PT  - JOURNAL ARTICLE
AU  - Jan Kassubek
AU  - Hans-Peter Müller
AU  - Kelly Del Tredici
AU  - Dorothée Lulé
AU  - Martin Gorges
AU  - Heiko Braak
AU  - Albert C Ludolph
TI  - Imaging the pathoanatomy of amyotrophic lateral sclerosis in vivo: targeting a propagation-based biological marker
AID  - 10.1136/jnnp-2017-316365
DP  - 2017 Nov 03
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - jnnp-2017-316365
4099  - http://jnnp.bmj.com/content/early/2017/11/03/jnnp-2017-316365.short
4100  - http://jnnp.bmj.com/content/early/2017/11/03/jnnp-2017-316365.full
AB  - Objective Neuropathological studies in amyotrophic lateral sclerosis (ALS) have shown a dissemination in a regional sequence in four anatomically defined patterns. The aim of this retrospective study was to see whether longitudinal diffusion tensor imaging (DTI) data support the pathological findings.Methods The application of DTI analysis to fibre structures that are prone to be involved at each neuropathological pattern of ALS was performed in a monocentre sample of 67 patients with ALS and 31 controls that obtained at least one follow-up scan after a median of 6 months.Results At the group level, longitudinal ALS data showed significant differences for the stage-related tract systems. At the individual level, 27% of the longitudinally scanned patients with ALS showed an increase in ALS stage, while the remaining were stable or were at the highest ALS stage. Longitudinal fractional anisotropy changes in the respective tract systems correlated significantly with the slope of the revised ALS functional rating scale.Interpretation The DTI-based protocol was able to image the disease patterns of ALS in vivo cross-sectionally and longitudinally, in support of DTI as a technical marker to image ALS stages.