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@ARTICLE{Farschtschi:125803,
author = {S. Farschtschi and V.-F. Mautner and M. Pham and R. Nguyen
and H. Kehrer-Sawatzki and S. Hutter$^*$ and R. E. Friedrich
and A. Schulz and H. Morrison and D. Jones$^*$ and M.
Bendszus and P. Bäumer$^*$},
title = {{M}ultifocal nerve lesions and {LZTR}1 germline mutations
in segmental schwannomatosis.},
journal = {Annals of neurology},
volume = {80},
number = {4},
issn = {0364-5134},
address = {Hoboken, NJ},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2017-01927},
pages = {625 - 628},
year = {2016},
abstract = {Schwannomatosis is a genetic disorder characterized by the
occurrence of multiple peripheral schwannomas. Segmental
schwannomatosis is diagnosed when schwannomas are restricted
to 1 extremity and is thought to be caused by genetic
mosaicism. We studied 5 patients with segmental
schwannomatosis through microstructural magnetic resonance
neurography and mutation analysis of NF2, SMARCB1, and
LZTR1. In 4 of 5 patients, subtle fascicular nerve lesions
were detected in clinically unaffected extremities. Two
patients exhibited LZTR1 germline mutations. This appears
contrary to a simple concept of genetic mosaicism and
suggests more complex and heterogeneous mechanisms
underlying the phenotype of segmental schwannomatosis than
previously thought. Ann Neurol 2016;80:625-628.},
keywords = {LZTR1 protein, human (NLM Chemicals) / Transcription
Factors (NLM Chemicals)},
cin = {B062 / E010},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)E010-20160331},
pnm = {315 - Imaging and radiooncology (POF3-315)},
pid = {G:(DE-HGF)POF3-315},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:27472264},
doi = {10.1002/ana.24753},
url = {https://inrepo02.dkfz.de/record/125803},
}