Nervenheilkunde 2007; 26(05): 362-367
DOI: 10.1055/s-0038-1626871
Original Article
Schattauer GmbH

Therapieoptionen bei der Amyotrophen Lateralsklerose (ALS)

Therapy options in amyotrophic lateral sclerosis (ALS)
J. Dorst
1   Abteilung für Neurologie, Universitätsklinikum Ulm
,
P. Kuehnlein
1   Abteilung für Neurologie, Universitätsklinikum Ulm
,
A. D. Sperfeld
1   Abteilung für Neurologie, Universitätsklinikum Ulm
,
A. C. Ludolph
1   Abteilung für Neurologie, Universitätsklinikum Ulm
› Author Affiliations
Further Information

Publication History

Eingegangen am: 11 February 2007

angenommen am: 15 February 2007

Publication Date:
20 January 2018 (online)

Zusammenfassung

Die Amyotrophe Lateralsklerose (ALS) ist eine chronischprogredient verlaufende Erkrankung, die vorwiegend das motorische Nervensystem betrifft und zu fortschreitenden Muskelatrophien und Paresen führt. Die Ätiologie der ALS ist weitgehend unbekannt, was die Entwicklung kausaler Therapiemöglichkeiten erschwert. Eine kurative Behandlungsmöglichkeit existiert nicht. Auf der Basis neuer Zugänge zur Pathogenese der Erkrankung sind in den letzten Jahren zahlreiche potenziell wirksame Substanzen entdeckt worden, welche im Tierversuch teilweise eine Verlangsamung des Krankheitsverlaufs bewirkten, in kontrollierten Therapiestudien am Menschen aber zumeist enttäuschende Ergebnisse lieferten. Daher kommt der palliativen ALS-Therapie mit der Behandlung häufiger Symptome wie Hypoventilation, Gewichtsabnahme oder Speichelfluss eine wichtige Bedeutung zu. Der folgende Artikel gibt eine Übersicht über den aktuellen Stand der kausalen und symptomatischen Therapiemöglichkeiten.

Summary

Amyotrophic lateral sclerosis (ALS) is a chronic progressive disease which affects predominantly the motor nervous system and leads to muscle atrophies and paralysis. The development of causal therapy options is difficult since the etiology of ALS is largely unknown. Based on new pathogenetic insights several potentially effective substances have been discovered recently which caused a slowdown of the disease in animal experiments. However, most of them showed disappointing results in placebo controlled studies in humans. The symptomatic therapy of ALS includes the treatment of frequent symptoms like hypoventilation, malnutrition, or pseudohypersalivation. This article describes current views of causal and symptomatic therapy options.

 
  • Literatur

  • 1 Bensimon G, Doble A. The tolerability of riluzole in the treatment of patients with amyotrophic lateral sclerosis. Expert Opin Drug Saf 2004; 03 (06) 525-534.
  • 2 Bensimon G, Lacomblez L, Meininger V. The ALS/Riluzole Study Group: A randomized controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 1994; 330: 585-591.
  • 3 Bortolotti M. Laryngospasm and reflex central apnoea caused by aspiration of reflexed gastric content in adults. Gut 1989; 30: 233-238.
  • 4 Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006; 05 (02) 140-147.
  • 5 Butz M, Wollinsky KH, Wiedemuth-Catrinescu U, Sperfeld A, Winter S, Mehrkens HH, Ludolph AC, Schreiber H. Longitudinal effects of noninvasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis. Am J Phys Med Rehabil 2003; 82 (08) 597-604.
  • 6 Cudkowicz ME, Shefner JM, Schoenfeld DA, Brown Jr RH, Johnson H, Qureshi M, Jacobs M, Rothstein JD, Appel SH, Pascuzzi RM, Heiman TDPatterson, Donofrio PD, David WS, Russell JA, Tandan R, Pioro EP, Felice KJ, Rosenfeld J, Mandler RN, Sachs GM, Bradley WG, Raynor EM, Baquis GD, Belsh JM, Novella S, Goldstein J, Hulihan J. Northeast ALS Consortium.A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurology 2003; 61 (04) 456-464.
  • 7 Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P. Nutritional status is a prognostic factor for survival in ALS patients. Neurology 1999; 53 (05) 1059-1063.
  • 8 Desnuelle C, Dib M, Garrel C, Favier A. A doubleblind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group. Amyotroph Lateral Scler Other Motor Neuron Disord 2001; 02 (01) 9-18.
  • 9 Dupuis L, Oudart H, Rene F, Gonzalez de Aguilar JL, Loeffler JP. Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model. Proc Natl Acad Sci USA 2004; 101 (30) 11159-11164 Epub 2004 Jul 19..
  • 10 Forbes RB, Colville S, Swingler RJ. Scottish Motor Neurone Disease Research Group. Frequency, timing and outcome of gastrostomy tubes for amyotrophic lateral sclerosis/motor neurone disease –a record linkage study from the Scottish Motor Neurone Disease Register.J Neurol 2004; 251 (07) 813-817.
  • 11 Giess R, Naumann M, Werner E, Riemann R, Beck M, Puls I, Reiners C, Toyka KV. Injections of botulinum toxin A into the salivary glands improve sialorrhoea in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2000; 69 (01) 121-123.
  • 12 Graf M, Ecker D, Horowski R, Kramer B, Riederer P, Gerlach M, Hager C, Ludolph AC, Becker G, Osterhage J, Jost WH, Schrank B, Stein C, Kostopulos P, Lubik S, Wekwerth K, Dengler R, Troeger M, Wuerz A, Hoge A, Schrader C, Schimke N, Krampfl K, Petri S, Zierz S, Eger K, Neudecker S, Traufeller K, Sievert M, Neundorfer B, Hecht M. German vitamin E/ALS Study Group. High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebocontrolled double-blind study. J Neural Transm 2005; 112 (05) 649-660 Epub 2004 Oct 27..
  • 13 Groeneveld GJ, Veldink JH, van der Tweel I, Kalmijn S, Beijer C, de Visser M, Wokke JH, Franssen H, van den Berg LH. A randomized sequential trial of creatine in amyotrophic lateral sclerosis. Ann Neurol 2003; 53 (04) 437-445.
  • 14 Iannaccone S, Ferini-Strambi L. Pharmacologic treatment of emotional lability. Clin Neuropharmacol 1996; 19: 532-535.
  • 15 Man-Son-Hing M, Wells G. Meta-analysis of efficacy of quinine for treatment of nocturnal leg cramps in elderly people. Br Med J 1995; 310: 13-17.
  • 16 Mathus-Vliegen LM, Louwerse LS, Merkus MP, Tytgat GN, Vianney de Jong JM. Percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis and impaired pulmonary function. Gastrointest Endosc 1994; 40 (04) 463-469.
  • 17 Mazzini L, Corra T, Zaccala M, Mora G, Del Piano M, Galante M. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol 1995; 242 (10) 695-698.
  • 18 Meininger V, Asselain B, Guillet P, Leigh PN, Ludolph A, Lacomblez L, Robberecht W. Pentoxifylline European Group. Pentoxifylline in ALS: a double-blind, randomized, multicenter, placebocontrolled trial. Neurology 2006; 66 (01) 88-92.
  • 19 Miller RG, Mitchell JD, Lyon M, Moore DH. Riluzol for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Amyotroph Lateral Scler Other Motor Neuron Disord 2003; 04: 191-206.
  • 20 Norris FUK, Sachais B, Carey M. Trial of bacolfen in amyotrophic lateral sclerosis. Arch Neurol 1979; 36: 715-716.
  • 21 Schiffer R, Herndon R, Rudick R. Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med 1985; 312: 1480-1482.
  • 22 Sperfeld AD, Hanemann CO, Ludolph AC, Kassubek J. Laryngospasm: An underdiagnosed symptom of X-linked spinobulbar muscular atrophy. Neurology 2005; 64: 753-754.
  • 23 Talmi Y, Finkelstein Y, Zohar Y. Reduction of salivary flow in amyotrophic lateral sclerosis with scopoderm TTS. Head Neck 1989; 11: 565.
  • 24 Winterholler M, Hecht M, Erbguth F, Neundörfer B. Überleben mit Heimbeatmung. Nervenarzt 68: 351-357.
  • 25 Marinella MA. The refeeding syndrome and hypophosphatemia. Nutrition Reviews 2003; 61: 320-323.