La maladie de Parkinson en France (serveur d'exploration)

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Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy

Identifieur interne : 001092 ( PascalFrancis/Corpus ); précédent : 001091; suivant : 001093

Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy

Auteurs : Dominique Caparros-Lefebvre ; Nicolas Sergeant ; Andrew Lees ; Agnes Camuzat ; Susan Daniel ; Annie Lannuzel ; Alexis Brice ; Eduardo Tolosa ; Andre Delacourte ; Charles Duyckaerts

Source :

RBID : Pascal:02-0317349

Abstract

An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.

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Pour connaître la documentation sur le format Inist Standard.

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A08 01  1  ENG  @1 Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy
A11 01  1    @1 CAPARROS-LEFEBVRE (Dominique)
A11 02  1    @1 SERGEANT (Nicolas)
A11 03  1    @1 LEES (Andrew)
A11 04  1    @1 CAMUZAT (Agnes)
A11 05  1    @1 DANIEL (Susan)
A11 06  1    @1 LANNUZEL (Annie)
A11 07  1    @1 BRICE (Alexis)
A11 08  1    @1 TOLOSA (Eduardo)
A11 09  1    @1 DELACOURTE (Andre)
A11 10  1    @1 DUYCKAERTS (Charles)
A14 01      @1 Neurologie, Centre Hospitalier Universitaire de la Guadeloupe @2 Pointe a Pitre, French West Indies @3 GLP @Z 1 aut. @Z 6 aut.
A14 02      @1 Laboratoire INSERM U 422 @2 Lille @3 FRA @Z 2 aut. @Z 9 aut.
A14 03      @1 Reta Lila Weston Institute of Neurological Studies, Royal Free and University College London Medical School @2 London @3 GBR @Z 3 aut. @Z 5 aut.
A14 04      @1 INSERM U 289 @2 Paris @3 FRA @Z 4 aut. @Z 7 aut.
A14 05      @1 Hospital Clinici Provincial de Barcelona @3 ESP @Z 8 aut.
A14 06      @1 Laboratoire de Neuropathologie, CHU Pitié-Salpêtrière @2 Paris @3 FRA @Z 10 aut.
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A21       @1 2002
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C01 01    ENG  @0 An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.
C02 01  X    @0 002B
N21       @1 175
N82       @1 DST

Format Inist (serveur)

NO : PASCAL 02-0317349 INIST
ET : Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy
AU : CAPARROS-LEFEBVRE (Dominique); SERGEANT (Nicolas); LEES (Andrew); CAMUZAT (Agnes); DANIEL (Susan); LANNUZEL (Annie); BRICE (Alexis); TOLOSA (Eduardo); DELACOURTE (Andre); DUYCKAERTS (Charles)
AF : Neurologie, Centre Hospitalier Universitaire de la Guadeloupe/Pointe a Pitre, French West Indies/Guadeloupe (1 aut., 6 aut.); Laboratoire INSERM U 422/Lille/France (2 aut., 9 aut.); Reta Lila Weston Institute of Neurological Studies, Royal Free and University College London Medical School/London/Royaume-Uni (3 aut., 5 aut.); INSERM U 289/Paris/France (4 aut., 7 aut.); Hospital Clinici Provincial de Barcelona/Espagne (8 aut.); Laboratoire de Neuropathologie, CHU Pitié-Salpêtrière/Paris/France (10 aut.)
DT : Publication en série; Niveau analytique
SO : Brain; ISSN 0006-8950; Royaume-Uni; Da. 2002; Vol. 125; No. p.4; Pp. 801-811; Bibl. 1 p.1/4
LA : Anglais
EA : An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.
CC : 002B
LO : INIST-998.354000100793420090
ID : 02-0317349

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Pascal:02-0317349

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<div type="abstract" xml:lang="en">An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.</div>
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<s0>An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.</s0>
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<ET>Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy</ET>
<AU>CAPARROS-LEFEBVRE (Dominique); SERGEANT (Nicolas); LEES (Andrew); CAMUZAT (Agnes); DANIEL (Susan); LANNUZEL (Annie); BRICE (Alexis); TOLOSA (Eduardo); DELACOURTE (Andre); DUYCKAERTS (Charles)</AU>
<AF>Neurologie, Centre Hospitalier Universitaire de la Guadeloupe/Pointe a Pitre, French West Indies/Guadeloupe (1 aut., 6 aut.); Laboratoire INSERM U 422/Lille/France (2 aut., 9 aut.); Reta Lila Weston Institute of Neurological Studies, Royal Free and University College London Medical School/London/Royaume-Uni (3 aut., 5 aut.); INSERM U 289/Paris/France (4 aut., 7 aut.); Hospital Clinici Provincial de Barcelona/Espagne (8 aut.); Laboratoire de Neuropathologie, CHU Pitié-Salpêtrière/Paris/France (10 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Brain; ISSN 0006-8950; Royaume-Uni; Da. 2002; Vol. 125; No. p.4; Pp. 801-811; Bibl. 1 p.1/4</SO>
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<EA>An unusually high frequency of atypical Parkinson syndrome has been delineated over the last 5 years in the French West Indies. Postural instability with early falls, prominent frontal lobe dysfunction and pseudo-bulbar palsy were common and three-quarters of the patients were L-dopa unresponsive. One-third of all patients seen had probable progressive supranuclear palsy (PSP). This new focus of atypical parkinsonism is reminiscent of the one described in Guam and may be linked to exposure to tropical plants containing mitochondrial complex I inhibitors (quinolines, acetogenins, rotenoids). Two hundred and twenty consecutive patients with Parkinson's syndrome seen by the neurology service at Pointe à Pitre, Guadeloupe University Hospital were studied. Currently accepted operational clinical criteria for Parkinson's syndromes were applied. The pathological findings of three patients who came to autopsy are reported. Fifty-eight patients had probable PSP, 96 had undetermined parkinsonism and 50 had Parkinson's disease, 15 had amyotrophic lateral sclerosis with parkinsonism and one had probable multiple system atrophy. All three PSP patients in whom post-mortem study was performed had early postural instability, gaze palsy and parkinsonian symptoms, followed by a frontolimbic dementia and corticobulbar signs. Neuropathological examination showed an accumulation of tau proteins, predominating in the midbrain. There was an exceptionally large accumulation of neuropil threads in Case 1. Biochemical studies detected a major doublet of pathological tau at 64 and 69 kDa in brain tissue homogenates. All cases were homozygous for the H1 tau haplotype, but no mutation of the tau gene was observed. Clinical, neuropathological and biochemical features were compatible with the diagnosis of PSP, although some unusual pathological features were noted in Case 1. A cluster of cases presenting with atypical parkinsonism is reported. Guadeloupean parkinsonism may prove to be a tauopathy identical or closely related to PSP.</EA>
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