MovDisordV3, PascalFrancis, Checkpoint, bibRecord, 000D99

Movement Disturbances in the Differential Diagnosis of Creutzfeldt-Jakob Disease

Identifieur interne : 000D99 ( PascalFrancis/Checkpoint ); précédent : 000D98; suivant : 000E00

Movement 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.

RBID : Pascal:09-0136793

Descripteurs français

Pascal (Inist)
- Démence d'Alzheimer, Ataxie, Pathologie du système nerveux, Diagnostic différentiel, Encéphalopathie spongiforme de Creutzfeldt-Jakob, Hypokinésie, Prion.

English descriptors

KwdEn :
- Alzheimer disease, Ataxia, Creutzfeldt-Jakob disease, Differential diagnostic, Hypokinesia, Nervous system diseases, Prion.

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.

Affiliations:

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Le document en format XML

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<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.</div>
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<fC07 i1="01" i2="X" l="SPA"><s0>Prion enfermedad</s0>
<s2>NM</s2>
</fC07>
<fC07 i1="02" i2="X" l="FRE"><s0>Infection</s0>
</fC07>
<fC07 i1="02" i2="X" l="ENG"><s0>Infection</s0>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Infección</s0>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Pathologie de l'encéphale</s0>
<s5>37</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Cerebral disorder</s0>
<s5>37</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Encéfalo patología</s0>
<s5>37</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Maladie dégénérative</s0>
<s5>38</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Degenerative disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0>
<s5>38</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE"><s0>Pathologie du   système nerveux central</s0>
<s5>39</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG"><s0>Central nervous system disease</s0>
<s5>39</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0>
<s5>39</s5>
</fC07>
<fC07 i1="06" i2="X" l="FRE"><s0>Trouble neurologique</s0>
<s5>41</s5>
</fC07>
<fC07 i1="06" i2="X" l="ENG"><s0>Neurological disorder</s0>
<s5>41</s5>
</fC07>
<fC07 i1="06" i2="X" l="SPA"><s0>Trastorno neurológico</s0>
<s5>41</s5>
</fC07>
<fC07 i1="07" i2="X" l="FRE"><s0>Trouble moteur</s0>
<s5>42</s5>
</fC07>
<fC07 i1="07" i2="X" l="ENG"><s0>Motor system disorder</s0>
<s5>42</s5>
</fC07>
<fC07 i1="07" i2="X" l="SPA"><s0>Trastorno motor</s0>
<s5>42</s5>
</fC07>
<fN21><s1>096</s1>
</fN21>
<fN44 i1="01"><s1>OTO</s1>
</fN44>
<fN82><s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
<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"><noRegion><name sortKey="Edler, Jan" sort="Edler, Jan" uniqKey="Edler J" first="Jan" last="Edler">Jan Edler</name>
</noRegion>
<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 Schaeffer, Walter J" sort="Schulz Schaeffer, Walter J" uniqKey="Schulz Schaeffer 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>
</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>

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	Movement Disorders (revue)
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Movement Disorders (revue)

Movement Disturbances in the Differential Diagnosis of Creutzfeldt-Jakob Disease

Movement Disturbances in the Differential Diagnosis of Creutzfeldt-Jakob Disease

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