Dentatorubral and pallidoluysian atrophy (DRPLA) Clinical and neuropathological findings in genetically confirmed north american and european pedigrees
Identifieur interne : 005366 ( Main/Exploration ); précédent : 005365; suivant : 005367Dentatorubral and pallidoluysian atrophy (DRPLA) Clinical and neuropathological findings in genetically confirmed north american and european pedigrees
Auteurs : M. W. Becher [Royaume-Uni] ; D. C. Rubinsztein [Royaume-Uni] ; J. Leggo [Royaume-Uni] ; M. V. Wagster [Royaume-Uni] ; O. C. Stine [Royaume-Uni] ; N. G. Ranen [Royaume-Uni] ; M. L. Franz [Royaume-Uni] ; M. H. Abbott [Royaume-Uni, États-Unis] ; M. Sherr [Royaume-Uni] ; J. C. Macmillan ; L. Barron [Royaume-Uni] ; M. Porteous [Royaume-Uni] ; P. S. Harper ; Ross [Royaume-Uni, États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 1997-07.
Descripteurs français
- Pascal (Inist)
- Wicri :
- geographic : États-Unis.
- topic : Homme.
English descriptors
- KwdEn :
- Adult, African Continental Ancestry Group (genetics), Atrophia, Atrophy, Brain (pathology), Brain (physiopathology), Brain Diseases (diagnosis), Brain Diseases (ethnology), Brain Diseases (genetics), Brain Diseases (physiopathology), Case study, Child, Chromosomes, Human, Pair 12 (genetics), Dentate Gyrus (pathology), Dentate nucleus, Dentatorubral and pallidoluysian atrophy, Diagnosis, Differential, Europe, European Continental Ancestry Group (genetics), Exploration, Family study, Female, Globus Pallidus (pathology), Great Britain, Human, Humans, Huntington's disease, Infant, Male, Middle Aged, Movement Disorders (diagnosis), Movement Disorders (ethnology), Movement Disorders (genetics), Movement Disorders (physiopathology), Nerve Degeneration (genetics), North America, Pallidum, Pathology, Pedigree, Phenotype, Red Nucleus (pathology), Red nucleus, Subthalamic nucleus, Trinucleotide Repeats, United States.
- MESH :
- geographic : Great Britain, United States.
- diagnosis : Brain Diseases, Movement Disorders.
- ethnology : Brain Diseases, Movement Disorders.
- genetics : African Continental Ancestry Group, Brain Diseases, Chromosomes, Human, Pair 12, European Continental Ancestry Group, Movement Disorders, Nerve Degeneration.
- pathology : Brain, Dentate Gyrus, Globus Pallidus, Red Nucleus.
- physiopathology : Brain, Brain Diseases, Movement Disorders.
- Adult, Atrophy, Child, Diagnosis, Differential, Female, Humans, Infant, Male, Middle Aged, Pedigree, Phenotype, Trinucleotide Repeats.
Abstract
Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant disorder that clinically overlaps with Huntington's disease (HD) and manifests combinations of chorea, myoclonus, seizures, ataxia, and dementia. DRPLA is caused by a CAG triplet repeat (CTG‐B37) expansion coding for polyglutamine on chromosome 12 and exhibits the genetic phenomenon of anticipation. This neurodegenerative disease has only rarely been reported in non‐Japanese pedigrees, and there are only a few neuropathological studies in genetically confirmed patients. We report 10 cases of DRPLA from two North American and two British pedigrees in which CTG‐B37 expansions have been demonstrated within each kindred (54‐83 repeats), individually in 8 of the 10 cases, and describe the neuropathological findings in 4 cases. Members of DRPLA kindreds have a wide range of clinical phenotypes and markedly variable ages at onset. The neuropathological spectrum is centered around the cerebellifugal and pallidofugal systems, but neurodegenerative changes can be found in many nuclei, tracts, and systems. Evidence of CTG‐B37 triplet repeat expansion should be sought in HD‐like cases that are negative for expanded triplet repeats within the HD IT15 gene or in autopsy cases with degeneration of the dentatorubral or pallidoluysian systems.
Url:
DOI: 10.1002/mds.870120408
Affiliations:
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W." last="Becher">M. W. Becher</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Pathology (Neuropathology Division) Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Rubinsztein, D C" sort="Rubinsztein, D C" uniqKey="Rubinsztein D" first="D. C." last="Rubinsztein">D. C. Rubinsztein</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>Department of East Anglian Regional Genetics Service Molecular Genetics Laboratory, Cambridge University, Cambridge</wicri:cityArea></affiliation></author><author><name sortKey="Leggo, J" sort="Leggo, J" uniqKey="Leggo J" first="J." last="Leggo">J. Leggo</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>Department of East Anglian Regional Genetics Service Molecular Genetics Laboratory, Cambridge University, Cambridge</wicri:cityArea></affiliation></author><author><name sortKey="Wagster, M V" sort="Wagster, M V" uniqKey="Wagster M" first="M. V." last="Wagster">M. V. Wagster</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Pathology (Neuropathology Division) Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Stine, O C" sort="Stine, O C" uniqKey="Stine O" first="O. C." last="Stine">O. C. Stine</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Psychiatry, Western General Hospital Edinburgh, Scotland, Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Ranen, N G" sort="Ranen, N G" uniqKey="Ranen N" first="N. G." last="Ranen">N. G. Ranen</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Psychiatry, Western General Hospital Edinburgh, Scotland, Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Franz, M L" sort="Franz, M L" uniqKey="Franz M" first="M. L." last="Franz">M. L. Franz</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Psychiatry, Western General Hospital Edinburgh, Scotland, Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Abbott, M H" sort="Abbott, M H" uniqKey="Abbott M" first="M. H." last="Abbott">M. H. Abbott</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Psychiatry, Western General Hospital Edinburgh, Scotland, Western General Hospital Edinburgh</wicri:cityArea></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Sherr, M" sort="Sherr, M" uniqKey="Sherr M" first="M." last="Sherr">M. Sherr</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Pathology (Neuropathology Division) Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Macmillan, J C" sort="Macmillan, J C" uniqKey="Macmillan J" first="J. C." last="Macmillan">J. C. Macmillan</name><affiliation><wicri:noCountry code="subField">Wales</wicri:noCountry></affiliation></author><author><name sortKey="Barron, L" sort="Barron, L" uniqKey="Barron L" first="L." last="Barron">L. Barron</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Human Genetics Unit, University of Edinburgh, Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Porteous, M" sort="Porteous, M" uniqKey="Porteous M" first="M." last="Porteous">M. Porteous</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Human Genetics Unit, University of Edinburgh, Western General Hospital Edinburgh</wicri:cityArea></affiliation></author><author><name sortKey="Harper, P S" sort="Harper, P S" uniqKey="Harper P" first="P. S." last="Harper">P. S. Harper</name><affiliation><wicri:noCountry code="subField">Wales</wicri:noCountry></affiliation></author><author><name sortKey="Ross" sort="Ross" uniqKey="Ross" last="Ross">Ross</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Écosse</region></placeName><wicri:cityArea>Department of Psychiatry, Western General Hospital Edinburgh, Scotland, Western General Hospital Edinburgh</wicri:cityArea></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Laboratory of Molecular Neurobiology, Johns Hopkins University School of Medicine, Baltimore, Maryland</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1997-07">1997-07</date><biblScope unit="vol">12</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="519">519</biblScope><biblScope unit="page" to="530">530</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">19DC31CF6C016C262FBC79B78D73E02B9290C841</idno><idno type="DOI">10.1002/mds.870120408</idno><idno type="ArticleID">MDS870120408</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>African Continental Ancestry Group (genetics)</term><term>Atrophia</term><term>Atrophy</term><term>Brain (pathology)</term><term>Brain (physiopathology)</term><term>Brain Diseases (diagnosis)</term><term>Brain Diseases (ethnology)</term><term>Brain Diseases (genetics)</term><term>Brain Diseases (physiopathology)</term><term>Case study</term><term>Child</term><term>Chromosomes, Human, Pair 12 (genetics)</term><term>Dentate Gyrus (pathology)</term><term>Dentate nucleus</term><term>Dentatorubral and pallidoluysian atrophy</term><term>Diagnosis, Differential</term><term>Europe</term><term>European Continental Ancestry Group (genetics)</term><term>Exploration</term><term>Family study</term><term>Female</term><term>Globus Pallidus (pathology)</term><term>Great Britain</term><term>Human</term><term>Humans</term><term>Huntington's disease</term><term>Infant</term><term>Male</term><term>Middle Aged</term><term>Movement Disorders (diagnosis)</term><term>Movement Disorders (ethnology)</term><term>Movement Disorders (genetics)</term><term>Movement Disorders (physiopathology)</term><term>Nerve Degeneration (genetics)</term><term>North America</term><term>Pallidum</term><term>Pathology</term><term>Pedigree</term><term>Phenotype</term><term>Red Nucleus (pathology)</term><term>Red nucleus</term><term>Subthalamic nucleus</term><term>Trinucleotide Repeats</term><term>United States</term></keywords><keywords scheme="MESH" type="geographic" xml:lang="en"><term>Great Britain</term><term>United States</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Brain Diseases</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="ethnology" xml:lang="en"><term>Brain Diseases</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>African Continental Ancestry Group</term><term>Brain Diseases</term><term>Chromosomes, Human, Pair 12</term><term>European Continental Ancestry Group</term><term>Movement Disorders</term><term>Nerve Degeneration</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term><term>Dentate Gyrus</term><term>Globus Pallidus</term><term>Red Nucleus</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain</term><term>Brain Diseases</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Atrophy</term><term>Child</term><term>Diagnosis, Differential</term><term>Female</term><term>Humans</term><term>Infant</term><term>Male</term><term>Middle Aged</term><term>Pedigree</term><term>Phenotype</term><term>Trinucleotide Repeats</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Amérique du Nord</term><term>Anatomopathologie</term><term>Atrophie</term><term>Etude cas</term><term>Etude familiale</term><term>Europe</term><term>Exploration</term><term>Homme</term><term>Noyau dentelé</term><term>Noyau rouge</term><term>Noyau sousthalamique</term><term>Pallidum</term></keywords><keywords scheme="Wicri" type="geographic" xml:lang="fr"><term>États-Unis</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant disorder that clinically overlaps with Huntington's disease (HD) and manifests combinations of chorea, myoclonus, seizures, ataxia, and dementia. DRPLA is caused by a CAG triplet repeat (CTG‐B37) expansion coding for polyglutamine on chromosome 12 and exhibits the genetic phenomenon of anticipation. This neurodegenerative disease has only rarely been reported in non‐Japanese pedigrees, and there are only a few neuropathological studies in genetically confirmed patients. We report 10 cases of DRPLA from two North American and two British pedigrees in which CTG‐B37 expansions have been demonstrated within each kindred (54‐83 repeats), individually in 8 of the 10 cases, and describe the neuropathological findings in 4 cases. Members of DRPLA kindreds have a wide range of clinical phenotypes and markedly variable ages at onset. The neuropathological spectrum is centered around the cerebellifugal and pallidofugal systems, but neurodegenerative changes can be found in many nuclei, tracts, and systems. Evidence of CTG‐B37 triplet repeat expansion should be sought in HD‐like cases that are negative for expanded triplet repeats within the HD IT15 gene or in autopsy cases with degeneration of the dentatorubral or pallidoluysian systems.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Maryland</li><li>Écosse</li></region></list><tree><noCountry><name sortKey="Harper, P S" sort="Harper, P S" uniqKey="Harper P" first="P. S." last="Harper">P. S. Harper</name><name sortKey="Macmillan, J C" sort="Macmillan, J C" uniqKey="Macmillan J" first="J. C." last="Macmillan">J. C. Macmillan</name></noCountry><country name="Royaume-Uni"><region name="Écosse"><name sortKey="Becher, M W" sort="Becher, M W" uniqKey="Becher M" first="M. W." last="Becher">M. W. Becher</name></region><name sortKey="Abbott, M H" sort="Abbott, M H" uniqKey="Abbott M" first="M. H." last="Abbott">M. H. Abbott</name><name sortKey="Barron, L" sort="Barron, L" uniqKey="Barron L" first="L." last="Barron">L. Barron</name><name sortKey="Franz, M L" sort="Franz, M L" uniqKey="Franz M" first="M. L." last="Franz">M. L. Franz</name><name sortKey="Leggo, J" sort="Leggo, J" uniqKey="Leggo J" first="J." last="Leggo">J. Leggo</name><name sortKey="Porteous, M" sort="Porteous, M" uniqKey="Porteous M" first="M." last="Porteous">M. Porteous</name><name sortKey="Ranen, N G" sort="Ranen, N G" uniqKey="Ranen N" first="N. G." last="Ranen">N. G. Ranen</name><name sortKey="Ross" sort="Ross" uniqKey="Ross" last="Ross">Ross</name><name sortKey="Rubinsztein, D C" sort="Rubinsztein, D C" uniqKey="Rubinsztein D" first="D. C." last="Rubinsztein">D. C. Rubinsztein</name><name sortKey="Sherr, M" sort="Sherr, M" uniqKey="Sherr M" first="M." last="Sherr">M. Sherr</name><name sortKey="Stine, O C" sort="Stine, O C" uniqKey="Stine O" first="O. C." last="Stine">O. C. Stine</name><name sortKey="Wagster, M V" sort="Wagster, M V" uniqKey="Wagster M" first="M. V." last="Wagster">M. V. Wagster</name></country><country name="États-Unis"><region name="Maryland"><name sortKey="Abbott, M H" sort="Abbott, M H" uniqKey="Abbott M" first="M. H." last="Abbott">M. H. Abbott</name></region><name sortKey="Ross" sort="Ross" uniqKey="Ross" last="Ross">Ross</name><name sortKey="Ross" sort="Ross" uniqKey="Ross" last="Ross">Ross</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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