Clinical use of creatine in neuromuscular and neurometabolic disorders

[Guest guest]

Subcell Biochem. 2007;46:183-204.

Clinical use of creatine in neuromuscular and neurometabolic

disorders.

Tarnopolsky MA.

Department of Pediatrics and Medicine (Neurology and Rehabilitation),

Neuromuscular and Neurometabolic Clinic, Rm 2H26, McMaster University

Medical Center, 1200 Main St. W., Hamilton, Ontario, Canada, L8N 3Z5.

Many of the neuromuscular (e.g., muscular dystrophy) and

neurometabolic (e.g., mitochondrial cytopathies) disorders share

similar final common pathways of cellular dysfunction that may be

favorably influenced by creatine monohydrate (CrM) supplementation.

Studies using the mdx model of Duchenne muscular dystrophy have found

evidence of enhanced mitochondrial function, reduced intra-cellular

calcium and improved performance with CrM supplementation. Clinical

trials in patients with Duchenne and Becker's muscular dystrophy have

shown improved function, fat-free mass, and some evidence of improved

bone health with CrM supplementation.

In contrast, the improvements in function in myotonic dystrophy and

inherited neuropathies (e.g., Charcot-Marie-Tooth) have not been

significant. Some studies in patients with mitochondrial cytopathies

have shown improved muscle endurance and body composition, yet other

studies did not find significant improvements in patients with

mitochondrial cytopathy. Lower-dose CrM supplementation in patients

with McArdle's disease (myophosphorylase deficiency) improved

exercise capacity, yet higher doses actually showed some indication

of worsened function. Based upon known cellular pathologies, there

are potential benefits from CrM supplementation in patients with

steroid myopathy, inflammatory myopathy, myoadenylate deaminase

deficiency, and fatty acid oxidation defects.

Larger randomized control trials (RCT) using homogeneous patient

groups and objective and clinically relevant outcome variables are

needed to determine whether creatine supplementation will be of

therapeutic benefit to patients with neuromuscular or neurometabolic

disorders. Given the relatively low prevalence of some of the

neuromuscular and neurometabolic disorders, it will be necessary to

use surrogate markers of potential clinical efficacy including

markers of oxidative stress, cellular energy charge, and gene

expression patterns.

[Guest guest]

This was my Neuro Dr for many years. He was the first to say I had CMT. His

partner now has me as a patient as the new was going to be the " CMT Guy " .

Have a wonderful Saturday.

Matt from Canada.