Reduction of acidosis
Lowering of lactic acid level does not reverse the damage done to the Central Nervous System of the patient and all the symptoms.
1.Ketogenic diet
-low carbohydrate but high in protein and fat
-treatment for E1 subunit deficiency of the complex
How it works?
Though the ketogenic diet has been used for a long period of time to treat PDCD,
how the ketogenic diet works treat the disease is still unknown.
The following are proposed mechanistic theories of chronic ketosis action:
- modification of the tricarboxylic acid cycle which increase γ-aminobutyric acid synthesis in the brain
- limit reactive oxygen species generation
- boost energy production in brain tissue.
As a result of the above actions, hyperpolarization of neurons occurs, stabilizing synaptic function and increasing resistance to seizures throughout the brain.
2.Oral citrate
3. Sodium Bicarbonate
Targeting the pyruvate dehydrogenase complex
(supplementation of cofactors to the enzyme)
optimizing the ability of the enzyme
Most PCD are responsive towards these cofactors
Dietary supplements
1.high doses of thiamine
-protect against neuropathy
2. lipoic acid
3. L-carnitine
4.Dichloroacetate
- reduces the inhibitory phosphorylation of pyruvate dehydrogenase complex
- studies shown effectiveness differs in the type of gene deletion that cause the mutation in the gene
After administration of 6 months:
-reduces lactic level after meals
Long-term use associated with:
reversible peripheral neuropathy( may reverse damage of peripheral nervous system)
elevation in liver transaminases(enzymes that indicate liver damage).
Sources
1.Richard E, F. (2012). Pyruvate dehydrogenase complex deficiency treatment & management. In MedScape Reference. Retrieved from http://emedicine.medscape.com/article/948360-treatment
2.Alexander L. Rogovik. (2010). Retrieved from website: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2902940/