Myopathy Mitochondrial Treatment

At this time no cure exists for this group of myopathies, but mitochondrial treatment is possible to ease symptoms. The range of syndromes attributed to mitochondrial myopathy can be mild to severe in their presentation. Many begin in infancy, while others lie dormant until the late teens or even well into adulthood.

Treatment options are broad and include:

  • medical intervention
  • natural remedies
  • physical therapies.

Newer research also indicates that a group of dietary supplements is beneficial. Antioxidants and enzymes may also play a role in such instances as lipid storage myopathy or the general chain of events for energy production within the body’s cells.

Mitochondrial Myopathy and Cell Structure

Mitochondria exist inside each cell in varying numbers. They’re often described as tiny powerhouses that send energy resources throughout the body. Through a series of chain reactions, this provides the ability for every organ, including muscles, brain and heart, to function.

A simplified explanation shows how potential treatments might actually work on misfiring cell behaviors.

  1. Food is consumed and converted to sugar and fat “fuel” molecules.
  2. Proteins pass these molecules into the mitochondria.
  3. Secondary proteins break the fuel molecules down and remove energy particles known as electrons.
  4. A third set of proteins, known as complexes I-V, convert the energy particles to adenosine triphosphate (ATP).
  5. Once distributed in the system, ATP is the energy resource behind bodily functions, including muscle usage and nerve structures.

Many different syndromes lie within the body of diseases known as mitochondrial myopathies. These generally affect muscular tissue first, followed by numerous symptoms that assault the entire body in various ways. Each syndrome is inherited from one parent or both. In some cases, individuals reach adulthood before exhibiting signs of a mitochondrial myopathy. Others develop one of these diseases in infancy or early childhood.

Treatment Options for Mitochondrial Myopathies

Mitochondrial treatment varies according to specific symptoms. As stated before, no cure exists for the genetic mutations that cause this group of diseases. However, research continues around the world into specific causes of gene mutations and how the damage occurs.

In the United Kingdom, National Hospital mitochondrial myopathy research is a focus in its Centre for Neuromuscular Disease. Other facilities, including many in the United States, continue with research into early testing and treatments.

The good news is that many symptoms associated with mitochondrial myopathies are common maladies, both mild and severe. That translates into a more balanced management system with numerous options for medical resources and other therapies.

Generalized symptoms that respond to treatments include:

  • Diabetes – Medications and diet adjustments can keep this condition under control in most cases.
  • Muscle fatigue – Specialized therapies and regular workouts, including resistance and endurance exercise programs, often help reduce extreme muscle fatigue.
  • Heart disorders – These can be managed with drugs and pacemakers.
  • Seizures – Anti-seizure medication can control episodes.
  • Stroke – Medications are available to reduce the risk of stroke.

Unfortunately, severe cases of any of these conditions may result in extreme disability and a shorter lifespan.

Diet as Treatment for Mitochondrial Myopathies

Adjustments or changes in diet can be effective treatment in some cases of mitochondrial myopathies. Because of the defective production of ATP in mitochondria, consuming healthy, energy-producing foods is an aid in the battle against muscle fatigue and exercise intolerance.

With the assistance of a nutritionist, developing a special diet and experimenting with different types of foods may also help reduce the progression of cognitive issues and digestive problems.

New Research in Treating Mitochondrial Myopathy

A handful of supplements are seeing some popularity and results among mitochondrial myopathy patients. Research continues with numerous studies. In the meantime, many of these are generally harmless in recommended quantities.

Antioxidants could one day play a role in treatment, along with specific vitamins and other supplements. It’s very possible that these antioxidants can reduce DNA damage by free radicals. Potentially beneficial antioxidants include:

  • coenzyme Q10
  • idebenone
  • lipoic acid
  • riboflavin
  • vitamin C
  • vitamin E
  • vitamin K.

In particular, three substances potentially have a greater influence on ATP production.

  • Carnitine, sold as L-Carnitine, is an amino acid that works to deliver fuel molecules to each mitochondrion. It can also act as a sweeper for any toxic elements produced in the process of ATP development.
  • Coenzyme Q10 exists in the transport chain of electrons. Research suggests that some mitochondrial diseases may develop from a deficiency of this enzyme.
  • Creatine is a naturally occurring substance that provides backup for ATP production. In the form of “creatine phosphate,” it releases phosphate to provide energy during an initial burst of exercise.

Enzyme treatments also show promise on particular subsets of this disease. Lipid storage mitochondrial myopathy, also known just as lipid storage myopathy, is one such condition in which a series of molecular events works to plant fat cells in the place of muscle.

Developing a Mitochondrial Myopathy Treatment Plan

It’s important to consult a professional specializing in metabolic disorders to develop an effective course of treatment for mitochondrial myopathy. Just as each individual differs in symptoms and severity, a change of diet or reaction to medications will also vary. Biochemical makeup determines how a patient will respond to any treatment.

Finding the proper mitochondrial treatment plan or maintenance program can translate into delaying or lessening symptoms and prolonging life.

Resources (2007). Lipid Storage Myopathies. Retrieved January 9, 2008, from the Encyclopedia Britannica Web site:

Hesterlee, Sharon (n.d.). Mitochondrial Myopathy: An Energy Crisis in the Cells. Retrieved January 9, 2008, from the Mitochondria Research Society Web site: (n.d.). Mitochondrial Myopathies. Retrieved January 9, 2008, from the Muscular Dystrophy Campaign Web site: