MovDisordV3, PascalFrancis, Corpus, bibRecord, 000542

Rapidly Progressive Diffuse Lewy Body Disease

Identifieur interne : 000542 ( PascalFrancis/Corpus ); précédent : 000541; suivant : 000543

Rapidly Progressive Diffuse Lewy Body Disease

Auteurs : Carles Gaig ; Francesc Valldeoriola ; Ellen Gelpi ; Mario Ezquerra ; Sara Llufriu ; Mariateresa Buongiorno ; Maria Jesus Rey ; Maria Jose Marti ; Francesc Graus ; Eduardo Tolosa

Source :

Movement disorders [ 0885-3185 ] ; 2011.

RBID : Pascal:11-0321224

Descripteurs français

Pascal (Inist)
- Démence à corps de Lewy, Pathologie du système nerveux, Corps Lewy, Encéphalopathie spongiforme de Creutzfeldt-Jakob, Prion, Corps Lewy maladie.

English descriptors

KwdEn :
- Creutzfeldt-Jakob disease, Lewy body, Lewy body dementia, Lewy body disease, Nervous system diseases, Prion.

Abstract

Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial ¹²³I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as ¹²³I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

A01	`01`	`1`		`@0 0885-3185`
A03		`1`		`@0 Mov. disord.`
A05				`@2 26`
A06				`@2 7`
A08	`01`	`1`	`ENG`	`@1 Rapidly Progressive Diffuse Lewy Body Disease`
A11	`01`	`1`		`@1 GAIG (Carles)`
A11	`02`	`1`		`@1 VALLDEORIOLA (Francesc)`
A11	`03`	`1`		`@1 GELPI (Ellen)`
A11	`04`	`1`		`@1 EZQUERRA (Mario)`
A11	`05`	`1`		`@1 LLUFRIU (Sara)`
A11	`06`	`1`		`@1 BUONGIORNO (Mariateresa)`
A11	`07`	`1`		`@1 JESUS REY (Maria)`
A11	`08`	`1`		`@1 JOSE MARTI (Maria)`
A11	`09`	`1`		`@1 GRAUS (Francesc)`
A11	`10`	`1`		`@1 TOLOSA (Eduardo)`
A14	`01`			`@1 Neurology Service, Hospital Clinic of Barcelona @2 Barcelona @3 ESP @Z 1 aut. @Z 2 aut. @Z 4 aut. @Z 5 aut. @Z 6 aut. @Z 8 aut. @Z 9 aut. @Z 10 aut.`
A14	`02`			`@1 Neurological Tissue Bank, Universitat of Barcelona-Hospital Clinic @2 Barcelona @3 ESP @Z 1 aut. @Z 3 aut. @Z 7 aut. @Z 9 aut.`
A20				`@1 1316-1323`
A21				`@1 2011`
A23	`01`			`@0 ENG`
A43	`01`			`@1 INIST @2 20953 @5 354000190480410180`
A44				`@0 0000 @1 © 2011 INIST-CNRS. All rights reserved.`
A45				`@0 36 ref.`
A47	`01`	`1`		`@0 11-0321224`
A60				`@1 P`
A61				`@0 A`
A64	`01`	`1`		`@0 Movement disorders`
A66	`01`			`@0 USA`
C01	`01`		`ENG`	@0 Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial ¹²³I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as ¹²³I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.
C02	`01`	`X`		`@0 002B17`
C02	`02`	`X`		`@0 002B17G`
C03	`01`	`X`	`FRE`	`@0 Démence à corps de Lewy @2 NM @5 01`
C03	`01`	`X`	`ENG`	`@0 Lewy body dementia @2 NM @5 01`
C03	`01`	`X`	`SPA`	`@0 Demencia cuerpos Lewy @2 NM @5 01`
C03	`02`	`X`	`FRE`	`@0 Pathologie du système nerveux @5 02`
C03	`02`	`X`	`ENG`	`@0 Nervous system diseases @5 02`
C03	`02`	`X`	`SPA`	`@0 Sistema nervioso patología @5 02`
C03	`03`	`X`	`FRE`	`@0 Corps Lewy @5 09`
C03	`03`	`X`	`ENG`	`@0 Lewy body @5 09`
C03	`03`	`X`	`SPA`	`@0 Cuerpo Lewy @5 09`
C03	`04`	`X`	`FRE`	`@0 Encéphalopathie spongiforme de Creutzfeldt-Jakob @5 10`
C03	`04`	`X`	`ENG`	`@0 Creutzfeldt-Jakob disease @5 10`
C03	`04`	`X`	`SPA`	`@0 Encefalopatía espongiforme Creutzfeldt-Jakob @5 10`
C03	`05`	`X`	`FRE`	`@0 Prion @5 78`
C03	`05`	`X`	`ENG`	`@0 Prion @5 78`
C03	`05`	`X`	`SPA`	`@0 Prion @5 78`
C03	`06`	`X`	`FRE`	`@0 Corps Lewy maladie @4 CD @5 96`
C03	`06`	`X`	`ENG`	`@0 Lewy body disease @4 CD @5 96`
C03	`06`	`X`	`SPA`	`@0 Cuerpo Lewy enfermedad @4 CD @5 96`
C07	`01`	`X`	`FRE`	`@0 Maladie à prions @2 NM`
C07	`01`	`X`	`ENG`	`@0 Prion disease @2 NM`
C07	`01`	`X`	`SPA`	`@0 Prion enfermedad @2 NM`
C07	`02`	`X`	`FRE`	`@0 Infection`
C07	`02`	`X`	`ENG`	`@0 Infection`
C07	`02`	`X`	`SPA`	`@0 Infección`
C07	`03`	`X`	`FRE`	`@0 Pathologie de l'encéphale @5 37`
C07	`03`	`X`	`ENG`	`@0 Cerebral disorder @5 37`
C07	`03`	`X`	`SPA`	`@0 Encéfalo patología @5 37`
C07	`04`	`X`	`FRE`	`@0 Maladie dégénérative @5 38`
C07	`04`	`X`	`ENG`	`@0 Degenerative disease @5 38`
C07	`04`	`X`	`SPA`	`@0 Enfermedad degenerativa @5 38`
C07	`05`	`X`	`FRE`	`@0 Pathologie du système nerveux central @5 39`
C07	`05`	`X`	`ENG`	`@0 Central nervous system disease @5 39`
C07	`05`	`X`	`SPA`	`@0 Sistema nervosio central patología @5 39`
N21				`@1 220`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

Format Inist (serveur)

NO :	PASCAL 11-0321224 INIST
ET :	Rapidly Progressive Diffuse Lewy Body Disease
AU :	GAIG (Carles); VALLDEORIOLA (Francesc); GELPI (Ellen); EZQUERRA (Mario); LLUFRIU (Sara); BUONGIORNO (Mariateresa); JESUS REY (Maria); JOSE MARTI (Maria); GRAUS (Francesc); TOLOSA (Eduardo)
AF :	Neurology Service, Hospital Clinic of Barcelona/Barcelona/Espagne (1 aut., 2 aut., 4 aut., 5 aut., 6 aut., 8 aut., 9 aut., 10 aut.); Neurological Tissue Bank, Universitat of Barcelona-Hospital Clinic/Barcelona/Espagne (1 aut., 3 aut., 7 aut., 9 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2011; Vol. 26; No. 7; Pp. 1316-1323; Bibl. 36 ref.
LA :	Anglais
EA :	Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial ¹²³I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as ¹²³I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.
CC :	002B17; 002B17G
FD :	Démence à corps de Lewy; Pathologie du système nerveux; Corps Lewy; Encéphalopathie spongiforme de Creutzfeldt-Jakob; Prion; Corps Lewy maladie
FG :	Maladie à prions; Infection; Pathologie de l'encéphale; Maladie dégénérative; Pathologie du système nerveux central
ED :	Lewy body dementia; Nervous system diseases; Lewy body; Creutzfeldt-Jakob disease; Prion; Lewy body disease
EG :	Prion disease; Infection; Cerebral disorder; Degenerative disease; Central nervous system disease
SD :	Demencia cuerpos Lewy; Sistema nervioso patología; Cuerpo Lewy; Encefalopatía espongiforme Creutzfeldt-Jakob; Prion; Cuerpo Lewy enfermedad
LO :	INIST-20953.354000190480410180
ID :	11-0321224

Links to Exploration step

Pascal:11-0321224

Le document en format XML

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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Rapidly Progressive Diffuse Lewy Body Disease</title>
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<author><name sortKey="Valldeoriola, Francesc" sort="Valldeoriola, Francesc" uniqKey="Valldeoriola F" first="Francesc" last="Valldeoriola">Francesc Valldeoriola</name>
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<author><name sortKey="Llufriu, Sara" sort="Llufriu, Sara" uniqKey="Llufriu S" first="Sara" last="Llufriu">Sara Llufriu</name>
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<author><name sortKey="Buongiorno, Mariateresa" sort="Buongiorno, Mariateresa" uniqKey="Buongiorno M" first="Mariateresa" last="Buongiorno">Mariateresa Buongiorno</name>
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<author><name sortKey="Jesus Rey, Maria" sort="Jesus Rey, Maria" uniqKey="Jesus Rey M" first="Maria" last="Jesus Rey">Maria Jesus Rey</name>
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<author><name sortKey="Jose Marti, Maria" sort="Jose Marti, Maria" uniqKey="Jose Marti M" first="Maria" last="Jose Marti">Maria Jose Marti</name>
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<author><name sortKey="Graus, Francesc" sort="Graus, Francesc" uniqKey="Graus F" first="Francesc" last="Graus">Francesc Graus</name>
<affiliation><inist:fA14 i1="01"><s1>Neurology Service, Hospital Clinic of Barcelona</s1>
<s2>Barcelona</s2>
<s3>ESP</s3>
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<affiliation><inist:fA14 i1="02"><s1>Neurological Tissue Bank, Universitat of Barcelona-Hospital Clinic</s1>
<s2>Barcelona</s2>
<s3>ESP</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
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</author>
<author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name>
<affiliation><inist:fA14 i1="01"><s1>Neurology Service, Hospital Clinic of Barcelona</s1>
<s2>Barcelona</s2>
<s3>ESP</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
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<series><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="2011">2011</date>
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<seriesStmt><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Creutzfeldt-Jakob disease</term>
<term>Lewy body</term>
<term>Lewy body dementia</term>
<term>Lewy body disease</term>
<term>Nervous system diseases</term>
<term>Prion</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Démence à corps de Lewy</term>
<term>Pathologie du   système nerveux</term>
<term>Corps Lewy</term>
<term>Encéphalopathie spongiforme de Creutzfeldt-Jakob</term>
<term>Prion</term>
<term>Corps Lewy maladie</term>
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<front><div type="abstract" xml:lang="en">Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial <sup>123</sup>
I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as <sup>123</sup>
I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.</div>
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<fA11 i1="05" i2="1"><s1>LLUFRIU (Sara)</s1>
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<fA11 i1="06" i2="1"><s1>BUONGIORNO (Mariateresa)</s1>
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<fA11 i1="07" i2="1"><s1>JESUS REY (Maria)</s1>
</fA11>
<fA11 i1="08" i2="1"><s1>JOSE MARTI (Maria)</s1>
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<fA11 i1="09" i2="1"><s1>GRAUS (Francesc)</s1>
</fA11>
<fA11 i1="10" i2="1"><s1>TOLOSA (Eduardo)</s1>
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<s2>Barcelona</s2>
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<s2>Barcelona</s2>
<s3>ESP</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>7 aut.</sZ>
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<fC01 i1="01" l="ENG"><s0>Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial <sup>123</sup>
I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as <sup>123</sup>
I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.</s0>
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<fC03 i1="03" i2="X" l="FRE"><s0>Corps Lewy</s0>
<s5>09</s5>
</fC03>
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<s5>09</s5>
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<fC03 i1="05" i2="X" l="FRE"><s0>Prion</s0>
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<server><NO>PASCAL 11-0321224 INIST</NO>
<ET>Rapidly Progressive Diffuse Lewy Body Disease</ET>
<AU>GAIG (Carles); VALLDEORIOLA (Francesc); GELPI (Ellen); EZQUERRA (Mario); LLUFRIU (Sara); BUONGIORNO (Mariateresa); JESUS REY (Maria); JOSE MARTI (Maria); GRAUS (Francesc); TOLOSA (Eduardo)</AU>
<AF>Neurology Service, Hospital Clinic of Barcelona/Barcelona/Espagne (1 aut., 2 aut., 4 aut., 5 aut., 6 aut., 8 aut., 9 aut., 10 aut.); Neurological Tissue Bank, Universitat of Barcelona-Hospital Clinic/Barcelona/Espagne (1 aut., 3 aut., 7 aut., 9 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2011; Vol. 26; No. 7; Pp. 1316-1323; Bibl. 36 ref.</SO>
<LA>Anglais</LA>
<EA>Background: Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. Objective: To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. Methods: Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). Results: Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial <sup>123</sup>
I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. Conclusions: Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as <sup>123</sup>
I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease.</EA>
<CC>002B17; 002B17G</CC>
<FD>Démence à corps de Lewy; Pathologie du   système nerveux; Corps Lewy; Encéphalopathie spongiforme de Creutzfeldt-Jakob; Prion; Corps Lewy maladie</FD>
<FG>Maladie à prions; Infection; Pathologie de l'encéphale; Maladie dégénérative; Pathologie du système nerveux central</FG>
<ED>Lewy body dementia; Nervous system diseases; Lewy body; Creutzfeldt-Jakob disease; Prion; Lewy body disease</ED>
<EG>Prion disease; Infection; Cerebral disorder; Degenerative disease; Central nervous system disease</EG>
<SD>Demencia cuerpos Lewy; Sistema nervioso patología; Cuerpo Lewy; Encefalopatía espongiforme Creutzfeldt-Jakob; Prion; Cuerpo Lewy enfermedad</SD>
<LO>INIST-20953.354000190480410180</LO>
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Movement Disorders (revue)

Rapidly Progressive Diffuse Lewy Body Disease

Rapidly Progressive Diffuse Lewy Body Disease

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