Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry
Identifieur interne : 001706 ( Istex/Corpus ); précédent : 001705; suivant : 001707Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry
Auteurs : Bala V. Manyam ; Arthur S. Walters ; Koteswara R. NarlaSource :
- Movement Disorders [ 0885-3185 ] ; 2001-03.
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- KwdEn :
Abstract
Clinical features in bilateral striopallidodentate calcinosis (BSPDC), popularly referred to as Fahr's disease (five autosomal dominant families and eight sporadic cases, n = 38), recruited through a registry, are reported. Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others. © 2001 Movement Disorder Society.
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DOI: 10.1002/mds.1049
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Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others. © 2001 Movement Disorder Society.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Bala V. Manyam MD</name><affiliations><json:string>Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas, USA</json:string></affiliations></json:item><json:item><name>Arthur S. Walters MD</name><affiliations><json:string>New Jersey Neuroscience Institute at JFK Medical Center, and Department of Neuroscience, Seton Hall University School of Graduate Medical Education, Edison, New Jersey, USA</json:string></affiliations></json:item><json:item><name>Koteswara R. 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Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. 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Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. 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Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others. © 2001 Movement Disorder Society.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Bilateral Striopallidodentate Calcinosis</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry</title></titleInfo><name type="personal"><namePart type="given">Bala V.</namePart><namePart type="family">Manyam</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas, USA</affiliation><description>Correspondence: Department of Neurology, Scott & White Clinic, Texas A&M Unversity, College of Medicine, 2401 South 31st Street, Temple, TX 76508</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Arthur S.</namePart><namePart type="family">Walters</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>New Jersey Neuroscience Institute at JFK Medical Center, and Department of Neuroscience, Seton Hall University School of Graduate Medical Education, Edison, New Jersey, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Koteswara R.</namePart><namePart type="family">Narla</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Southern Illinois University School of Medicine, Springfield, Illinois, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">article</genre><originInfo><publisher>John Wiley & Sons, Inc.</publisher><place><placeTerm type="text">New York</placeTerm></place><dateIssued encoding="w3cdtf">2001-03</dateIssued><dateCaptured encoding="w3cdtf">1998-08-10</dateCaptured><dateValid encoding="w3cdtf">2000-09-02</dateValid><copyrightDate encoding="w3cdtf">2001</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">3</extent><extent unit="tables">1</extent><extent unit="references">48</extent><extent unit="words">3362</extent></physicalDescription><abstract lang="en">Clinical features in bilateral striopallidodentate calcinosis (BSPDC), popularly referred to as Fahr's disease (five autosomal dominant families and eight sporadic cases, n = 38), recruited through a registry, are reported. Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (± S.D.) age of Registry patients was 43 ± 21 and that of literature was 38 ± 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others. © 2001 Movement Disorder Society.</abstract><subject lang="en"><genre>Keywords</genre><topic>bilateral striopallidodentate calcinosis (BSPDC)</topic><topic>Fahr's disease</topic><topic>basal ganglia</topic><topic>calcium</topic><topic>computed tomography</topic><topic>electronic planimeter</topic><topic>coordinate digitizer</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title><subTitle>Official Journal of the Movement Disorder Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><subject><genre>article category</genre><topic>Article</topic></subject><identifier type="ISSN">0885-3185</identifier><identifier type="eISSN">1531-8257</identifier><identifier type="DOI">10.1002/(ISSN)1531-8257</identifier><identifier type="PublisherID">MDS</identifier><part><date>2001</date><detail type="volume"><caption>vol.</caption><number>16</number></detail><detail type="issue"><caption>no.</caption><number>2</number></detail><extent unit="pages"><start>258</start><end>264</end><total>7</total></extent></part></relatedItem><identifier type="istex">C98609A64894CECEF7DE5368B3E4A897CB967BBF</identifier><identifier type="DOI">10.1002/mds.1049</identifier><identifier type="ArticleID">MDS1049</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2001 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>John Wiley & Sons, Inc.</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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