Creatine for amyotrophic lateral sclerosis/motor neuron disease

Pastula, Daniel M.; Moore, Dan H.; Bedlack, Richard

doi:10.1002/14651858.cd005225.pub2

Cited by 34 publications

(8 citation statements)

References 38 publications

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“…As respiratory dysfunction is mainly caused by muscle weakness and produces a restrictive pattern, one of the most utilized measures in clinical trials is the forced vital capacity (FVC) [22,27,38]. FVC essentially measures the full extent of a breath capable by a patient, excluding the dead space of the lung and upper airways.…”

Section: Respiratory Functionmentioning

confidence: 99%

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Rutkove

2015

Neurotherapeutics

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Progressive weakness remains the clinical hallmark of amyotrophic lateral sclerosis (ALS). Accordingly, a variety of tools has been developed to capture this disease feature, including questionnaires, such as the ALS-functional rating scale, strength testing, pulmonary function tests, electrophysiologic measures, including motor unit number estimation, and imaging techniques. Despite this plethora of approaches, there is little agreement as to what measures to use in a given clinical trial or in the clinic during routine patient care. Part of the reason for this uncertainty is that ALS is a remarkably protean disease. Some individuals progress rapidly, others slowly; some patients have considerable upper motor neuron dysfunction, whereas others have little; and there is considerable variation in the sequence of body regions affected, in some the disease beginning in the bulbar musculature and in others in one arm or one leg. Here, I present a variety of basic and more complex clinical measures for potential use in therapeutic trials with the aim of offering a balanced and practical set of recommendations, as well as considerations for future studies.

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Section: Respiratory Functionmentioning

confidence: 99%

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Rutkove

2015

Neurotherapeutics

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“…Supplements of creatine, an organic acid involved in ATP production, have been correlated with increased survival time in ALS mouse models. 155 Unfortunately, human trials with creatine supplements have had mixed results.…”

Section: Applicationsmentioning

confidence: 99%

Metal imaging in neurodegenerative diseases

2012

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Metal ions are known to play an important role in many neurodegenerative diseases including Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and prion diseases. In these diseases, aberrant metal binding or improper regulation of redox active metal ions can induce oxidative stress by producing cytotoxic reactive oxygen species (ROS). Altered metal homeostasis is also frequently seen in the diseased state. As a result, the imaging of metals in intact biological cells and tissues has been very important for understanding the role of metals in neurodegenerative diseases. A wide range of imaging techniques have been utilized, including X-ray fluorescence microscopy (XFM), particle induced X-ray emission (PIXE), energy dispersive X-ray spectroscopy (EDS), laser ablation inductively coupled mass spectrometry (LA-ICP-MS), and secondary ion mass spectrometry (SIMS), all of which allow for the imaging of metals in biological specimens with high spatial resolution and detection sensitivity. These techniques represent unique tools for advancing the understanding of the disease mechanisms and for identifying possible targets for developing treatments. In this review, we will highlight the advances in neurodegenerative disease research facilitated by metal imaging techniques.

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“…Traditional meta-analysis, which aggregates the results of multiple, heavily overlapping clinical/epidemiological studies into a larger virtual study from which relationships across a broader array of conditions can be examined and overall statistical power can be increased, has been successfully used to examine individual clinical treatments (Miller, Mitchell et al 2007;Pastula, Moore et al 2010). Much can and has been honed from using traditonal meta-analysis to examine clinical trials.…”

Section: Dynamic Meta-analysis As a Means Of Experimental And Clinicamentioning

confidence: 99%