Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry.
Identifieur interne : 003E06 ( PubMed/Checkpoint ); précédent : 003E05; suivant : 003E07Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry.
Auteurs : B V Manyam [États-Unis] ; A S Walters ; K R NarlaSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2001.
English descriptors
- KwdEn :
- Adult, Age Distribution, Atrophy (pathology), Brain Diseases (complications), Brain Diseases (diagnosis), Brain Diseases (epidemiology), Calcinosis (diagnosis), Calcinosis (epidemiology), Calcinosis (genetics), Corpus Striatum (pathology), Corpus Striatum (radiography), Dentate Gyrus (pathology), Dentate Gyrus (radiography), Female, Functional Laterality (physiology), Globus Pallidus (pathology), Globus Pallidus (radiography), Humans, Male, Middle Aged, Movement Disorders (diagnosis), Movement Disorders (epidemiology), Movement Disorders (etiology), Registries, Severity of Illness Index, Thalamus (pathology), Tomography, X-Ray Computed.
- MESH :
- complications : Brain Diseases.
- diagnosis : Brain Diseases, Calcinosis, Movement Disorders.
- epidemiology : Brain Diseases, Calcinosis, Movement Disorders.
- etiology : Movement Disorders.
- genetics : Calcinosis.
- pathology : Atrophy, Corpus Striatum, Dentate Gyrus, Globus Pallidus, Thalamus.
- physiology : Functional Laterality.
- radiography : Corpus Striatum, Dentate Gyrus, Globus Pallidus.
- Adult, Age Distribution, Female, Humans, Male, Middle Aged, Registries, Severity of Illness Index, Tomography, X-Ray Computed.
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.
PubMed: 11295778
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sortKey="Narla, K R" sort="Narla, K R" uniqKey="Narla K" first="K R" last="Narla">K R Narla</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2001">2001</date><idno type="RBID">pubmed:11295778</idno><idno type="pmid">11295778</idno><idno type="wicri:Area/PubMed/Corpus">003D83</idno><idno type="wicri:Area/PubMed/Curation">003D83</idno><idno type="wicri:Area/PubMed/Checkpoint">003E06</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry.</title><author><name sortKey="Manyam, B V" sort="Manyam, B V" uniqKey="Manyam B" first="B V" last="Manyam">B V Manyam</name><affiliation wicri:level="1"><nlm:affiliation>Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas 76508, USA. bmanyam@swmail.sw.org</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas 76508</wicri:regionArea><wicri:noRegion>Texas 76508</wicri:noRegion></affiliation></author><author><name sortKey="Walters, A S" sort="Walters, A S" uniqKey="Walters A" first="A S" last="Walters">A S Walters</name></author><author><name sortKey="Narla, K R" sort="Narla, K R" uniqKey="Narla K" first="K R" last="Narla">K R Narla</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2001" type="published">2001</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Age Distribution</term><term>Atrophy (pathology)</term><term>Brain Diseases 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xml:lang="en"><term>Brain Diseases</term><term>Calcinosis</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Brain Diseases</term><term>Calcinosis</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Calcinosis</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Atrophy</term><term>Corpus Striatum</term><term>Dentate Gyrus</term><term>Globus Pallidus</term><term>Thalamus</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Functional Laterality</term></keywords><keywords scheme="MESH" qualifier="radiography" xml:lang="en"><term>Corpus Striatum</term><term>Dentate Gyrus</term><term>Globus Pallidus</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Age Distribution</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Registries</term><term>Severity of Illness Index</term><term>Tomography, X-Ray Computed</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml: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.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">11295778</PMID><DateCreated><Year>2001</Year><Month>04</Month><Day>11</Day></DateCreated><DateCompleted><Year>2001</Year><Month>08</Month><Day>30</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>16</Volume><Issue>2</Issue><PubDate><Year>2001</Year><Month>Mar</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry.</ArticleTitle><Pagination><MedlinePgn>258-64</MedlinePgn></Pagination><Abstract><AbstractText>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.</AbstractText><CopyrightInformation>Copyright 2001 Movement Disorder Society.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Manyam</LastName><ForeName>B V</ForeName><Initials>BV</Initials><AffiliationInfo><Affiliation>Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas 76508, USA. bmanyam@swmail.sw.org</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Walters</LastName><ForeName>A S</ForeName><Initials>AS</Initials></Author><Author ValidYN="Y"><LastName>Narla</LastName><ForeName>K R</ForeName><Initials>KR</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000328">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D017677">Age Distribution</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001284">Atrophy</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001927">Brain Diseases</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000150">complications</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000453">epidemiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D002114">Calcinosis</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000453">epidemiology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000235">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003342">Corpus Striatum</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000530">radiography</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D018891">Dentate Gyrus</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000530">radiography</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005260">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D007839">Functional Laterality</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005917">Globus Pallidus</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000530">radiography</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008875">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009069">Movement Disorders</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000453">epidemiology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000209">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D012042">Registries</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012720">Severity of Illness Index</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013788">Thalamus</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014057">Tomography, X-Ray Computed</DescriptorName></MeshHeading></MeshHeadingList><NumberOfReferences>48</NumberOfReferences></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2001</Year><Month>4</Month><Day>11</Day><Hour>10</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2001</Year><Month>8</Month><Day>31</Day><Hour>10</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2001</Year><Month>4</Month><Day>11</Day><Hour>10</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">11295778</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>États-Unis</li></country></list><tree><noCountry><name sortKey="Narla, K R" sort="Narla, K R" uniqKey="Narla K" first="K R" last="Narla">K R Narla</name><name sortKey="Walters, A S" sort="Walters, A S" uniqKey="Walters A" first="A S" last="Walters">A S Walters</name></noCountry><country name="États-Unis"><noRegion><name sortKey="Manyam, B V" sort="Manyam, B V" uniqKey="Manyam B" first="B V" last="Manyam">B V Manyam</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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