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Steinbach, Robert [VerfasserIn]; Prell, Tino [VerfasserIn]; Gaur, Nayana [VerfasserIn]; Rödiger, Annekathrin [VerfasserIn]; Gaser, Christian [VerfasserIn]; Mayer, Thomas E. [VerfasserIn]; Witte, Otto W. [VerfasserIn]; Großkreutz, Julian [VerfasserIn]

Patterns of grey and white matter changes differ between bulbar and limb onset amyotrophic lateral sclerosis

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  • Medientyp: E-Artikel
  • Titel: Patterns of grey and white matter changes differ between bulbar and limb onset amyotrophic lateral sclerosis
  • Beteiligte: Steinbach, Robert [VerfasserIn]; Prell, Tino [VerfasserIn]; Gaur, Nayana [VerfasserIn]; Rödiger, Annekathrin [VerfasserIn]; Gaser, Christian [VerfasserIn]; Mayer, Thomas E. [VerfasserIn]; Witte, Otto W. [VerfasserIn]; Großkreutz, Julian [VerfasserIn]
  • Erschienen: 15 April 2021
  • Erschienen in: NeuroImage: Clinical ; 30(2021) Artikel-Nummer 102674, 12 Seiten
  • Sprache: Englisch
  • DOI: 10.1016/j.nicl.2021.102674
  • ISSN: 2213-1582
  • Identifikator:
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: Abstract: Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that is characterized by a high heterogeneity in patients’ disease course. Patients with bulbar onset of symptoms (b-ALS) have a poorer prognosis than patients with limb onset (l-ALS). However, neuroimaging correlates of the assumed biological difference between b-ALS and l-ALS may have been obfuscated by patients’ diversity in the disease course. We conducted Voxel-Based-Morphometry (VBM) and Tract-Based-Spatial-Statistics (TBSS) in a group of 76 ALS patients without clinically relevant cognitive deficits. The subgroups of 26 b-ALS and 52 l-ALS patients did not differ in terms of disease Phase or disease aggressiveness according to the D50 progression model. VBM analyses showed widespread ALS-related changes in grey and white matter, that were more pronounced for b-ALS. TBSS analyses revealed that b-ALS was predominantly characterized by frontal fractional anisotropy decreases. This demonstrates a higher degree of neurodegenerative burden for the group of b-ALS patients in comparison to l-ALS. Correspondingly, higher bulbar symptom burden was associated with right-temporal and inferior-frontal grey matter density decreases as well as fractional anisotropy decreases in inter-hemispheric and long association tracts. Contrasts between patients in Phase I and Phase II further revealed that b-ALS was characterized by an early cortical pathology and showed a spread only outside primary motor regions to frontal and temporal areas. In contrast, l-ALS showed ongoing structural integrity loss within primary motor-regions until Phase II. We therefore provide a strong rationale to treat both onset types of disease separately in ALS studies.
  • Zugangsstatus: Freier Zugang