INFORMATION ON CEREBRAL BERIBERI

Vitamins are substances that the human body requires but is unable to create and therefore, must obtain outwardly. Deficiencies in three B vitamins, B1 or thiamine, B3 or niacin and B12 or cobalamin are known to cause neurological disorders. Thiamine deficiencies result in a disease called beriberi, which causes peripheral neurological dysfunction and cerebral neuropathy. Niacin deficiencies cause a wasting disease known as pellagra, which affects the skin, mucous membranes, gastrointestinal area as well as the brain, spinal cord and peripheral nerves. Cobalamin deficiencies most often result in the disease pernicious anemia. Neurological symptoms of pernicious anemia include lack of sensation in the extremities, impaired coordination and a ringing in the ears.

CAUSES OF CEREBRAL BERIBERI

Thiamine deficiencies are caused by an insufficient intake of thiamine. In most developed countries, getting enough thiamine is not a problem since it is found in all vegetables, particularly the outer layer of grains. It is sent in refined sugars or fats and is not found in animal tissue. Diets rich in foods that contain thiaminases, enzymes that break down thiamine, such as milled rice, shrimp, mussels, clams, fresh fish and raw meat may be linked with thiamine deficiencies.

Thiamine is absorbed through the digestive tract by a combination of active and passive absorption. It is stored in the body as thiamine diphosphate, also called thiamine pyrophosphate, and thiamine triphosphate. Thiamine diphosphate is the active form and it is used as a coenzyme in a number of steps in cellular respiration. Thiamine may also have an important role in the function of nerve cells independent of cellular respiration. It is found in the cell membranes of nerve axons, and electrical stimulation of nerve cells causes a release of thiamine.

Early thiamine deficiency produces fatigue, abdominal pain, constipation, irritation, loss of memory, chest pain, anorexia and sleep disturbance. As the deficiency progresses, it can be divided as dry beriberi or wet beriberi depending on the activity of the patient. Many persons experience a mixture of the two types of beriberi, although pure forms do occur.

SYMPTOMS OF CEREBRAL BERIBERI

Symptoms occur with equal intensity on both sides of the body and usually start in the legs. Impaired motor and reflex function coupled with pain, numbness and cramps are indicative of the disease. As the disease advances, ankle and knee jerk reactions will be lost, muscle tone in the calf and thigh will atrophy and eventually the patient will suffer from foot drop and toe drop. The arms may begin to show symptoms of neurological dysfunction after the legs are already symptomatic. Histological (tissue) tests may indicate patchy degradation of myelin in muscle tissues.

Wernicke-Korsakoff syndrome, also called cerebral beriberi, occurs in extreme cases of dry beriberi. The premature stage is called Korsakoff's syndrome and it is characterized by confusion, the inability to learn, amnesia and telling stories that bear no relation to reality.

If a person has a high caloric intake and reasonable levels of activity, but has a diet with insufficient thiamine, myocardial dysfunction termed wet beriberi may result. This disease consists of vasodilatation and high cardiac output, preservation of salt and water, and ultimate damage to the heart muscle. A person suffering from wet beriberi will exhibit rapid heartbeat (tachycardia), swelling (edema), high blood pressure, and chest pain.

TREATMENT OF CEREBRAL BERIBERI

In most cases, rapid administration of intravenous thiamine will reduce symptoms of thiamine deficiency. Continued dosages of the vitamin should be continued for several weeks accompanied by a nutritious diet. Following recovery, a diet containing one to two times the recommended daily allowance of thiamine (1-1.5 mg per day) should be maintained.

Niacin deficiency
Niacin deficiency can be treated effectively with replacement of niacin in the diet. In the case of Hartnup disease, large quantities of niacin may be required for effective reversal of symptoms.

Vitamin B12 deficiency
Vitamin B12 deficiency responds well to administration of cobalamin. Because absorption in the small intestine is often part of the problem, the best way to administer cobalamin is by intramuscular injection on a daily basis. After 6 weeks, the injections can be decreased to monthly for the rest of the patient's life. Usually, response to this treatment alleviates all symptoms of the disease. In severe cases, a blood transfusion may be needed and neurological conditions may not be entirely reversed.