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@ARTICLE{Traschtz:1007199,
author = {Traschütz, Andreas and Adarmes-Gomez, Astrid D. and
Anheim, Mathieu and Baets, Jonathan and Falkenburger, Björn
H. and Gburek-Augustat, Janina and Doss, Sarah and Kamm,
Christoph and Klivenyi, Peter and Grobe-Einsler, Marcus and
Klopstock, Thomas and Minnerop, Martina and Münchau,
Alexander and Pane, Chiara and Renaud, Mathilde and
Santorelli, Filippo M. and Schöls, Ludger and Timmann,
Dagmar and Vielhaber, Stefan and Haack, Tobias B. and van de
Warrenburg, Bart P. and Zanni, Ginevra and Synofzik,
Matthis},
title = {{A}utosomal {R}ecessive {C}erebellar {A}taxias in {E}urope:
{F}requency, {O}nset, and {S}everity in 677 {P}atients},
journal = {Movement disorders},
volume = {38},
number = {6},
issn = {0885-3185},
address = {New York, NY},
publisher = {Wiley},
reportid = {FZJ-2023-01978},
pages = {1109-1112},
year = {2023},
abstract = {Progress in next-generation sequencing has led to an
explosion of novel genes and phenotypes of autosomal
recessive cerebellar ataxias (ARCAs) in the last decade,
with >170 recessive conditions manifesting with ataxia
identified.1 With large-scale natural history and
mechanistic treatment trials on the horizon for many ARCAs,
up-to-date knowledge is required not only on relative
frequencies but also on real-world age and disease severity
distributions as key information for trial design planning
and recruitment. In this multicenter study, we provide data
on the relative frequency of ARCAs in Europe, delineate the
spectrum of age at disease onset, and present real-world
data on disease severity distributions of patients with ARCA
that help to inform future trial planning.},
cin = {INM-1},
ddc = {610},
cid = {I:(DE-Juel1)INM-1-20090406},
pnm = {5251 - Multilevel Brain Organization and Variability
(POF4-525)},
pid = {G:(DE-HGF)POF4-5251},
typ = {PUB:(DE-HGF)16},
pubmed = {37027459},
UT = {WOS:000989750000001},
doi = {10.1002/mds.29397},
url = {https://juser.fz-juelich.de/record/1007199},
}