Effects of riluzole on symptom progression in amyotrophic lateral sclerosis

Sojka, Peter; Andersen, P. M.; Forsgren, Lars

doi:10.1016/s0140-6736(05)60977-4

Cited by 18 publications

(9 citation statements)

References 4 publications

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“…Nonetheless, the staggering complexity of this disorder and its fast progression have hampered the efforts to find an effective treatment. As a result, riluzole is the only FDA-approved drug for this disease, leading to a modest increase in survival (9).…”

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confidence: 99%

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

Ferraiuolo

Meyer

Sherwood

et al. 2016

Proc. Natl. Acad. Sci. U.S.A.

150

142

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Oligodendrocytes have recently been implicated in the pathophysiology of amyotrophic lateral sclerosis (ALS). Here we show that, in vitro, mutant superoxide dismutase 1 (SOD1) mouse oligodendrocytes induce WT motor neuron (MN) hyperexcitability and death. Moreover, we efficiently derived human oligodendrocytes from a large number of controls and patients with sporadic and familial ALS, using two different reprogramming methods. All ALS oligodendrocyte lines induced MN death through conditioned medium (CM) and in coculture. CM-mediated MN death was associated with decreased lactate production and release, whereas toxicity in coculture was lactate-independent, demonstrating that MN survival is mediated not only by soluble factors. Remarkably, human SOD1 shRNA treatment resulted in MN rescue in both mouse and human cultures when knockdown was achieved in progenitor cells, whereas it was ineffective in differentiated oligodendrocytes. In fact, early SOD1 knockdown rescued lactate impairment and cell toxicity in all lines tested, with the exclusion of samples carrying chromosome 9 ORF 72 (C9orf72) repeat expansions. These did not respond to SOD1 knockdown nor did they show lactate release impairment. Our data indicate that SOD1 is directly or indirectly involved in ALS oligodendrocyte pathology and suggest that in this cell type, some damage might be irreversible. In addition, we demonstrate that patients with C9ORF72 represent an independent patient group that might not respond to the same treatment.oligodendrocytes | amyotrophic lateral sclerosis | SOD1 | C9orf72 | lactate

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confidence: 99%

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

Ferraiuolo

Meyer

Sherwood

et al. 2016

Proc. Natl. Acad. Sci. U.S.A.

150

142

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“…Other trials of riluzole have been undertaken (Yanagisawa et al 1997;Sojka et al 1997;Riviere et al 1998;Kalra et al 1998;Desiato et al 1999;Arriada-Mendicoa et al 1999;Pongratz et al 2000;Couratier et al 2000;Debove C et al 2001;Lacomblez et al 2002), but the only trial to qualify for the Cochrane Review (Miller et al 2002), in addition to the two above, was of 168 patients outside the inclusion criteria for the Lacomblez and colleagues dose-ranging study . For the primary outcome measure (mortality or tracheostomy), at all time points at a dose of 100 mg/day, there was a 16% relative hazard reduction which did not quite reach statistical signifi cance (P = 0.056).…”

Section: The Meta-analysismentioning

confidence: 99%

Riluzole and Motor Neurone Disease

et al. 2003

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“…Four RCTs were found that met the inclusion criteria for this review. [42][43][44][45] Eight further papers [46][47][48][49][50][51][52][53] that were possibly eligible based on their titles and abstracts were examined, but excluded for reasons explained below. None of the excluded studies were RCTs.…”

Section: Studies Identifiedmentioning

confidence: 99%

“…The study by Riviere and co-workers (1998) 46 re-analysed previous trial data, and was therefore excluded. Trials by Sojka and colleagues (1997), 47 Kalra and colleagues (1998), 48 Gawel (unpublished; 1999), 49 Desiato and co-workers (1999) 51 and Couratier and co-workers (2000) 53 were excluded because subjects were not randomised. The trials by Arrida-Mendicoa and colleagues (1999) 50 and Pongratz and co-workers (1999) 52 were excluded because they did not use controls.…”

Section: Excluded Studiesmentioning

confidence: 99%

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The clinical effectiveness and cost-effectiveness of riluzole for motor neurone disease: a rapid and systematic review

Stewart

Sandercock

Bryan

et al. 2001

Health Technol Assess

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T he NHS R&D Health Technology Assessment (HTA) Programme was set up in 1993 to ensure that high-quality research information on the costs, effectiveness and broader impact of health technologies is produced in the most efficient way for those who use, manage and provide care in the NHS. The research reported in this monograph was commissioned by the HTA Programme on behalf of the National Institute for Clinical Excellence (NICE). Rapid reviews are completed in a limited time to inform the appraisal and guideline development processes managed by NICE. The review brings together evidence on key aspects of the use of the technology concerned. However, appraisals and guidelines produced by NICE are informed by a wide range of sources. The research reported in this monograph was funded as project number 00/01/01. The views expressed in this publication are those of the authors and not necessarily those of the HTA Programme, NICE or the Department of Health. The editors wish to emphasise that funding and publication of this research by the NHS should not be taken as implicit support for any recommendations made by the authors. Criteria for inclusion in the HTA monograph series Reports are published in the HTA monograph series if (1) they have resulted from work commissioned for the HTA Programme, and (2) they are of a sufficiently high scientific quality as assessed by the referees and editors. Reviews in Health Technology Assessment are termed 'systematic' when the account of the search, appraisal and synthesis methods (to minimise biases and random errors) would, in theory, permit the replication of the review by others.

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Effects of riluzole on symptom progression in amyotrophic lateral sclerosis

Cited by 18 publications

References 4 publications

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

Riluzole and Motor Neurone Disease

The clinical effectiveness and cost-effectiveness of riluzole for motor neurone disease: a rapid and systematic review

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