% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{SchleFreyer:155566,
author = {Schüle-Freyer, Rebecca and Timmann, Dagmar and Erasmus,
Corrie E and Reichbauer, Jennifer and Wayand, Melanie and
van de Warrenburg, Bart and Schöls, Ludger and Wilke, Carlo
and Bevot, Andrea and Zuchner, Stephan and Beltran, Sergi
and Laurie, Steven and Matalonga, Leslie and Graessner, Holm
and Synofzik, Matthis},
collaboration = {Solve-RD-DITF-RND and Consortium, Solve-RD},
title = {{S}olving unsolved rare neurological diseases-a
{S}olve-{RD} viewpoint.},
journal = {European journal of human genetics},
volume = {29},
number = {9},
issn = {1476-5438},
address = {Basingstoke},
publisher = {Stockton Press},
reportid = {DZNE-2021-00744},
pages = {1332-1336},
year = {2021},
abstract = {Rare genetic neurological disorders (RND; ORPHA:71859) are
a heterogeneous group of disorders comprising >1700 distinct
genetic disease entities. However, genetic discoveries have
not yet translated into dramatic increases of diagnostic
yield and indeed rates of molecular genetic diagnoses have
been stuck at about $30–50\%$ across NGS modalities and
RND phenotypes [1, 2]. Existence of yet unknown disease
genes as well as shortcomings of commonly employed NGS
technologies and analysis pipelines in detecting certain
variant types are typically cited to explain the low
diagnosis rates.To increase the diagnostic yield in RNDs -
one of the four focus disease groups in Solve-RD - we follow
two major approaches, that we will here present and
exemplify: (i) systematic state-of the art re-analysis of
large cohorts of unsolved whole-exome/genome sequencing
(WES/WGS) RND datasets; and (ii) novel-omics approaches.
Based on the way Solve-RD systematically organizes
researchers’ expertise to channel this approach [3], the
European Reference Network for Rare Neurological Diseases
(ERN-RND) has established its own Data Interpretation Task
Force (DITF) within SOLVE-RD, which is currently composed of
clinical and genetic experts from 29 sites in 15 European
countries.},
keywords = {Datasets as Topic / Genetic Testing: methods / Genetic
Testing: standards / Genomics: methods / Genomics: standards
/ Humans / Nervous System Diseases: genetics / Nervous
System Diseases: pathology / Practice Guidelines as Topic /
Rare Diseases: genetics / Rare Diseases: pathology / Exome
Sequencing: methods / Exome Sequencing: standards},
cin = {AG Gasser / AG Maetzler / Core ICRU},
ddc = {610},
cid = {I:(DE-2719)1210000 / I:(DE-2719)5000024 /
I:(DE-2719)1240005},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC8440537},
pubmed = {pmid:33972714},
doi = {10.1038/s41431-021-00901-1},
url = {https://pub.dzne.de/record/155566},
}