Klinische Neurophysiologie 2010; 41 - ID68
DOI: 10.1055/s-0030-1250897

Whole brain-based multiparametric MRI analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging

A Unrath 1, HP Mueller 1, A Riecker 1, AD Sperfeld 1, 2, AC Ludolph 1, J Kassubek 1
  • 1Universität Ulm, Neurologie, Ulm, Deutschland
  • 2HELIOS Kliniken Bad Saarow, Neurologie, Bad Saarow, Deutschland

Introduction: Different motor neuron disorders (MNDs) are mainly defined by the clinical presentation based on the predominance of upper or lower motor neuron impairment and the course of the disease. The present study was designed to investigate three different rare MNDs, i.e. primary lateral sclerosis (PLS), hereditary spastic paraparesis (HSP), and X-linked spinobulbar muscular atrophy (X-SBMA), by use of whole-brain based Diffusion Tensor Imaging (DTI) analysis in comparison with matched controls.

Material and Methods: Fractional anisotropy (FA) mapping from DTI was applied to data sets of 25 PLS patients (8m/17f, 60±9 years), 24 HSP patients (12m/12f, 50±9 years), 20 X-SBMA patients (20m/0f, 50±8 years), and a sample of healthy controls, age and gender matched to the respective groups. All data analyses were performed in one common analysis software environment (TIFT – Tensor Imaging and Fiber Tracking).

Results: This analysis of white matter (WM) impairment revealed widespread and characteristic patterns of alterations within the motor system with a predominant deterioration of the corticospinal tract (CST) in HSP and PLS patients according to the clinical presentation and also in patients with X-SBMA to a lesser degree, but also WM changes in projections to the limbic system and within distinct areas of the corpus callosum (CC), the latter both for HSP and PLS patients.

Conclusion: In summary, DTI was able to define a characteristic WM pathoanatomy in motor and extra-motor brain areas, such as the CC and the limbic projectional system, for different MNDs via whole brain-based FA assessment. Future advanced MRI-based investigations might help to provide a fingerprint-identification of MNDs.

Fig.1: Results of the whole brain-based FA analysis of patients vs. controls – thresholded at p<0.05, corrected for multiple comparisons: left: PLS patients vs. controls, center: HSP patients vs. controls, right: X-SBMA patients vs. controls.