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Degeneration der Motoneurone

Amyotrophe Lateralsklerose früh erkennen und behandeln

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Zusammenfassung

Die verschiedenen Behandlungsoptionen der amyotrophen Lateralsklerose adäquat einzusetzen erfordert eine genaue Kenntnis möglicher klinischer Manifestationen und Differenzialdiagnosen und die Abschätzung des individuellen Krankheitsverlaufs. Im Mittelpunkt stehen bisher aufgrund fehlender kurativer Therapieansätze eine krankheitsmodifizierende und symptomatische Behandlung sowie die Hilfsmittelverordnung.

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Literatur

  1. Rosenbohm A, Peter RS, Erhardt S, Lulé D, Rothenbacher D, Ludolph AC et al. Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol 2017; 264(4):749–57

    Article  Google Scholar 

  2. Marin B, Boumediene F, Logroscino G, Couratier P, Babron MC, Leutenegger AL et al. Variation in worldwide incidence of amyotrophic lateral sclerosis: a meta-analysis. Int J Epidemiol 2017; 46(1):57–74

    PubMed  Google Scholar 

  3. Johnston CA, Stanton BR, Turner MR, Gray R, Blunt AH, Butt D et al. Amyotrophic lateral sclerosis in an urban setting: a population based study of inner city London. J Neurol 2006; 253(12):1642–3

    Article  Google Scholar 

  4. Uenal H, Rosenbohm A, Kufeldt J, Weydt P, Goder K, Ludolph AC et al. Incidence and geographical variation of amyotrophic lateral sclerosis (ALS) in Southern Germany—completeness of the ALS registry Swabia. PLoS One 2014; 9(4):e93932

    Article  Google Scholar 

  5. Burrell JR, Halliday GM, Kril JJ, Ittner LM, Gotz J, Kiernan MC et al. The frontotemporal dementia-motor neuron disease continuum. Lancet 2016;388(10047): 919–31

    Article  Google Scholar 

  6. Weishaupt JH, Hyman T, Dikic I. Common Molecular Pathways in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Trends Mol Med 2016; 22(9):769–83

    Article  CAS  Google Scholar 

  7. Müller K, Brenner D, Weydt P, Meyer T, Grehl T, Petri S et al. Comprehensive analysis of the mutation spectrum in 301 German ALS families. J Neurol Neurosurg Psychiatry 2018;89(8): 817–27

    Article  Google Scholar 

  8. Taylor JP, Brown RH, Cleveland DW. Decoding ALS: from genes to mechanism. Nature 2016; 539(7628):197–206

    Article  Google Scholar 

  9. Al-Chalabi A, Fang F, Hanby MF, Leigh PN, Shaw CE, Ye W et al. An estimate of amyotrophic lateral sclerosis heritability using twin data. J Neurol Neurosurg Psychiatry 2010; 81(12):1324–6

    Article  CAS  Google Scholar 

  10. Al-Chalabi A, Calvo A, Chio A, Colville S, Ellis CM, Hardiman O et al. Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study. Lancet Neurol 2014; 13(11):1108–13

    Article  Google Scholar 

  11. Brettschneider J, Del Tredici K, Toledo JB, Robinson JL, Irwin DJ, Grossman M et al. Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Ann Neurol 2013; 74(1):20–38

    Article  CAS  Google Scholar 

  12. Gorges M, Del Tredici K, Dreyhaupt J, Braak H, Ludolph AC, Müller H et al. Corticoefferent pathology distribution in amyotrophic lateral sclerosis: in vivo evidence from a meta-analysis of diffusion tensor imaging data. Sci Rep 2018; 8(1):15389

    Article  Google Scholar 

  13. Kang SH, Li Y, Fukaya M, Lorenzini I, Cleveland DW, Ostrow LW et al. Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci 2013; 16(5):571–79

    Article  CAS  Google Scholar 

  14. Philips T, Rothstein JD. Glial cells in amyotrophic lateral sclerosis. Experimental Neurology 2014; 262(1):111–20

    Article  CAS  Google Scholar 

  15. Hammad M, Silva A, Glass J, Sladky JT, Benatar M. Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology 2007; 69(24):2236–42

    Article  CAS  Google Scholar 

  16. Beeldman E, Raaphorst J, Klein Twennaar M, de Visser M, Schmand BA, de Haan RJ. The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry 2016; 87(6):611–9

    Article  Google Scholar 

  17. Abrahams S, Newton J, Niven E, Foley J, Bak TH. Screening for cognition and behaviour changes in ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2014; 15(1-2):9–14

    Article  Google Scholar 

  18. Al-Chalabi A, Hardiman O, Kiernan MC, Chiò A, Rix-Brooks B, van den Berg, Leonard H. Amyotrophic lateral sclerosis: moving towards a new classification system. Lancet Neurol 2016; 15(11):1182–94

    Article  Google Scholar 

  19. Braak H, Brettschneider J, Ludolph AC, Lee VM, Trojanowski JQ, Del Tredici K. Amyotrophic lateral sclerosis—a model of corticofugal axonal spread. Nature reviews. Neurology 2013; 9(12):708–14

    CAS  PubMed  Google Scholar 

  20. Gerardo-Nava J, Mayorenko II, Grehl T, Steinbusch HWM, Weis J, Brook GA. Differential pattern of neuroprotection in lumbar, cervical and thoracic spinal cord segments in an organotypic rat model of glutamate-induced excitotoxicity. J Chem Neuroanat 2013; 53:11–7

    Article  CAS  Google Scholar 

  21. Hübers A, Hildebrandt V, Petri S, Kollewe K, Hermann A, Storch A et al. Clinical features and differential diagnosis of flail arm syndrome. J Neurol 2016; 263(2):390–5

    Article  Google Scholar 

  22. Ince PG, Evans J, Knopp M, Forster G, Hamdalla HHM, Wharton SB et al. Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS. Neurology 2003 22; 60(8):1252–8

    Article  CAS  Google Scholar 

  23. Müller H, Agosta F, Riva N, Spinelli EG, Comi G, Ludolph AC et al. Fast progressive lower motor neuron disease is an ALS variant: A two-centre tract of interest-based MRI data analysis. Neuroimage Clin 2018; 17:145–52

    Article  Google Scholar 

  24. de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J et al. Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol 2008; 119(3):497–503

    Article  Google Scholar 

  25. Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis 2000; 1(5):293–9

    Article  CAS  Google Scholar 

  26. Agosta F, Al-Chalabi A, Filippi M, Hardiman O, Kaji R, Meininger V et al. The El Escorial criteria: strengths and weaknesses. Amyotroph Lateral Scler Frontotemporal Degener 2015; 16(1-2):1–7

    Article  Google Scholar 

  27. Ludolph AC, Drory V, Hardiman O, Nakano I, Ravits J, Robberecht W et al. A revision of the El Escorial criteria - 2015. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2015; 16(5-6):291–2

    Article  Google Scholar 

  28. Ludolph, Dengler, Grehl, Hecht, Meyer, Löscher, Petri, Weber, Weishaupt. Leitlinien für Diagnostik und Therapie in der Neurologie: Amyotrophe Lateralsklerose (Motoneuronerkrankungen) 2015; https://www.dgn.org/leitlinien/3012-ll-18-ll-amyotrophe-lateralsklerose-motoneuronerkrankungen.

  29. Zhouwei X, Henderson RD, David M, McCombe PA. Neurofilaments as Biomarkers for Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-Analysis. PLoS One 2016; 11(10):e0164625

    Article  Google Scholar 

  30. Ludolph AC, Schuster J, Dorst J, Dupuis L, Dreyhaupt J, Weishaupt JH et al. Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial. Lancet Neurol 2018; 17(8):681–8

    Article  CAS  Google Scholar 

  31. Abe K, Itoyama Y, Sobue G, Tsuji S, Aoki M, Doyu M et al. Confirmatory double-blind, parallel-group, placebo-controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener 2014; 15(7-8):610–7

    Article  CAS  Google Scholar 

  32. Beghi E, Chiò A, Couratier P, Esteban J, Hardiman O, Logroscino G et al. The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials. Amyotroph Lateral Scler 2011; 12(1):1–10

    Article  Google Scholar 

  33. Nicholson KA, Cudkowicz ME, Berry JD. Clinical Trial Designs in Amyotrophic Lateral Sclerosis: Does One Design Fit All? Neurotherapeutics 2015; 12(2):376–83

    Article  CAS  Google Scholar 

  34. Wills A, Hubbard J, Macklin EA, Glass J, Tandan R, Simpson EP et al. Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. The Lancet 2014; 383(9934):2065–72

    Article  CAS  Google Scholar 

  35. Dorst J, Dupuis L, Petri S, Kollewe K, Abdulla S, Wolf J et al. Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study. J Neurol 2015; 262(4):849–58

    Article  Google Scholar 

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Correspondence to Albert Ludolph.

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Die Autoren erklären, dass sie sich bei der Erstellung des Beitrages von keinen wirtschaftlichen Interessen leiten ließen und dass keine potenziellen Interessenkonflikte vorliegen. Der Verlag erklärt, dass die inhaltliche Qualität des Beitrages von zwei unabhängigen Gutachtern geprüft wurde. Die Werbung in dieser Zeitschriftenausgabe hat keinen Bezug zur CME-Fortbildung. Der Verlag garantiert, dass die CME-Fortbildung sowie die CME-Fragen frei sind von werblichen Aussagen und keinerlei Produktempfehlungen enthalten. Dies gilt insbesondere für Präparate, die zur Therapie des dargestellten Krankheitsbildes geeignet sind.

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Witzel, S., Ludolph, A. Amyotrophe Lateralsklerose früh erkennen und behandeln. DNP 20, 56–68 (2019). https://doi.org/10.1007/s15202-019-2110-2

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