Leigh Syndrome: Treatment, Cost and Side Effects

Leigh Syndrome is an inherited rare neurodegenerative condition usually seen in infancy, and is characterized by progressive loss of movement and mental abilities typically resulting in death within 2-3 years due to respiratory failure. This rare genetic disorder is characterized by progressive damage to the central nervous system, and is caused by a defect in the functioning of mitochondria within the cells of your body. Rarely do people develop symptoms of the condition until later stage in life. However, the usual signs of leigh syndrome include diarrhea, vomiting, dysphagia or difficulty in swallowing food, which often results in prevention of growth. Those affected with the disorder may develop involuntary muscle contraction or dystonia, weak muscle tone or hypotonia, difficulty in movement and balance, and loss of sensation in the limbs.

There is no specific treatment for Leigh Syndrome. However, the treatment depends upon its symptoms. After confirming the diagnosis, the doctor usually prescribes Thiamin or Vitamin- B1, Riboflavin or Vitamin B2 and coenzyme Q10. Sodium citrate or sodium bicarbonate is prescribed to manage lactic acidosis. Other than this, a ketogenic diet is prescribed to patients suffering from pyruvate dehydrogenase deficiency. This type of diet is typically a low-carbohydrate, adequate-protein and high-fat diet which in the form of medicine is used to treat refractory epilepsy in children.

There is no specific treatment for Leigh Syndrome. However, the treatment depends upon its symptoms. After confirming the diagnosis, the doctor usually prescribes Thiamin or Vitamin- B1, Riboflavin or Vitamin B2 and coenzyme Q10. Sodium citrate or sodium bicarbonate is prescribed to manage lactic acidosis. Other than this, a ketogenic diet is prescribed to patients suffering from pyruvate dehydrogenase deficiency. This type of diet is typically a low-carbohydrate, adequate-protein and high-fat diet which in the form of medicine is used to treat refractory epilepsy in children.

Individuals with deficiency of the enzyme pyruvate dehydrogenase complex are eligible for the disease. This is characterized by the accumulation of lactic acid in the body and a variety of neurological disorders. The usual signs of leigh syndrome include diarrhea, vomiting, dysphagia or difficulty in swallowing food, which often results in prevention of growth. So if you see any of these following symptoms, you should consult a doctor.

If you do not see any symptoms of leigh syndrome or if you do not have a genetic history of the disease, then you need not undergo any treatment.

Leigh syndrome treatment usually does not bear any side effect. Even if there is, thiamine can cause mild side effects like nausea, mild rash, itching, sweating etc.The symptoms of lactic acidosis is often treated with sodium bicarbonate and sodium citreate. But these medications do not have any impact in healing the disease. they work nly to eleive the symptoms. Oral dichloroacetate (DCA) can have serious side effects like peripheral neuropathy, fatigue, nerve pain and confusion. If coenzyme Q10 goes untolerated, it can cause mild side effects like nausea, vomiting, diarrhoea and loss of appetite. It can also lower your blood pressure and cause skin allergy.

Post treatment guidelines for leigh syndrome usually involves following the prescribed vitamin supplements and medications, maintaining a balanced, nutritional diet with low carbs, high fat and adequate protein.

Since leigh syndrome is not completely curable, the prognosis for individuals suffering from the condition is very poor. The exact time to recover from the disease is not known.

The exact price for the treatment of leigh syndrome is not known.

The results of leigh syndrome treatment are not permanent because the condition is not completely curable. Those with mitochondrial complex deficiency and pyruvate dehydrogenase deficiency survive only a few years, while those with partial deficiencies may live for about 6-7 years.

Alternative treatment for leigh syndrome involves a few home remedies such as consumption of asparagus, flaxseeds, mushrooms, spinach, pistachio, green peas, herbs, fish. These food items mostly contain Vitamin B1 which helps slow down the damage caused by the disease. However, the alternative treatments are only temporary options of treating the condition, and not a permanent cure to it.