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@ARTICLE{Ganos:164476,
author = {Ganos, Christos and Biskup, Saskia and Krüger, Stefanie
and Meyer-Osores, Aracelli and Hodecker, Sibylle and Hagel,
Christian and Schöls, Ludger and Bhatia, Kailash P and
Münchau, Alexander},
title = {{D}ystonia with aphonia, slow horizontal saccades, epilepsy
and photic myoclonus: a novel syndrome?},
journal = {Parkinsonism $\&$ related disorders},
volume = {20},
number = {3},
issn = {1353-8020},
address = {Amsterdam [u.a.]},
publisher = {Elsevier Science},
reportid = {DZNE-2022-01028},
pages = {328 - 331},
year = {2014},
abstract = {Dystonia with anarthria and/or aphonia is a rare syndromic
association. Here we present two cases with slowly
progressive, severe generalized dystonia and aphonia, slow
horizontal saccades, epilepsy and photic myoclonus.Detailed
clinical data were collected over two decades in the female
(index) patient and for nine years in her similarly affected
son. Sanger sequencing followed by exome sequencing was
performed.Both patients had leg onset generalized dystonia
with gradual rostral spread including prominent facial and
oro-mandibular involvement. The index patient was anarthric,
her son aphonic. Both had saccadic slowing, more marked for
the horizontal plane, and subclinical epileptic activity.
The index patient also had photic myoclonus and a combined
axonal and demyelinating neuropathy. Known genetic causes of
similar syndromes were not identified.These cases with
caudo-rostrally spreading generalized dystonia with
prominent facial and oro-mandibular involvement, severe
speech impairment, marked slowing of horizontal saccades,
and photic myoclonus likely represent a novel entity.},
keywords = {Adult / Aphonia: complications / Aphonia: diagnosis /
Dystonia: complications / Dystonia: diagnosis / Epilepsy:
complications / Epilepsy: diagnosis / Female / Humans / Male
/ Middle Aged / Myoclonus: complications / Myoclonus:
diagnosis / Ocular Motility Disorders: complications /
Ocular Motility Disorders: diagnosis / Pedigree / Saccades:
physiology / Syndrome / Aphonia (Other) / Dystonia (Other) /
Epilepsy (Other) / Myoclonus (Other) / Slow horizontal
saccades (Other)},
cin = {Tübingen common / AG Gasser},
ddc = {610},
cid = {I:(DE-2719)6000018 / I:(DE-2719)1210000},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:24359844},
doi = {10.1016/j.parkreldis.2013.11.011},
url = {https://pub.dzne.de/record/164476},
}