Creatine is a supplement that can be bought over the counter at health stores and pharmacies, or can be ordered "on line". Make sure that the one you choose is Consumer Lab approved.

Mechanism of Action: Creatine is an energy chemical that that has been proposed to correct the low energy state that is caused by Huntington's.

Rationale for Use in Huntington's: In several research studies, creatine has decreased brain damage and improved energy function in the brains of HD model mice [Ferrante RJ 2000] and HD people [Hersch SM 2006]. However, mouse studies performed for CHDI foundation found no benefit.

In small pilot human study, it was suggested that it prevented disease progression in HD people for both one and two years [Tabrizi SJ 2003, Tabrizi SJ 2005]. In other studies, creatine has been shown to reduce biomarkers of oxidative injury in brain [Hersch SM 2006]. Though not published yet, Dr. Diana Rosas reported that high dose creatine can decrease brain loss in one region of the brain by 50% over a two year period (see report on HD Lighthouse). Also not published, but presented at a Neuroscience Biomarker meeting, the 20 grams per day dosage has been shown to decrease the oxidative biomarker 8OH2dG to very close to normal levels (see page 31 of the presentation at Institute Of Medicine Forum by Dr. Steven Hersch). Unfortunately these results have not been published in a peer reviewed journal, nor has the oxidative marker study been confirmed.

Dosage: My opinion is that if you take this supplement it should be in the 10 - 15 grams/day, taken in doses twice daily. There is evidence that 5 grams per day is ineffective. I specifically recommend against high doses unless you are in a clinical trial. If you take high doses, it is essential that you are in good health, without other medical problems such as diabetes and kidney disease. And that you must avoid dehydration and have regular kidney and liver blood testing.

Whatever the dose, make sure that the creatine product is Consumer Lab approved. Consumer Lab checks for a toxic chemical (dicyandiamide) that is present in some preparations. This is particularly important when using high dosages. The powder monohydrate form of creatine is best value. Creatine should be stored dry, in a covered container at room temperature. Powders should be taken shortly after mixing with liquid because activity can be lost if kept in liquid form.

Side Effects: Creatine can cause diarrhea, severe muscle cramping or pain, and vomiting. Creatine is a "salt" and at high dosages will cause thirst. Drink plenty of fluids and avoid dehydration. Always divide the dose and take it two times a day.

Sources: Avicena NEOtine is a "medical grade" product is used in clinical trials. Cost for 10 grams per day is $46 per month. NEOtine can be obtained from Avicena's online store.

The sources listed below supply products that are CL (Consumer Lab) approved. Costs vary per manufacturer and distributor, but can be as low as $6-$7 per month for the 10 gram per day dose.

References

Ferrante RJ, Andreassen OA, Jenkins BG, Dedeoglu A, Kuemmerle S, Kubilus JK, Kaddurah-Daouk R, Hersch SM, Beal MF. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci. 2000 Jun 15;20(12):4389-97. PubMed abstract

Hersch SM, Gevorkian S, Marder K, Moskowitz C, Feigin A, Cox M, Como P, Zimmerman C, Lin M, Zhang L, Ulug AM, Beal MF, Matson W, Bogdanov M, Ebbel E, Zaleta A, Kaneko Y, Jenkins B, Hevelone N, Zhang H, Yu H, Schoenfeld D, Ferrante R, Rosas HD. Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG. Neurology. 2006 Jan 24;66(2):250-2. PubMed abstract

Tabrizi SJ, Blamire AM, Manners DN, Rajagopalan B, Styles P, Schapira AH, Warner TT. Creatine therapy for Huntington's disease: clinical and MRS findings in a 1-year pilot study. Neurology. 2003 Jul 8;61(1):141-2. PubMed abstract

Tabrizi SJ, Blamire AM, Manners DN, Rajagopalan B, Styles P, Schapira AH, Warner TT. High-dose creatine therapy for Huntington disease: a 2-year clinical and MRS study. Neurology. 2005 May 10;64(9):1655-6. PubMed abstract