Bildgebende Verfahren bei amyotropher Lateralsklerose und frontotemporaler Demenz

Zusammenfassung

Aufgrund genetischer und neuropathologischer Befunde kann man die amyotrophe Lateralsklerose (ALS) und Frontotemporale Demenz (FTD) als 2 Entitäten eines Krankheitskontinuums betrachten. Moderne bildgebende Verfahren unterstützen diese Ansicht, indem sie ähnlich gelagerte strukturelle und funktionelle Veränderungen der grauen und weißen Substanz bei beiden Erkrankungen nachweisen. Für die ALS ist entscheidend, dass ihre Pathologie deutlich über die motorischen Areale hinausgeht und die Schädigung der weißen Substanz ein wichtiger Bestandteil der Pathophysiologie ist, wobei zeitgleich hyper- und hypokonnektive Areale existieren. Im Umkehrschluss bestätigen die Imaging-Verfahren, dass die FTD nicht allein durch die frontale Atrophie bestimmt wird, sondern auch motorische Areale einbezogen sind und durch die vermutlich kompensatorische Rekrutierung von verschiedenen Hirnarealen gekennzeichnet ist. Diese Übersichtsarbeit stellt die wesentlichen Ergebnisse zur konventionellen MRT und moderner Bildgebung bei ALS und FTD dar: Voxel-Basierte-Morphometrie, Diffusions-Tensor-Imaging, Magnetization-Transfer-Imaging, MR-Spektroskopie, funktionelles MRT.

Abstract

On the basis of recent genetic and neuropathological findings, amyotrophic lateral sclerosis and frontotemporal dementia can be regarded as 2 manifestations of a continuous disease. Novel imaging techniques showing comparable structural and functional changes in patient’s white and grey matter support this view. Pathological changes in ALS clearly extend beyond the motor areas and disturbances in white matter, characterised by functional hypo- and hyperconnective areas, are a crucial factor in this disease. Vice versa FTD is not restricted to frontal atrophy, but shows involvement of motor areas and recruitment of compensatory brain regions. This review focus on the main results, obtained from conventional imaging and modern MRI techniques in ALS and FTD such as voxel-based morphometry, diffusion tensor imaging, magnetisation transfer imaging, MR spectroscopy, and functional MRI.

Schlüsselwörter

amyotrophe Lateralsklerose (ALS) - frontotemporale Demenz (FTD) - bildgebende Verfahren - Diagnostik - MRT

Key words

amyotrophic lateral sclerosis (ALS) - frontotemporal dementia (FTD) - imaging procedures - diagnostics - MRI

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