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@ARTICLE{Stehr:272507,
author = {Stehr, Antonia M and Koeglsperger, Thomas and Jacob,
Maureen and Rhodio, Valerio and Winkelmann, Juliane and
Hopfner, Franziska and Zech, Michael},
title = {{T}remor-{D}ominant {M}ovement {D}isorder in {ANKRD}11-
{A}ssociated {KBG} {S}yndrome.},
journal = {Tremor and other hyperkinetic movements},
volume = {14},
number = {1},
issn = {2160-8288},
address = {New York, NY},
publisher = {Center for Digital Research and Scholarship},
reportid = {DZNE-2024-01183},
pages = {48},
year = {2024},
abstract = {KBG syndrome is a monogenic disorder caused by heterozygous
pathogenic variants in ANKRD11. A recent single-case study
suggested that the clinical spectrum of KBG syndrome,
classically defined by distinctive craniofacial traits and
developmental delay, may include movement disorders.We
report a 24-year-old patient harboring a pathogenic de novo
ANKRD11 frameshift variant. The phenotype was dominated by a
progressive tremor-dominant movement disorder, characterized
by rest, intention and postural tremor of the hands, voice
tremor, head and tongue tremor, increased muscle tone and
signs of ataxia. Additionally, the patient had a history of
mild developmental delay and epilepsy.Adding to the recently
described individual, our present patient highlights the
relevance of movement disorders as a clinically relevant
manifestation of KBG syndrome. ANKRD11 pathogenic variants
should be considered in the differential diagnosis of
combined tremor syndromes.},
keywords = {Humans / Tremor: genetics / Tremor: physiopathology / Young
Adult / Repressor Proteins: genetics / Male / Intellectual
Disability: genetics / Intellectual Disability:
physiopathology / Facies / Frameshift Mutation /
Microcephaly: genetics / Microcephaly: complications /
Microcephaly: physiopathology / Tooth Abnormalities:
genetics / Tooth Abnormalities: physiopathology / Bone
Diseases, Developmental: genetics / Bone Diseases,
Developmental: physiopathology / Bone Diseases,
Developmental: complications / Bone Diseases, Developmental:
diagnosis / Female / Abnormalities, Multiple / ANKRD11
(Other) / KBG syndrome (Other) / combined tremor syndrome
(Other) / tremor (Other) / ANKRD11 protein, human (NLM
Chemicals) / Repressor Proteins (NLM Chemicals)},
cin = {AG Höglinger / Clinical Research (Munich)},
ddc = {610},
cid = {I:(DE-2719)1110002 / I:(DE-2719)1111015},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39346806},
pmc = {pmc:PMC11428658},
doi = {10.5334/tohm.926},
url = {https://pub.dzne.de/record/272507},
}