A motor neuron is a nerve cell that serves to transmit information between the nervous system and muscles in the body. There is an upper and a lower motor neuron. The upper is located in the brain, and the lower in the spinal cord. When you want to make a movement (eg grab a glass) then a command is transmitted via the upper motor neuron in the brain to the lower motor neuron in the spinal cord which in turn transmits the command to the group of arm muscles involved in that action.
In ALS, the upper and lower motor neurons fail, leading to a weaker and then to a cessation of transmission between the brain and the voluntary muscles. There is a deterioration of the muscles that atrophy (disappear), weaken and there is the appearance of fasciculations (the feeling of muscle vibration under the skin). The disease is more common in men and most often occurs between 40. and 70. that year although it can occur in any age group.
Symptoms of the disease
The disease’s characteristic is that all the muscles in the body can be affected so that in addition to the muscles of the arms and legs, the muscles that participate in speech, chewing, swallowing, and breathing are also weakened. The ability to reason, think, look, feel, taste, smell, and hear, and the sense of emptying the bladder and intestines remain intact.
The disease begins slowly with a mild muscle group weakness and gradually affects others. It usually begins with weakness in the arms, legs and eventually spreads to speech, swallowing, and breathing. Less commonly, it can begin with swallowing and speech disorders called bulbar symptoms. As the disease progresses, the muscles’ weakness increases, and eventually, they become completely taken away.
Symptoms of ALS can be:
- weakness in the arms or legs (e.g., clumsiness occurs, difficulty in buttoning, tying shoes, turning a key in a lock, cutting, getting out of bed, washing, etc.).
- speech problems (speech is quieter and slurred, speech “through the nose,” difficulty in pronouncing longer words and sentences, slurred)
- disturbances in chewing and swallowing first some food, and finally every form of food and saliva
- at the end of the respiratory disturbance, which is why they must be connected to artificial ventilation apparatus
The last stage occurs on average within 3-5 years from the disease’s onset, although some patients survive for up to ten years. The cause of death is mainly complications of the most common infection, such as pneumonia.
The cause of ALS is unknown. In 90% of cases, it occurs sporadically, while about 10% occur as a hereditary form. In 20% of hereditary cases, a change was found in enzyme one gene (SOD1). Science is still looking for other gene changes that could play a role in ALS onset.
In sporadic cases, it is thought that the cause could be in the increased activity of glutamate (a substance in the nervous system, a type of neurotransmitter, a transmitter) that nerve cells use to transmit signals. Patients with ALS have too much glutamate, which can cause nerve cell death. Other possible causes, such as viruses, environmental toxins, and others, are also under investigation.
Diagnostic tests
ALS is a disease that is difficult to diagnose at first. There is no single test that can make a safe diagnosis, and it is necessary to do more tests and rule out other similar diseases.
In addition to the neurological examination, they are made depending on the clinical picture:
- EMNG – a method that examines the electrical activity in muscles and nerve conduction
- MR of the brain and spinal cord
- laboratory blood tests and urine, cerebrospinal fluid, etc.
- sometimes a muscle biopsy
ALS therapy
Unfortunately, ALS therapy does not yet exist. The only drug I have approved so far is RILUZOL (RILUTEK), which is believed to slow down the development of the disease and prolong the patient’s life for a shorter time (approximately three months). It works by reducing the level of glutamate in the nervous system. It does not affect mortality, i.e., there is no difference in the number of deaths in patients taking Rilutek and those who did not. It must not be given to liver disease patients, and patients taking it must constantly monitor liver function via liver enzymes (AST, ALT, and GGT).
Other medicines used in people with ALS help relieve certain symptoms such as stiffness in the arms and legs (Baclofen-Lioresal), irregular stools, severe salivation (difficulty swallowing) of saliva that cannot be swallowed), pain, depression etc.
Milder physical therapy is also useful in exercises to preserve strength and mobility in muscles and joints.
Speech exercises in which speech techniques are learned for easier pronunciation and comprehension. Afterward, patients who are no longer able to speak can use a personal computer and thus communicate.
As the disease progresses, respiratory problems occur, so it is necessary to facilitate breathing with an oxygen mask and an apparatus for blowing air into the lungs. Finally, it is necessary to connect to a respirator (breathing apparatus).
Dietary recommendations
Many ingredients added to food products today, whether used in processing or in improving the taste and appearance of food, are known to be neurotoxic, and some foods are believed to be neuroprotective.
Recommendations:
1. eliminate all processed foods from your diet and foods containing artificial colors, additives, artificial flavors, artificial sweeteners, and preservatives.
One of the most dangerous food supplements is glutamate because patients with ALS already have elevated levels of glutamate in their bodies. Namely, there is a difference between natural glutamic acid, which is found in natural, unprocessed food and necessary for the functioning of the human body, and artificially obtained glutamic acid, which is neurotoxic.
In short, any semi-finished or finished food product most likely contains glutamate, which includes ready-made food additives, ready-made soups, soup cubes, gelatin, ultra-pasteurized dairy products, sauces, and ready-made salad dressings, and so on.
2. avoid Aspartate – artificial sweetener and, like other artificial sweeteners, should be eliminated from the diet
3. throw out gluten-containing foods (a protein found in wheat, barley, and rye)
Foods that do NOT contain gluten are rye, buckwheat, soy, potatoes, and corn, and this is allowed.
4. throw out dairy products since they contain glutamate due to the process of “ultra pasteurization” when milk is subjected to high temperatures.
5. throw out the sugar (replacement Xylitol recommended)
In short, eat as many fresh fruits, vegetables, meat, and eggs from organic farming, fish in moderation, potatoes, corn, soy, rye, buckwheat, oils rich in omega 3 and 9 such as olive oil, sesame oil, seeds, hazelnuts, almonds, walnuts (not peanuts). Prepare freshly cooked food without ready-made food additives. Eat less and more often.
Conclusion
ALS is a severe and fatal disease. With a good knowledge of the course of the disease, possible complications, and opportunities provided in helping to treat at home, it is possible to facilitate treatment and prepare for the difficulties that follow and affect the patient and his family.
