Progressive supranuclear palsy and corticobasal degeneration : Lumping versus splitting
Identifieur interne :
001D83 ( PascalFrancis/Corpus );
précédent :
001D82;
suivant :
001D84
Progressive supranuclear palsy and corticobasal degeneration : Lumping versus splitting
Auteurs : Tomaso Scaravilli ;
Eduardo Tolosa ;
Isidre FerrerSource :
-
Movement disorders [ 0885-3185 ] ; 2005.
RBID : Pascal:05-0457435
Descripteurs français
English descriptors
Abstract
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are both sporadic disorders with tau pathology. Criteria have been defined that in most instances allow for adequate diagnosis of the two disorders both clinically and neuropathologically; however, overlap is not uncommon. For example, patients with PSP may present with severe unilateral apraxia and supranuclear gaze palsy can occur in CBD. Pathological overlap also occurs and pathologically "mixed" cases are encountered. Common to both these two tauopathies is that isoforms of four-repeat tau due to splicing of exon 10 define the tau filamentous aggregates. This is in contrast to other tau disorders such as Pick's with three-repeat tau aggregates. Additional evidence for a causal link between PSP and CBD is the finding that both disorders are homozygous for the H1 tau haplotype. Furthermore, in some families with parkinsonism linked to defined mutations of the tau gene (FTDP-17), involved relatives have presented with PSP whereas others with the CBD phenotype. Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out.
Notice en format standard (ISO 2709)
Pour connaître la documentation sur le format Inist Standard.
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| A01 | 01 | 1 | | @0 0885-3185 |
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| A03 | | 1 | | @0 Mov. disord. |
|---|
| A05 | | | | @2 20 |
|---|
| A06 | | | | @3 SUP12 |
|---|
| A08 | 01 | 1 | ENG | @1 Progressive supranuclear palsy and corticobasal degeneration : Lumping versus splitting |
|---|
| A09 | 01 | 1 | ENG | @1 Atypical Parkinsonian Disorders |
|---|
| A11 | 01 | 1 | | @1 SCARAVILLI (Tomaso) |
|---|
| A11 | 02 | 1 | | @1 TOLOSA (Eduardo) |
|---|
| A11 | 03 | 1 | | @1 FERRER (Isidre) |
|---|
| A12 | 01 | 1 | | @1 POEWE (Werner) @9 ed. |
|---|
| A12 | 02 | 1 | | @1 WENNING (Gregor K.) @9 ed. |
|---|
| A14 | 01 | | | @1 Department of Neuroscience, University of Padua @2 Padua @3 ITA @Z 1 aut. |
|---|
| A14 | 02 | | | @1 Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clinic de Malalties del Sistema Nervlós. Hospital Clinic de Barcelona, Institut d'lnvestigacions Biomèdiques August Pi i Sunyer (IDIBAPS) @2 Barcelona @3 ESP @Z 2 aut. |
|---|
| A14 | 03 | | | @1 Institut de Neuropatologia, Servei Anatomia Patològica, Hospital de Bellvitge @2 Hospitalet de Llobregat @3 ESP @Z 3 aut. |
|---|
| A15 | 01 | | | @1 Department of Neurology, Medical University Innsbruck @2 Innsbruck @3 AUT @Z 1 aut. @Z 2 aut. |
|---|
| A20 | | | | @2 S21-S28 |
|---|
| A21 | | | | @1 2005 |
|---|
| A23 | 01 | | | @0 ENG |
|---|
| A43 | 01 | | | @1 INIST @2 20953 @5 354000132714250040 |
|---|
| A44 | | | | @0 0000 @1 © 2005 INIST-CNRS. All rights reserved. |
|---|
| A45 | | | | @0 75 ref. |
|---|
| A47 | 01 | 1 | | @0 05-0457435 |
|---|
| A60 | | | | @1 P @2 C |
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| A61 | | | | @0 A |
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| A64 | 01 | 1 | | @0 Movement disorders |
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| A66 | 01 | | | @0 USA |
|---|
| C01 | 01 | | ENG | @0 Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are both sporadic disorders with tau pathology. Criteria have been defined that in most instances allow for adequate diagnosis of the two disorders both clinically and neuropathologically; however, overlap is not uncommon. For example, patients with PSP may present with severe unilateral apraxia and supranuclear gaze palsy can occur in CBD. Pathological overlap also occurs and pathologically "mixed" cases are encountered. Common to both these two tauopathies is that isoforms of four-repeat tau due to splicing of exon 10 define the tau filamentous aggregates. This is in contrast to other tau disorders such as Pick's with three-repeat tau aggregates. Additional evidence for a causal link between PSP and CBD is the finding that both disorders are homozygous for the H1 tau haplotype. Furthermore, in some families with parkinsonism linked to defined mutations of the tau gene (FTDP-17), involved relatives have presented with PSP whereas others with the CBD phenotype. Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. |
|---|
| C02 | 01 | X | | @0 002B17 |
|---|
| C02 | 02 | X | | @0 002B17G |
|---|
| C02 | 03 | X | | @0 002B09M |
|---|
| C03 | 01 | X | FRE | @0 Système nerveux pathologie @5 01 |
|---|
| C03 | 01 | X | ENG | @0 Nervous system diseases @5 01 |
|---|
| C03 | 01 | X | SPA | @0 Sistema nervioso patología @5 01 |
|---|
| C03 | 02 | X | FRE | @0 Dégénérescence @5 02 |
|---|
| C03 | 02 | X | ENG | @0 Degeneration @5 02 |
|---|
| C03 | 02 | X | SPA | @0 Degeneración @5 02 |
|---|
| C03 | 03 | X | FRE | @0 Etude comparative @5 09 |
|---|
| C03 | 03 | X | ENG | @0 Comparative study @5 09 |
|---|
| C03 | 03 | X | SPA | @0 Estudio comparativo @5 09 |
|---|
| N21 | | | | @1 318 |
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| N44 | 01 | | | @1 OTO |
|---|
| N82 | | | | @1 OTO |
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|
|---|
| pR |
| A30 | 01 | 1 | ENG | @1 Atypical Parkinsonian Disorders - From Protein Dysfunction to Therapeutic Intervention. International Meeting @3 Innsbruck AUT @4 2003-02-19 |
|---|
|
|---|
Format Inist (serveur)
| NO : | PASCAL 05-0457435 INIST |
|---|
| ET : | Progressive supranuclear palsy and corticobasal degeneration : Lumping versus splitting |
|---|
| AU : | SCARAVILLI (Tomaso); TOLOSA (Eduardo); FERRER (Isidre); POEWE (Werner); WENNING (Gregor K.) |
|---|
| AF : | Department of Neuroscience, University of Padua/Padua/Italie (1 aut.); Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clinic de Malalties del Sistema Nervlós. Hospital Clinic de Barcelona, Institut d'lnvestigacions Biomèdiques August Pi i Sunyer (IDIBAPS)/Barcelona/Espagne (2 aut.); Institut de Neuropatologia, Servei Anatomia Patològica, Hospital de Bellvitge/Hospitalet de Llobregat/Espagne (3 aut.); Department of Neurology, Medical University Innsbruck/Innsbruck/Autriche (1 aut., 2 aut.) |
|---|
| DT : | Publication en série; Congrès; Niveau analytique |
|---|
| SO : | Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2005; Vol. 20; No. SUP12; S21-S28; Bibl. 75 ref. |
|---|
| LA : | Anglais |
|---|
| EA : | Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are both sporadic disorders with tau pathology. Criteria have been defined that in most instances allow for adequate diagnosis of the two disorders both clinically and neuropathologically; however, overlap is not uncommon. For example, patients with PSP may present with severe unilateral apraxia and supranuclear gaze palsy can occur in CBD. Pathological overlap also occurs and pathologically "mixed" cases are encountered. Common to both these two tauopathies is that isoforms of four-repeat tau due to splicing of exon 10 define the tau filamentous aggregates. This is in contrast to other tau disorders such as Pick's with three-repeat tau aggregates. Additional evidence for a causal link between PSP and CBD is the finding that both disorders are homozygous for the H1 tau haplotype. Furthermore, in some families with parkinsonism linked to defined mutations of the tau gene (FTDP-17), involved relatives have presented with PSP whereas others with the CBD phenotype. Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. |
|---|
| CC : | 002B17; 002B17G; 002B09M |
|---|
| FD : | Système nerveux pathologie; Dégénérescence; Etude comparative |
|---|
| ED : | Nervous system diseases; Degeneration; Comparative study |
|---|
| SD : | Sistema nervioso patología; Degeneración; Estudio comparativo |
|---|
| LO : | INIST-20953.354000132714250040 |
|---|
| ID : | 05-0457435 |
|---|
Links to Exploration step
Pascal:05-0457435
Le document en format XML
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<ET>Progressive supranuclear palsy and corticobasal degeneration : Lumping versus splitting</ET>
<AU>SCARAVILLI (Tomaso); TOLOSA (Eduardo); FERRER (Isidre); POEWE (Werner); WENNING (Gregor K.)</AU>
<AF>Department of Neuroscience, University of Padua/Padua/Italie (1 aut.); Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clinic de Malalties del Sistema Nervlós. Hospital Clinic de Barcelona, Institut d'lnvestigacions Biomèdiques August Pi i Sunyer (IDIBAPS)/Barcelona/Espagne (2 aut.); Institut de Neuropatologia, Servei Anatomia Patològica, Hospital de Bellvitge/Hospitalet de Llobregat/Espagne (3 aut.); Department of Neurology, Medical University Innsbruck/Innsbruck/Autriche (1 aut., 2 aut.)</AF>
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