Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes
ObjectiveTo analyse clinicoepidemiological features of neuromyelitis optica in a large cohort and to compare the differences between onset age, gender and clinical phenotypes.MethodsAntiaquaporin-4 antibody (AQP4-ab) levels were tested in 2366 serum samples of patients diagnosed as having central ne...
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description | ObjectiveTo analyse clinicoepidemiological features of neuromyelitis optica in a large cohort and to compare the differences between onset age, gender and clinical phenotypes.MethodsAntiaquaporin-4 antibody (AQP4-ab) levels were tested in 2366 serum samples of patients diagnosed as having central nervous system inflammatory demyelinating disorders by their referring physicians. AQP4-ab was measured by indirect immunofluorescence staining using human AQP4-transfected HEK 293 cells. A blinded analysis was performed and was combined with clinical information.ResultsA total of 583 patients (91.4% women) were AQP4-ab-positive. The average onset age was 42.9±15.9 years. According to MRI studies, spinal-cord lesions were detected in 85.3% of the patients, longitudinally extensive transverse myelitis in 72.7% and cerebral lesions in 51.1%. Unilateral or bilateral blindness was observed in 16.2% of patients, 19.8% were associated with Sjögren syndrome, and 13.6% were associated with thyroid diseases. Myelin basic protein was detected in the cerebrospinal fluid of 57.5% patients. In addition, men presented with an older onset age, a greater number of brainstem MRI lesions and positive myelin basic protein in the cerebrospinal fluid. All child-onset patients ( |
doi_str_mv | 10.1136/jnnp-2011-300403 |
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AQP4-ab was measured by indirect immunofluorescence staining using human AQP4-transfected HEK 293 cells. A blinded analysis was performed and was combined with clinical information.ResultsA total of 583 patients (91.4% women) were AQP4-ab-positive. The average onset age was 42.9±15.9 years. According to MRI studies, spinal-cord lesions were detected in 85.3% of the patients, longitudinally extensive transverse myelitis in 72.7% and cerebral lesions in 51.1%. Unilateral or bilateral blindness was observed in 16.2% of patients, 19.8% were associated with Sjögren syndrome, and 13.6% were associated with thyroid diseases. Myelin basic protein was detected in the cerebrospinal fluid of 57.5% patients. In addition, men presented with an older onset age, a greater number of brainstem MRI lesions and positive myelin basic protein in the cerebrospinal fluid. All child-onset patients (<15 years, n=9) presented with optic neuritis as the first symptom, while older-onset patients presented with myelitis. Twenty patients initially developed limited brain lesions, and seven of these patients did not develop optic or spinal lesions during the 1–5-year follow-up period.ConclusionsThe clinical characteristics of AQP4-ab-positive patients were similar. However, optic neuritis was more common in paediatric patients, while myelitis was more common in older patients. A small number of patients exhibited only cerebral, brainstem, or cerebellar lesions during the initial several years and lower Extended Disability Status Scale scores.</description><identifier>ISSN: 0022-3050</identifier><identifier>EISSN: 1468-330X</identifier><identifier>DOI: 10.1136/jnnp-2011-300403</identifier><identifier>PMID: 21665917</identifier><identifier>CODEN: JNNPAU</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd</publisher><subject>Adult ; Age ; Age of Onset ; Aminoacid disorders ; Antibodies ; Antibodies - blood ; Aquaporin 4 - immunology ; Biological and medical sciences ; Biomarkers - blood ; Biomarkers - cerebrospinal fluid ; Brain - pathology ; Cohort Studies ; CSF ; Disease ; Errors of metabolism ; Female ; Humans ; infectious diseases ; Japan - epidemiology ; Magnetic Resonance Imaging - methods ; Male ; Medical sciences ; Metabolic diseases ; Middle Aged ; multiple sclerosis ; Nervous system ; Neuroimaging - methods ; Neuroimmunology ; Neurology ; Neuromyelitis Optica - blood ; Neuromyelitis Optica - cerebrospinal fluid ; Neuromyelitis Optica - diagnosis ; Neuromyelitis Optica - epidemiology ; Neuromyelitis Optica - immunology ; Neuromyelitis Optica - pathology ; parkinson's disease ; Patients ; Phenotype ; Sex Characteristics ; Spinal cord ; Spinal Cord - pathology ; steroids ; Thyroid gland</subject><ispartof>Journal of neurology, neurosurgery and psychiatry, 2011-12, Vol.82 (12), p.1360-1364</ispartof><rights>2011, Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><rights>2015 INIST-CNRS</rights><rights>Copyright: 2011 (c) 2011, Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b528t-c5e60dff07cd57f4cf903cc9caef95cce5e8d2bf1cd9c08304a311ff18301c4b3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://jnnp.bmj.com/content/82/12/1360.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttps://jnnp.bmj.com/content/82/12/1360.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,314,776,780,3183,23550,27901,27902,77569,77600</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=25228897$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21665917$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Nagaishi, Akiko</creatorcontrib><creatorcontrib>Takagi, Mineo</creatorcontrib><creatorcontrib>Umemura, Atsushi</creatorcontrib><creatorcontrib>Tanaka, Masami</creatorcontrib><creatorcontrib>Kitagawa, Yoko</creatorcontrib><creatorcontrib>Matsui, Makoto</creatorcontrib><creatorcontrib>Nishizawa, Masatoyo</creatorcontrib><creatorcontrib>Sakimura, Kenji</creatorcontrib><creatorcontrib>Tanaka, Keiko</creatorcontrib><title>Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes</title><title>Journal of neurology, neurosurgery and psychiatry</title><addtitle>J Neurol Neurosurg Psychiatry</addtitle><description>ObjectiveTo analyse clinicoepidemiological features of neuromyelitis optica in a large cohort and to compare the differences between onset age, gender and clinical phenotypes.MethodsAntiaquaporin-4 antibody (AQP4-ab) levels were tested in 2366 serum samples of patients diagnosed as having central nervous system inflammatory demyelinating disorders by their referring physicians. AQP4-ab was measured by indirect immunofluorescence staining using human AQP4-transfected HEK 293 cells. A blinded analysis was performed and was combined with clinical information.ResultsA total of 583 patients (91.4% women) were AQP4-ab-positive. The average onset age was 42.9±15.9 years. According to MRI studies, spinal-cord lesions were detected in 85.3% of the patients, longitudinally extensive transverse myelitis in 72.7% and cerebral lesions in 51.1%. Unilateral or bilateral blindness was observed in 16.2% of patients, 19.8% were associated with Sjögren syndrome, and 13.6% were associated with thyroid diseases. Myelin basic protein was detected in the cerebrospinal fluid of 57.5% patients. In addition, men presented with an older onset age, a greater number of brainstem MRI lesions and positive myelin basic protein in the cerebrospinal fluid. All child-onset patients (<15 years, n=9) presented with optic neuritis as the first symptom, while older-onset patients presented with myelitis. Twenty patients initially developed limited brain lesions, and seven of these patients did not develop optic or spinal lesions during the 1–5-year follow-up period.ConclusionsThe clinical characteristics of AQP4-ab-positive patients were similar. However, optic neuritis was more common in paediatric patients, while myelitis was more common in older patients. A small number of patients exhibited only cerebral, brainstem, or cerebellar lesions during the initial several years and lower Extended Disability Status Scale scores.</description><subject>Adult</subject><subject>Age</subject><subject>Age of Onset</subject><subject>Aminoacid disorders</subject><subject>Antibodies</subject><subject>Antibodies - blood</subject><subject>Aquaporin 4 - immunology</subject><subject>Biological and medical sciences</subject><subject>Biomarkers - blood</subject><subject>Biomarkers - cerebrospinal fluid</subject><subject>Brain - pathology</subject><subject>Cohort Studies</subject><subject>CSF</subject><subject>Disease</subject><subject>Errors of metabolism</subject><subject>Female</subject><subject>Humans</subject><subject>infectious diseases</subject><subject>Japan - epidemiology</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Middle Aged</subject><subject>multiple sclerosis</subject><subject>Nervous system</subject><subject>Neuroimaging - methods</subject><subject>Neuroimmunology</subject><subject>Neurology</subject><subject>Neuromyelitis Optica - blood</subject><subject>Neuromyelitis Optica - cerebrospinal fluid</subject><subject>Neuromyelitis Optica - diagnosis</subject><subject>Neuromyelitis Optica - epidemiology</subject><subject>Neuromyelitis Optica - immunology</subject><subject>Neuromyelitis Optica - pathology</subject><subject>parkinson's disease</subject><subject>Patients</subject><subject>Phenotype</subject><subject>Sex Characteristics</subject><subject>Spinal cord</subject><subject>Spinal Cord - pathology</subject><subject>steroids</subject><subject>Thyroid gland</subject><issn>0022-3050</issn><issn>1468-330X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNqF0cuLFDEQB-Agiju7evckAREP0ppKOv3wJoPrg2WFxdctpDMVN2N30ibd6Pz3pulxBS-bS0LqqyLhR8gjYC8ARPVy7_1YcAZQCMZKJu6QDZRVUwjBvt0lG8Y4zxXJTshpSnu2rKa9T044VJVsod4Qs-2dd0b31KKe5oiJBks9zjEMB-zd5PLFOGVBnaea9jp-R_pBj9pjQmrCdYjTq7wPo44uBU87nH4hejpeow_TYcT0gNyzuk_48Lifkc_nbz5t3xUXH9--376-KDrJm6kwEiu2s5bVZidrWxrbMmFMazTaVhqDEpsd7yyYXWtYI1ipBYC1kI9gyk6ckWfr3DGGnzOmSQ0uGez7_NYwJ9UCQAWCw-2SCSjLuhZZPvlP7sMcff6GgroBXlYCqqzYqkwMKUW0aoxu0PGggKklKbUkpZak1JpUbnl8HDx3A-5uGv5Gk8HTI9Ap52Oj9salf05y3jTt4orVuTTh75u6jj9UVYtaqssvW3Upyysurr6qxT9ffTfsb3_mHzL4ucQ</recordid><startdate>20111201</startdate><enddate>20111201</enddate><creator>Nagaishi, Akiko</creator><creator>Takagi, Mineo</creator><creator>Umemura, Atsushi</creator><creator>Tanaka, Masami</creator><creator>Kitagawa, Yoko</creator><creator>Matsui, Makoto</creator><creator>Nishizawa, Masatoyo</creator><creator>Sakimura, Kenji</creator><creator>Tanaka, Keiko</creator><general>BMJ Publishing Group Ltd</general><general>BMJ Publishing Group</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>7TK</scope></search><sort><creationdate>20111201</creationdate><title>Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes</title><author>Nagaishi, Akiko ; Takagi, Mineo ; Umemura, Atsushi ; Tanaka, Masami ; Kitagawa, Yoko ; Matsui, Makoto ; Nishizawa, Masatoyo ; Sakimura, Kenji ; Tanaka, Keiko</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b528t-c5e60dff07cd57f4cf903cc9caef95cce5e8d2bf1cd9c08304a311ff18301c4b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Adult</topic><topic>Age</topic><topic>Age of Onset</topic><topic>Aminoacid disorders</topic><topic>Antibodies</topic><topic>Antibodies - blood</topic><topic>Aquaporin 4 - immunology</topic><topic>Biological and medical sciences</topic><topic>Biomarkers - blood</topic><topic>Biomarkers - cerebrospinal fluid</topic><topic>Brain - pathology</topic><topic>Cohort Studies</topic><topic>CSF</topic><topic>Disease</topic><topic>Errors of metabolism</topic><topic>Female</topic><topic>Humans</topic><topic>infectious diseases</topic><topic>Japan - epidemiology</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Middle Aged</topic><topic>multiple sclerosis</topic><topic>Nervous system</topic><topic>Neuroimaging - methods</topic><topic>Neuroimmunology</topic><topic>Neurology</topic><topic>Neuromyelitis Optica - blood</topic><topic>Neuromyelitis Optica - cerebrospinal fluid</topic><topic>Neuromyelitis Optica - diagnosis</topic><topic>Neuromyelitis Optica - epidemiology</topic><topic>Neuromyelitis Optica - immunology</topic><topic>Neuromyelitis Optica - pathology</topic><topic>parkinson's disease</topic><topic>Patients</topic><topic>Phenotype</topic><topic>Sex Characteristics</topic><topic>Spinal cord</topic><topic>Spinal Cord - pathology</topic><topic>steroids</topic><topic>Thyroid gland</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Nagaishi, Akiko</creatorcontrib><creatorcontrib>Takagi, Mineo</creatorcontrib><creatorcontrib>Umemura, Atsushi</creatorcontrib><creatorcontrib>Tanaka, Masami</creatorcontrib><creatorcontrib>Kitagawa, Yoko</creatorcontrib><creatorcontrib>Matsui, Makoto</creatorcontrib><creatorcontrib>Nishizawa, Masatoyo</creatorcontrib><creatorcontrib>Sakimura, Kenji</creatorcontrib><creatorcontrib>Tanaka, Keiko</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>Neurosciences Abstracts</collection><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Nagaishi, Akiko</au><au>Takagi, Mineo</au><au>Umemura, Atsushi</au><au>Tanaka, Masami</au><au>Kitagawa, Yoko</au><au>Matsui, Makoto</au><au>Nishizawa, Masatoyo</au><au>Sakimura, Kenji</au><au>Tanaka, Keiko</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes</atitle><jtitle>Journal of neurology, neurosurgery and psychiatry</jtitle><addtitle>J Neurol Neurosurg Psychiatry</addtitle><date>2011-12-01</date><risdate>2011</risdate><volume>82</volume><issue>12</issue><spage>1360</spage><epage>1364</epage><pages>1360-1364</pages><issn>0022-3050</issn><eissn>1468-330X</eissn><coden>JNNPAU</coden><abstract>ObjectiveTo analyse clinicoepidemiological features of neuromyelitis optica in a large cohort and to compare the differences between onset age, gender and clinical phenotypes.MethodsAntiaquaporin-4 antibody (AQP4-ab) levels were tested in 2366 serum samples of patients diagnosed as having central nervous system inflammatory demyelinating disorders by their referring physicians. AQP4-ab was measured by indirect immunofluorescence staining using human AQP4-transfected HEK 293 cells. A blinded analysis was performed and was combined with clinical information.ResultsA total of 583 patients (91.4% women) were AQP4-ab-positive. The average onset age was 42.9±15.9 years. According to MRI studies, spinal-cord lesions were detected in 85.3% of the patients, longitudinally extensive transverse myelitis in 72.7% and cerebral lesions in 51.1%. Unilateral or bilateral blindness was observed in 16.2% of patients, 19.8% were associated with Sjögren syndrome, and 13.6% were associated with thyroid diseases. Myelin basic protein was detected in the cerebrospinal fluid of 57.5% patients. In addition, men presented with an older onset age, a greater number of brainstem MRI lesions and positive myelin basic protein in the cerebrospinal fluid. All child-onset patients (<15 years, n=9) presented with optic neuritis as the first symptom, while older-onset patients presented with myelitis. Twenty patients initially developed limited brain lesions, and seven of these patients did not develop optic or spinal lesions during the 1–5-year follow-up period.ConclusionsThe clinical characteristics of AQP4-ab-positive patients were similar. However, optic neuritis was more common in paediatric patients, while myelitis was more common in older patients. A small number of patients exhibited only cerebral, brainstem, or cerebellar lesions during the initial several years and lower Extended Disability Status Scale scores.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd</pub><pmid>21665917</pmid><doi>10.1136/jnnp-2011-300403</doi><tpages>5</tpages></addata></record> |
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subjects | Adult Age Age of Onset Aminoacid disorders Antibodies Antibodies - blood Aquaporin 4 - immunology Biological and medical sciences Biomarkers - blood Biomarkers - cerebrospinal fluid Brain - pathology Cohort Studies CSF Disease Errors of metabolism Female Humans infectious diseases Japan - epidemiology Magnetic Resonance Imaging - methods Male Medical sciences Metabolic diseases Middle Aged multiple sclerosis Nervous system Neuroimaging - methods Neuroimmunology Neurology Neuromyelitis Optica - blood Neuromyelitis Optica - cerebrospinal fluid Neuromyelitis Optica - diagnosis Neuromyelitis Optica - epidemiology Neuromyelitis Optica - immunology Neuromyelitis Optica - pathology parkinson's disease Patients Phenotype Sex Characteristics Spinal cord Spinal Cord - pathology steroids Thyroid gland |
title | Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes |
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