Imaging the pathoanatomy of amyotrophic lateral sclerosis in vivo: targeting a propagation-based biological marker

ObjectiveNeuropathological 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.MethodsThe 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.ResultsAt 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.InterpretationThe 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.

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PB 1 In vivo assessment of neurodegeneration in the brain by serial longitudinal diffusion tensor imaging – an application to amyotrophic lateral sclerosis

Clinical Neurophysiology ◽

10.1016/j.clinph.2017.06.060 ◽

2017 ◽

Vol 128 (10) ◽

pp. e315

Author(s):

H.P. Müller ◽

I. Kobor ◽

U. Bogdahn ◽

A.C. Ludolph ◽

A. Khomenko ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Diffusion Tensor Imaging ◽

Diffusion Tensor ◽

Longitudinal Diffusion ◽

The Brain ◽

Lateral Sclerosis ◽

In Vivo Assessment

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Neuroimaging to Investigate Multisystem Involvement and Provide Biomarkers in Amyotrophic Lateral Sclerosis

BioMed Research International ◽

10.1155/2014/467560 ◽

2014 ◽

Vol 2014 ◽

pp. 1-10 ◽

Cited By ~ 21

Author(s):

Pierre-François Pradat ◽

Mohamed-Mounir El Mendili

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Motor Neurons ◽

Diffusion Tensor ◽

Sensory System ◽

Diagnostic Value ◽

Resonance Spectroscopy ◽

Multisystem Disorder ◽

Individual Level ◽

Lateral Sclerosis

Neuroimaging allows investigating the extent of neurological systems degeneration in amyotrophic lateral sclerosis (ALS). Advanced MRI methods can detect changes related to the degeneration of upper motor neurons but have also demonstrated the participation of other systems such as the sensory system or basal ganglia, demonstratingin vivothat ALS is a multisystem disorder. Structural and functional imaging also allows studying dysfunction of brain areas associated with cognitive signs. From a biomarker perspective, numerous studies using diffusion tensor imaging showed a decrease of fractional anisotropy in the intracranial portion of the corticospinal tract but its diagnostic value at the individual level remains limited. A multiparametric approach will be required to use MRI in the diagnostic workup of ALS. A promising avenue is the new methodological developments of spinal cord imaging that has the advantage to investigate the two motor system components that are involved in ALS, that is, the lower and upper motor neuron. For all neuroimaging modalities, due to the intrinsic heterogeneity of ALS, larger pooled banks of images with standardized image acquisition and analysis procedures are needed. In this paper, we will review the main findings obtained with MRI, PET, SPECT, and nuclear magnetic resonance spectroscopy in ALS.

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Detection of White Matter Ultrastructural Changes for Amyotrophic Lateral Sclerosis Characterization: A Diagnostic Study from Dti-Derived Data

Brain Sciences ◽

10.3390/brainsci10120996 ◽

2020 ◽

Vol 10 (12) ◽

pp. 996

Author(s):

Fabiola De Marchi ◽

Alessandro Stecco ◽

Zeno Falaschi ◽

Francesco Filippone ◽

Alessio Pasché ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

White Matter ◽

Rating Scale ◽

Diffusion Tensor ◽

Brain Mri ◽

Ultrastructural Changes ◽

Cerebral Peduncle ◽

Diagnostic Study ◽

Lateral Sclerosis

In amyotrophic lateral sclerosis (ALS), magnetic resonance imaging (MRI) allows investigation at the microstructural level, employing techniques able to reveal white matter changes. In the current study, a diffusion tensor imaging (DTI) analysis, with a collection of apparent diffusion coefficient (ADC) and fractional anisotropy (FA) indexes, was performed in ALS patients to correlate geno- and phenotype features with MRI data, to investigate an in-vivo correlation of different neuropathological patterns. All patients who underwent the MR-DTI analysis were retrospectively recruited. MRI scan was collected within three months from diagnosis. FA and ADC values were collected in corpus callosum (CC), corona radiata (CR), cerebral peduncle (CR), cerebellar peduncle (CbP) and corticospinal tract at posterior limb of internal capsule (CST). DTI analysis performed in the whole ALS cohort revealed significant FA reduction and ADC increase in all selected regions, as widespread changes. Moreover, we observed a higher value of FA in rCR in bulbar patients. A positive correlation between ALS Functional Rating Scale-Revised and FA in rCP was evident. In consideration of the non-invasiveness, the reliability and the easy reproducibility of the method, we believe that brain MRI with DTI analyses may represent a valid tool usable as a diagnostic marker in ALS.

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