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Pallidal stimulation in siblings with pantothenate kinase-associated neurodegeneration: Four-year follow-up

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F11%3A7253" target="_blank" >RIV/00216208:11110/11:7253 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/11:7015 RIV/00064203:_____/11:7015 RIV/00064165:_____/11:7253

  • Result on the web

    <a href="http://dx.doi.org/10.1002/mds.23349" target="_blank" >http://dx.doi.org/10.1002/mds.23349</a>

  • DOI - Digital Object Identifier

Alternative languages

  • Result language

    angličtina

  • Original language name

    Pallidal stimulation in siblings with pantothenate kinase-associated neurodegeneration: Four-year follow-up

  • Original language description

    PKAN - pantothenate kinase-associated neurodegeneration, formerly Hallervorden-Spatz disease is a devastating disease. One of the main symptoms is pharmacoresistant generalised dystonia. Neurosurgical treatmen of generalised dystonia - deep brain stimulation of pallidum internum (DBS GPi) has been successfully used in PKAN patients since 2002. We report 2 siblings with PKAN treated by DBS GPi. Clinically dominated pharmacoresistant generalised dystonia - axial, oromandibular and in extremities. In the boy, Burke-Fahn-Marsden dystonia rating scale improved by 81% respectively 51% 3 months respectively 4 years and 5 months after operation. In the girl, the improvement was 41% respectively 28% 3 months respectively 4 years after operation. There were no side effects and both patients benefited from the treatment.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    FG - Paediatrics

  • OECD FORD branch

Result continuities

  • Project

    Result was created during the realization of more than one project. More information in the Projects tab.

  • Continuities

    P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)<br>Z - Vyzkumny zamer (s odkazem do CEZ)

Others

  • Publication year

    2011

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Movement Disorders

  • ISSN

    0885-3185

  • e-ISSN

  • Volume of the periodical

    26

  • Issue of the periodical within the volume

    1

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    4

  • Pages from-to

    184-187

  • UT code for WoS article

    000288178400037

  • EID of the result in the Scopus database