New way to assess and track HSP

Imaging ‘white matter’ in the brain

Diffusion Tensor Imaging (DTI) used in this German study has shown up subtle abnormalities in brain white matter in HSPers. This is of value in two important ways:

  • Early alterations can be detected and tracked over time, and

  • Abnormailities imaged correlate with HSP symptoms.


Hereditary spastic paraplegias (HSP) are genetically and clinically

heterogeneous neurodegenerative disorders. Most MR (imaging) studies on HSP include very heterogeneous samples of patients, and findings were inconsistent.


Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated.


Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms.


Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.

SOURCE: Movement Disorders Published Online: 28 Jul 2010

Specific pattern of early white-matter changes in pure hereditary spastic paraplegia

Thomas Duning, MD 1 * , Tobias Warnecke, MD 1 , Anja Schirmacher, PhD 1, Hagen Schiffbauer, MD 2, Hubertus Lohmann, PhD 1, Siawoosh Mohammadi, PhD 1, Peter Young, MD 1, Michael Deppe, PhD 1 1

Department of Neurology, University Hospital of Münster, Münster, Germany 2Department of Clinical Radiology, University Hospital of Münster, Münster, Germany

email: Thomas Duning ([email protected])

*Correspondence to Thomas Duning, Department of Neurology, University Hospital of Muenster, Albert-Schweitzer-Str. 33, 48149 Muenster, Germany

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