TY - JOUR
AU - Rosenbohm, Angela
AU - Del Tredici, Kelly
AU - Braak, Heiko
AU - Huppertz, Hans-Jürgen
AU - Ludolph, Albert
AU - Müller, Hans-Peter
AU - Kassubek, Jan
TI - Involvement of cortico-efferent tracts in flail arm syndrome: a tract-of-interest-based DTI study.
JO - Journal of neurology
VL - 269
IS - 5
SN - 0340-5354
CY - Berlin
PB - Springer
M1 - DZNE-2021-01445
SP - 2619-2626
PY - 2022
N1 - ISSN 1432-1459 not unique: **2 hits**. (CC BY)
AB - Flail arm syndrome is a restricted phenotype of motor neuron disease that is characterized by progressive, predominantly proximal weakness and atrophy of the upper limbs.The study was designed to investigate specific white matter alterations in diffusion tensor imaging (DTI) data from flail arm syndrome patients using a hypothesis-guided tract-of-interest-based approach to identify in vivo microstructural changes according to a neuropathologically defined amyotrophic lateral sclerosis (ALS)-related pathology of the cortico-efferent tracts.DTI-based white matter mapping was performed both by an unbiased voxel-wise statistical comparison and by a hypothesis-guided tract-wise analysis of fractional anisotropy (FA) maps according to the neuropathological ALS-propagation pattern for 43 flail arm syndrome patients vs 43 'classical' ALS patients vs 40 matched controls.The analysis of white matter integrity demonstrated regional FA reductions for the flail arm syndrome group predominantly along the CST. In the tract-specific analysis according to the proposed sequential cerebral pathology pattern of ALS, the flail arm syndrome patients showed significant alterations of the specific tract systems that were identical to 'classical' ALS if compared to controls.The DTI study including the tract-of-interest-based analysis showed a microstructural involvement pattern in the brains of flail arm syndrome patients, supporting the hypothesis that flail arm syndrome is a phenotypical variant of ALS.
KW - Amyotrophic Lateral Sclerosis: complications
KW - Amyotrophic Lateral Sclerosis: diagnostic imaging
KW - Amyotrophic Lateral Sclerosis: pathology
KW - Anisotropy
KW - Arm: diagnostic imaging
KW - Arm: pathology
KW - Brain Mapping
KW - Diffusion Tensor Imaging: methods
KW - Disease Progression
KW - Humans
KW - Image Processing, Computer-Assisted: methods
KW - Pyramidal Tracts
KW - Vascular Diseases
KW - Amyotrophic lateral sclerosis (Other)
KW - Diffusion tensor imaging (Other)
KW - Flail arm syndrome (Other)
KW - Magnetic resonance imaging (Other)
KW - Motor neuron disease (Other)
LB - PUB:(DE-HGF)16
C2 - pmc:PMC9021061
C6 - pmid:34676447
DO - DOI:10.1007/s00415-021-10854-6
UR - https://pub.dzne.de/record/162790
ER -