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
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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
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FG - Paediatrics
OECD FORD branch
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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
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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
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