, R A</creatorcontrib><creatorcontrib>Hermans, J</creatorcontrib><creatorcontrib>Vegter-van der Vlis, M</creatorcontrib><creatorcontrib>van Ommen, G J</creatorcontrib><creatorcontrib>Bruyn, G W</creatorcontrib><title>Duration of illness in Huntington's disease is not related to age at onset</title><title>Journal of neurology, neurosurgery and psychiatry</title><addtitle>J Neurol Neurosurg Psychiatry</addtitle><description>The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Biological and medical sciences</subject><subject>Chromosome Aberrations - genetics</subject><subject>Chromosome Disorders</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Family Health</subject><subject>Female</subject><subject>Humans</subject><subject>Huntington Disease - genetics</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Neurology</subject><subject>Pedigree</subject><subject>Sex Factors</subject><issn>0022-3050</issn><issn>1468-330X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1993</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp90c2L1DAYBvAgyjq7evMqBBT3YsekaZP0IsisusqiLH7ewtv27Zixk4xJuuh_b8oMg3qwlxyeHw9PeQl5wNmScyGfbZzbLWu55MtG3yILXkldCMG-3iYLxsqyEKxmd8lpjBs2f7o5ISe6KptsFuTtxRQgWe-oH6gdR4cxUuvo5eSSdevk3XmkvY0IEamN1PlEA46QsKfJU1gjhUS9i5jukTsDjBHvH94z8unVy4-ry-Lq_es3qxdXRVszmQqoVKsVMujKgTGpGULFOhBa5RgZ16DbpseSq6FGCUzVTS8rnbHkPdNSnJHn-97d1G6x79ClAKPZBbuF8Mt4sObvxNlvZu1vDGe8UqrKBU8OBcH_mDAms7Wxw3EEh36KRtW1rIRUGT76B278FFz-OcOVEkw0rJnrnu5VF3yMAYfjFM7MfCEzX8jU0nDT6Mwf_jn_iA8nyfnjQw6xg3EI4Dobj6ySqpSaZ1bsmY0Jfx5jCN9NXq5q8-7zylxcf_gitLw2sz_f-3a7-f_A35zZtLE</recordid><startdate>199301</startdate><enddate>199301</enddate><creator>Roos, R A</creator><creator>Hermans, J</creator><creator>Vegter-van der Vlis, M</creator><creator>van Ommen, G J</creator><creator>Bruyn, G W</creator><general>BMJ Publishing Group Ltd</general><general>BMJ</general><general>BMJ Publishing Group LTD</general><scope>BSCLL</scope><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>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>88I</scope><scope>8AF</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>M2P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>199301</creationdate><title>Duration of illness in Huntington's disease is not related to age at onset</title><author>Roos, R A ; Hermans, J ; Vegter-van der Vlis, M ; van Ommen, G J ; Bruyn, G W</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b506t-a47b87e0ac2f00680ea40ca387506e018a8b9de217f5e6a0759d6482f061d0863</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1993</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Biological and medical sciences</topic><topic>Chromosome Aberrations - genetics</topic><topic>Chromosome Disorders</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Family Health</topic><topic>Female</topic><topic>Humans</topic><topic>Huntington Disease - genetics</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Neurology</topic><topic>Pedigree</topic><topic>Sex Factors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Roos, R A</creatorcontrib><creatorcontrib>Hermans, J</creatorcontrib><creatorcontrib>Vegter-van der Vlis, M</creatorcontrib><creatorcontrib>van Ommen, G J</creatorcontrib><creatorcontrib>Bruyn, G W</creatorcontrib><collection>Istex</collection><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>Nursing & Allied Health Database</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>Science Database (Alumni Edition)</collection><collection>STEM Database</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>BMJ Journals</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>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest Psychology</collection><collection>Science Database</collection><collection>Nursing & Allied Health Premium</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><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Roos, R A</au><au>Hermans, J</au><au>Vegter-van der Vlis, M</au><au>van Ommen, G J</au><au>Bruyn, G W</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Duration of illness in Huntington's disease is not related to age at onset</atitle><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle><addtitle>J Neurol Neurosurg Psychiatry</addtitle><date>1993-01</date><risdate>1993</risdate><volume>56</volume><issue>1</issue><spage>98</spage><epage>100</epage><pages>98-100</pages><issn>0022-3050</issn><eissn>1468-330X</eissn><coden>JNNPAU</coden><abstract>The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd</pub><pmid>8429330</pmid><doi>10.1136/jnnp.56.1.98</doi><tpages>3</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0022-3050 |
| ispartof | Journal of neurology, neurosurgery and psychiatry, 1993-01, Vol.56 (1), p.98-100 |
| issn | 0022-3050 1468-330X |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1014774 |
| source | MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; Alma/SFX Local Collection |
| subjects | Adolescent Adult Aged Biological and medical sciences Chromosome Aberrations - genetics Chromosome Disorders Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Family Health Female Humans Huntington Disease - genetics Male Medical sciences Middle Aged Neurology Pedigree Sex Factors |
| title | Duration of illness in Huntington's disease is not related to age at onset |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-09T20%3A06%3A56IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Duration%20of%20illness%20in%20Huntington's%20disease%20is%20not%20related%20to%20age%20at%20onset&rft.jtitle=Journal%20of%20neurology,%20neurosurgery%20and%20psychiatry&rft.au=Roos,%20R%20A&rft.date=1993-01&rft.volume=56&rft.issue=1&rft.spage=98&rft.epage=100&rft.pages=98-100&rft.issn=0022-3050&rft.eissn=1468-330X&rft.coden=JNNPAU&rft_id=info:doi/10.1136/jnnp.56.1.98&rft_dat=%3Cproquest_pubme%3E75564367%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1773039094&rft_id=info:pmid/8429330&rfr_iscdi=true |