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@ARTICLE{Scaravilli:272819,
author = {Scaravilli, Alessandra and Tranfa, Mario and Pontillo,
Giuseppe and Brais, Bernard and De Michele, Giovanna and La
Piana, Roberta and Saccà, Francesco and Santorelli, Filippo
Maria and Synofzik, Matthis and Brunetti, Arturo and
Cocozza, Sirio},
title = {{CHARON}: {A}n {I}maging-{B}ased {D}iagnostic {A}lgorithm
to {N}avigate {T}hrough the {S}ea of {H}ereditary
{D}egenerative {A}taxias.},
journal = {The cerebellum},
volume = {23},
number = {5},
issn = {1473-4222},
address = {New York},
publisher = {Springer US},
reportid = {DZNE-2024-01237},
pages = {2122 - 2129},
year = {2024},
abstract = {The complexity in diagnosing hereditary degenerative
ataxias lies not only in their rarity, but also in the
variety of different genetic conditions that can determine
sometimes similar and overlapping clinical findings. In this
light, Magnetic Resonance Imaging (MRI) plays a key role in
the evaluation of these conditions, being a fundamental
diagnostic tool needed not only to exclude other causes
determining the observed clinical phenotype, but also to
proper guide to an adequate genetic testing. Here, we
propose an MRI-based diagnostic algorithm named CHARON
(Characterization of Hereditary Ataxias Relying On
Neuroimaging), to help in disentangling among the numerous,
and apparently very similar, hereditary degenerative
ataxias. Being conceived from a neuroradiological
standpoint, it is based primarily on an accurate evaluation
of the observed MRI findings, with the first and most
important being the pattern of cerebellar atrophy. Along
with the evaluation of the presence, or absence, of
additional signal changes and/or supratentorial involvement,
CHARON allows for the identification of a small groups of
ataxias sharing similar imaging features. The integration of
additional MRI findings, demographic, clinical and
laboratory data allow then for the identification of
typical, and in some cases pathognomonic, phenotypes of
hereditary ataxias.},
subtyp = {Review Article},
keywords = {Humans / Algorithms / Magnetic Resonance Imaging: methods /
Neuroimaging: methods / Algorithm (Other) / Ataxia (Other) /
Magnetic Resonance Imaging (Other) / Neuroimaging (Other)},
cin = {AG Gasser},
ddc = {610},
cid = {I:(DE-2719)1210000},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC11489197},
pubmed = {pmid:38436911},
doi = {10.1007/s12311-024-01677-y},
url = {https://pub.dzne.de/record/272819},
}