Amyotrophic lateral sclerosis: a critical review of riluzole treatment
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Keywords

survival
efficacy
review literature
riluzole
cost-benefit analysis

Abstract

INTRODUCTION: amyotrophic lateral sclerosis (ALS) is a degenerative disease of quickly evolution and inexorably fatal, as a consequence of the degeneration in the motor neurons of cortex, bulb, and spinal cord. Its diagnosis requires upper and lower motor neuron signs. The excitotoxicidad by glutamates is one of its possible causes, but agrees to it understand as a multifactorial alterations. Riluzole is the only one approved medication (FDA in US) to improve the survival of patients with ALS.

OBJECTIVE: to evaluate the efficacy of riluzole in the survival free of assisted ventilation, time free of dysphagia, dysarthria and quality of life in adult patients with ALS compared with placebo or not treatment, a random assignation of patients to intervention and when cut points were measured in a valid an accurately way

RESULTS: of 233 articles, 200 were clinical controlled trials, 27 did not include the riluzole, one was done in children, four were repeated and another was already included in the Cochrane review of 2003. The study of Bensimon in 1999 showed improvement in survival for the patients with ALS of bulbar start. A new study, of the same author in 2001 with 168 patients with advanced illness failed to show differences in favor of the treatment, but concluded that the dose of 50 mgrs. twice a day had the best relation risk/benefit. The Cochrane reviews of 2004 and 2007 concluded that the dose of 100 mgrs day is reasonably safe and can prolong the average survival in two to three months.

CONCLUSION: riluzole can prolong the average life of the patients with ALS. A judicious analysis of cost/opportunity is recommended.


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References

Brooks BR. Clinical Epidemiology of Amyotrophic lateral sclerosis. Neuroepidemiology 1996; 14:399-420.

Norris F, Shepperd R, Dennis E, Mukay E, Elias L, Holden D, Norris H. Onset, natural history and outcome in idiopathic adult motor neuron disease. J Neurol Sci 1993; 118:48-55.

Ringuel SP, Murphy JR, Bryan W, England JD, Miller RG, et al The natural history of amyotrophic lateral sclerosis. Neurology 1993; 43:1316-1322.

Van Damme P, Dewil M, Robberecht W, Van Den Bosch L. Excitotoxicity and amyotrophic lateral sclerosis. Neurodegener Dis. 2005; 2(3-4):147-59.

Van Den Bosch L, Van Damme P, Bogaert E, Robberecht W. The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis. Biochim Biophys Acta 2006 Nov-Dec; 1762(11-12):1068-82.

Paillisse C, Lacomblez L, Dib M, Bensimon G, García-Acosta S, Meininger V. Prognostic factors for survival in amyotrophic lateral sclerosis patients treated with riluzole. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Mar; 6(1):37-44.

Meininger V. Clinical trials in ALS: what did we learn from recent trials in humans? Neurodegener Dis 2005; 2(3-4):208.

Cheung YK, Gordon PH, Levin B. Selecting promising ALS therapies in clinical trials. Neurology 2006 Nov 28; 67(10):1748-51

Miller RG, Mitchell JD, Lyon, M, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database of Systematic Reviews. (4):CD001447.

Bensimon G. Lacomblez L. Delumeau JC. Bejuit R. Truffinet P. Meininger V. RILUZOLE/ALS Study Group II. A study of RILUZOLE in the treatment of advanced stage or elderly patients with amyotrophic lateral sclerosis. Journal of Neurology 249(5):609-15, 2002 May.

Bensimon G., Lacomblez L., Meininger V. for The ALS/Riluzole Study Group. A Controlled Trial of Riluzole in Amyotrophic Lateral Sclerosis. N Engl J Med 1994; 330: 585-591

Lewis P. Rowland. Riluzole for the Treatment of Amyotrophic Lateral Sclerosis -- Too Soon to Tell? N Eng J. Med 1994; 330:636-637.

Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Lancet 1996 May 25;347(9013):1425-31

Yanagisawa N, Tashiro K, Tohgi H, Mizuno Y, Kowa H, Kimuma J, et al. Efficacy and safety of riluzole in patients with amyotrophic lateral sclerosis: double-blind placebo- controlled study in Japan. Igakuno Ayumi 1997;182:851-866

Bensimon G, Lacomblez L, Delumeau JC, Bejuit R, Truffinet P, Meininger V. Riluzole/ALS Study Group. A study of riluzole in the treatment of advanced stage or elderly patients with amyotrophic lateral sclerosis. Journal of Neurology 2002;249(5):609-15.

Miller R, Mitchell J, Lyon M, Moore D. Riluzole for amyotrophic lateral sclerosis. Cochrane Database Syst Rev 2007 Jan 24;( 1):CD001447.

Rowland LP, Schneider NA. Amyotrophic Lateral Sclerosis. N Engl J Med 2001; 344:1688-1700

AAN Clinical Practice Guidelines. American Academy of Neurology 2003 Edition

Xu, Leyan; Yan, Jun; Chen, David; Welsh, Annie M.; Hazel, Thomas; Johe, Karl; Hatfield, et al. Human Neural Stem Cell Grafts Ameliorate Motor Neuron Disease in SOD-1 Transgenic Rats. Transplantation. 82(7):865-875, October 15, 2006.

Veldink JH, Wokke JHJ, van del Wal G et al. Euthanasia and physician assisted suicide among patients with ALS in the Netherlands. N Engl J Med 2002; 346:1638-1644

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