All That Is Positive: Dr. Greg on Curcumin, Anti-oxidants, and Exercise

[Guest guest]

Dr. Greg Speaks out for CMT Awareness Week on Curcumin, Exercise, and

Anti-oxidants*

Folks often hear how Charcot-Marie-Tooth disease (CMT) is a rare disease that

isn't really a big deal. I would beg to differ. In fact, the family of

hereditary peripheral neuropathies that makes up CMT comprises some of the most

common neuromuscular disorders affecting humans. Thus CMT is not rare and it

can be a very disabling disease. The severity of CMT varies depending on what

is the causative gene. However it is not quite that simple. We have made huge

strides in identifying the genetic mutations underlying many forms of CMT.

However there is considerable phenotypic variability. This means that two

people who each have the same genotypic form of CMT type (i.e., the same genetic

mutation), may physically look much different. This complicates things and makes

doing research much more difficult.

Our initial longitudinal data collected 20 years ago confirmed that CMT has a

very heterogeneous phenotypical expression. Most folks with CMT will have

prominent atrophy of hand and feet musculature yet there is diffuse muscle

weakness, even affecting proximal muscles. On average, CMT subjects produce

20-40% less force than normal controls when strength is tested with quantitative

isometric and isokinetic measures. In fact, there may be significant weakness

noted of quantitative testing even when manual muscle testing (MMT) shows normal

strength. MMT is what your doctor does in clinic when he or she asks you to

flex your biceps, etc.

So how can we make the nerves in people with CMT work better?

Research is now focusing on new ways to help the body deal with Peripheral

Myelin Protein 22 (PMP22) over expression. Indeed, too much of a good thing is

a bad thing. Advances in molecular biology and genetic manipulation techniques

have allowed the development of animal models of some of these CMT types,

allowing more productive scientific exploration of possible treatments. Recent

treatment advances that have been effective in animal models include oral

supplementation with Curcumin and Vitamin C (ascorbic acid), and the use of

Onapristone, a progesterone antagonist. While it is not clear if those

treatments will be of help, there are many other exciting areas of research that

hold great promise to help people who are dealing with CMT.

Even though Onapristone was effective in a rat model of CMT1A the currently

available progesterone antagonists are too toxic to be safely administered to

humans. However, nerve growth factors are safe and neurotrophin-3 (NT3), a

neurotrophic factor known to promote axonal growth, has shown positive results

in animal models and in a small pilot study in human CMT (1A) patients.

Clinical trials of Vitamin C are on-going and Curcumin trials will likely be

forthcoming. Curcumin may be found in curry, a common ingredient in East Indian

foods.

Our past work showed that cardiopulmonary responses to exercise testing were

markedly abnormal, showing reduced aerobic capacity. This would suggest that

some folks with CMT are out of shape! We now know that gentle, properly done

exercise can translate to improved functional evaluations and timed motor

performance tests. Thus exercise can improve scores on timed motor performance

testing, and translate to less impairment of motor performance skills (and

hopefully reduced disability).

While ongoing molecular genetic research continues to identify more of the

mutant genes and proteins that cause the various disease subtypes, clinical

research should continue to focus on developing pharmaceutical and

rehabilitative therapies to ameliorate nerve degeneration and ultimately improve

function for patients with CMT.

In the meantime CMT patients should exercise and eat a lot of vitamin C,

Curcumin, and other anti-oxidant vitamins. Make sure you medical management is

optimized in a comprehensive, multidisciplinary setting involving neurologists,

physiatrists, orthopedic surgeons, physical and occupational therapists, and

orthotists. All of this treatment should help maximize independence and

quality of life.

Dr/ is Co-Director of the MDA/ALS Center, and a Professor in the School

of Rehabilitation Management at the University of Seattle in Washington.

* This article cannot be reprinted elsewhere without permission of the author.