Movement disturbances in the differential diagnosis of Creutzfeldt‐Jakob disease
Identifieur interne : 002201 ( Main/Exploration ); précédent : 002200; suivant : 002202Movement disturbances in the differential diagnosis of Creutzfeldt‐Jakob disease
Auteurs : Jan Edler [Allemagne] ; Brit Mollenhauer [États-Unis] ; Uta Heinemann [Allemagne] ; Daniela Varges [Allemagne] ; Carola Werner [Allemagne] ; Inga Zerr [Allemagne] ; Walter J. Schulz-Schaeffer [Allemagne]Source :
- Movement Disorders [ 0885-3185 ] ; 2009-02-15.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Adult, Aged, Aged, 80 and over, Alzheimer Disease (diagnosis), Alzheimer Disease (epidemiology), Alzheimer disease, Alzheimer's disease, Ataxia, Ataxia (diagnosis), Ataxia (epidemiology), Codon, Creutzfeldt-Jakob Syndrome (diagnosis), Creutzfeldt-Jakob Syndrome (epidemiology), Creutzfeldt-Jakob Syndrome (genetics), Creutzfeldt-Jakob disease, Diagnosis, Differential, Differential diagnostic, Female, Humans, Hypokinesia, Hypokinesia (diagnosis), Hypokinesia (epidemiology), Male, Middle Aged, Movement Disorders (diagnosis), Movement Disorders (epidemiology), Movement Disorders (genetics), Muscle Rigidity (diagnosis), Muscle Rigidity (epidemiology), Nervous system diseases, Phenotype, Polymorphism, Genetic, Prevalence, Prion, Prions (genetics), ataxia, cogwheel rigidity, dysmetria, hypokinesia, prion disease.
- MESH :
- chemical , genetics : Prions.
- chemical : Codon.
- diagnosis : Alzheimer Disease, Ataxia, Creutzfeldt-Jakob Syndrome, Hypokinesia, Movement Disorders, Muscle Rigidity.
- epidemiology : Alzheimer Disease, Ataxia, Creutzfeldt-Jakob Syndrome, Hypokinesia, Movement Disorders, Muscle Rigidity.
- genetics : Creutzfeldt-Jakob Syndrome, Movement Disorders.
- Adult, Aged, Aged, 80 and over, Diagnosis, Differential, Female, Humans, Male, Middle Aged, Phenotype, Polymorphism, Genetic, Prevalence.
Abstract
Movement disturbances are common in dementia disorders and are a central feature of the clinical classification criteria of Creutzfeldt‐Jakob disease (CJD). Polymorphism at codon 129 of the prion protein gene is known to determine the clinical picture of CJD. The frequency and characteristics of movement disturbances in other dementing disorders, such as Alzheimer's disease (AD), is barely known and leads to misdiagnoses. We investigated the occurrence and characteristics of movement disturbances in 143 patients neuropathologically confirmed with CJD (n = 100), AD (n = 29), dementia with Lewy bodies (DLB) (n = 7), or other diagnoses (n = 7). All patients had been referred with the differential diagnosis of prion disease. Ataxia and dysmetria were significantly more frequent in CJD than in AD or DLB patients, whereas hypokinesia was up to five times more frequent in AD or DLB (P < 0.05). Using an ordered logistic regression to identify constellations of movement disturbances, the diagnosis of CJD was likely in patients presenting ataxia but not hypokinesia. The reverse situation was statistically associated with AD. Ataxia and cogwheel rigidity were associated with valine‐homozygosity and akinesia with methionine‐homozygosity in the CJD patients. Our results indicate that the careful assessment of movement disturbances may be helpful in the differential diagnosis of Creutzfeldt‐Jakob disease. © 2008 Movement Disorder Society
Url:
DOI: 10.1002/mds.22253
Affiliations:
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Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, National Reference Center for TSE Surveillance, University Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation></author><author><name sortKey="Mollenhauer, Brit" sort="Mollenhauer, Brit" uniqKey="Mollenhauer B" first="Brit" last="Mollenhauer">Brit Mollenhauer</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Center for Neurologic Diseases, Department of Neurology, Brigham and Women's Hospital, Harvard Institutes of Medicine, Boston, Massachusetts</wicri:regionArea><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Heinemann, Uta" sort="Heinemann, Uta" uniqKey="Heinemann U" first="Uta" last="Heinemann">Uta Heinemann</name><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, National Reference Center for TSE Surveillance, University Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation></author><author><name sortKey="Varges, Daniela" sort="Varges, Daniela" uniqKey="Varges D" first="Daniela" last="Varges">Daniela Varges</name><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, National Reference Center for TSE Surveillance, University Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation></author><author><name sortKey="Werner, Carola" sort="Werner, Carola" uniqKey="Werner C" first="Carola" last="Werner">Carola Werner</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Institute of Medical Statistics of the University Medical Center Göttingen</wicri:regionArea></affiliation></author><author><name sortKey="Zerr, Inga" sort="Zerr, Inga" uniqKey="Zerr I" first="Inga" last="Zerr">Inga Zerr</name><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, National Reference Center for TSE Surveillance, University Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Inga Zerr, National Reference Center for TSE Surveillance, Dept of Neurology, University Medical Center Göttingen, Robert‐Koch‐Str. 40, 37075 Göttingen, GermanyWalter J. Schulz‐Schaeffer, Prion and Dementia Research Unit, Dept. of Neuropathology, University Medical Center Göttingen, Robert‐Koch‐Str. 40, 37075 Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation></author><author><name sortKey="Schulz Chaeffer, Walter J" sort="Schulz Chaeffer, Walter J" uniqKey="Schulz Chaeffer W" first="Walter J." last="Schulz-Schaeffer">Walter J. Schulz-Schaeffer</name><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neuropathology, Prion and Dementia Research Unit, University Medical Center, Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Inga Zerr, National Reference Center for TSE Surveillance, Dept of Neurology, University Medical Center Göttingen, Robert‐Koch‐Str. 40, 37075 Göttingen, GermanyWalter J. Schulz‐Schaeffer, Prion and Dementia Research Unit, Dept. of Neuropathology, University Medical Center Göttingen, Robert‐Koch‐Str. 40, 37075 Göttingen</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Göttingen</settlement></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</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="2009-02-15">2009-02-15</date><biblScope unit="vol">24</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="350">350</biblScope><biblScope unit="page" to="356">356</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">D3A12AD7488A2FEEDDBAB66E9AA71A012D893126</idno><idno type="DOI">10.1002/mds.22253</idno><idno type="ArticleID">MDS22253</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Alzheimer Disease (diagnosis)</term><term>Alzheimer Disease (epidemiology)</term><term>Alzheimer disease</term><term>Alzheimer's disease</term><term>Ataxia</term><term>Ataxia (diagnosis)</term><term>Ataxia (epidemiology)</term><term>Codon</term><term>Creutzfeldt-Jakob Syndrome (diagnosis)</term><term>Creutzfeldt-Jakob Syndrome (epidemiology)</term><term>Creutzfeldt-Jakob Syndrome (genetics)</term><term>Creutzfeldt-Jakob disease</term><term>Diagnosis, Differential</term><term>Differential diagnostic</term><term>Female</term><term>Humans</term><term>Hypokinesia</term><term>Hypokinesia (diagnosis)</term><term>Hypokinesia (epidemiology)</term><term>Male</term><term>Middle Aged</term><term>Movement Disorders (diagnosis)</term><term>Movement Disorders (epidemiology)</term><term>Movement Disorders (genetics)</term><term>Muscle Rigidity (diagnosis)</term><term>Muscle Rigidity (epidemiology)</term><term>Nervous system diseases</term><term>Phenotype</term><term>Polymorphism, Genetic</term><term>Prevalence</term><term>Prion</term><term>Prions (genetics)</term><term>ataxia</term><term>cogwheel rigidity</term><term>dysmetria</term><term>hypokinesia</term><term>prion disease</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Prions</term></keywords><keywords scheme="MESH" type="chemical" xml:lang="en"><term>Codon</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Alzheimer Disease</term><term>Ataxia</term><term>Creutzfeldt-Jakob Syndrome</term><term>Hypokinesia</term><term>Movement Disorders</term><term>Muscle Rigidity</term></keywords><keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Alzheimer Disease</term><term>Ataxia</term><term>Creutzfeldt-Jakob Syndrome</term><term>Hypokinesia</term><term>Movement Disorders</term><term>Muscle Rigidity</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Creutzfeldt-Jakob Syndrome</term><term>Movement Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Diagnosis, Differential</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Phenotype</term><term>Polymorphism, Genetic</term><term>Prevalence</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Ataxie</term><term>Diagnostic différentiel</term><term>Démence d'Alzheimer</term><term>Encéphalopathie spongiforme de Creutzfeldt-Jakob</term><term>Hypokinésie</term><term>Pathologie du système nerveux</term><term>Prion</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Movement disturbances are common in dementia disorders and are a central feature of the clinical classification criteria of Creutzfeldt‐Jakob disease (CJD). Polymorphism at codon 129 of the prion protein gene is known to determine the clinical picture of CJD. The frequency and characteristics of movement disturbances in other dementing disorders, such as Alzheimer's disease (AD), is barely known and leads to misdiagnoses. We investigated the occurrence and characteristics of movement disturbances in 143 patients neuropathologically confirmed with CJD (n = 100), AD (n = 29), dementia with Lewy bodies (DLB) (n = 7), or other diagnoses (n = 7). All patients had been referred with the differential diagnosis of prion disease. Ataxia and dysmetria were significantly more frequent in CJD than in AD or DLB patients, whereas hypokinesia was up to five times more frequent in AD or DLB (P < 0.05). Using an ordered logistic regression to identify constellations of movement disturbances, the diagnosis of CJD was likely in patients presenting ataxia but not hypokinesia. The reverse situation was statistically associated with AD. Ataxia and cogwheel rigidity were associated with valine‐homozygosity and akinesia with methionine‐homozygosity in the CJD patients. Our results indicate that the careful assessment of movement disturbances may be helpful in the differential diagnosis of Creutzfeldt‐Jakob disease. © 2008 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>États-Unis</li></country><region><li>Basse-Saxe</li><li>Massachusetts</li></region><settlement><li>Göttingen</li></settlement></list><tree><country name="Allemagne"><region name="Basse-Saxe"><name sortKey="Edler, Jan" sort="Edler, Jan" uniqKey="Edler J" first="Jan" last="Edler">Jan Edler</name></region><name sortKey="Edler, Jan" sort="Edler, Jan" uniqKey="Edler J" first="Jan" last="Edler">Jan Edler</name><name sortKey="Heinemann, Uta" sort="Heinemann, Uta" uniqKey="Heinemann U" first="Uta" last="Heinemann">Uta Heinemann</name><name sortKey="Schulz Chaeffer, Walter J" sort="Schulz Chaeffer, Walter J" uniqKey="Schulz Chaeffer W" first="Walter J." last="Schulz-Schaeffer">Walter J. Schulz-Schaeffer</name><name sortKey="Schulz Chaeffer, Walter J" sort="Schulz Chaeffer, Walter J" uniqKey="Schulz Chaeffer W" first="Walter J." last="Schulz-Schaeffer">Walter J. Schulz-Schaeffer</name><name sortKey="Varges, Daniela" sort="Varges, Daniela" uniqKey="Varges D" first="Daniela" last="Varges">Daniela Varges</name><name sortKey="Werner, Carola" sort="Werner, Carola" uniqKey="Werner C" first="Carola" last="Werner">Carola Werner</name><name sortKey="Zerr, Inga" sort="Zerr, Inga" uniqKey="Zerr I" first="Inga" last="Zerr">Inga Zerr</name><name sortKey="Zerr, Inga" sort="Zerr, Inga" uniqKey="Zerr I" first="Inga" last="Zerr">Inga Zerr</name></country><country name="États-Unis"><region name="Massachusetts"><name sortKey="Mollenhauer, Brit" sort="Mollenhauer, Brit" uniqKey="Mollenhauer B" first="Brit" last="Mollenhauer">Brit Mollenhauer</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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