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Development of neurodegeneration in amyotrophic lateral sclerosis: from up or down?

  • Neurology and Preclinical Neurological Studies - Review Article
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Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease associated with neurodegeneration and intracellular pathological 43-kDa transactive response sequence DNA-binding protein (TDP-43) positive inclusions. The various clinical symptoms, such as motor disorders and cognitive impairment, reflect the degeneration of certain areas of the nervous system. Since the discovery of the significance of pathological TDP-43 for human disease including ALS, there has been an increasing number of studies reporting on the distribution and severity of neurodegeneration. These have rekindled the old debate about whether the first or second motor neuron is the primary site of degeneration in ALS. To shed light on this question, the following is a review of the relevant neuropathological studies.

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Authors and Affiliations

  1. Department of Neurology, Hegau-Bodensee-Klinikum Singen, Virchowstr. 10, 78224, Singen (Hohentwiel), Germany

    F. Geser

  2. Division of Neurobiology, Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria

    L. Fellner & G. K. Wenning

  3. Department of Pathology, Neuropathology and Molecular Pathology, Medical University of Innsbruck, Innsbruck, Austria

    J. Haybaeck

  4. Department of Neuropathology, Diagnostic and Research Institute of Pathology, Medical University of Graz, Graz, Austria

    J. Haybaeck

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  1. F. Geser
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  2. L. Fellner
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  3. J. Haybaeck
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  4. G. K. Wenning
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Correspondence to F. Geser.

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Geser, F., Fellner, L., Haybaeck, J. et al. Development of neurodegeneration in amyotrophic lateral sclerosis: from up or down?. J Neural Transm 127, 1097–1105 (2020). https://doi.org/10.1007/s00702-020-02213-y

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  • DOI: https://doi.org/10.1007/s00702-020-02213-y

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