Treatment of amyotrophic lateral sclerosis abroad
Amyotrophic lateral sclerosis (ALS, Lou Gehrig’s disease) – is an incurable, slowly, but stable progressing disease of the central nervous system that is associated with the death of motor neurons (motor nerve cells). It is impossible to treat this disease.
By now, no drugs were found that would considerably slow down the progression of this illness. Amyotrophic lateral sclerosis results in patient’s death in several years after the diagnosis was confirmed.
Causes of Amyotrophic lateral sclerosis
In the majority of cases the cause of amyotrophic lateral sclerosis remains unknown. At this moment, what is known about the etiology of the illness is:
- 10% of cases are hereditary pathologies, over 90% are sporadic. Hereditary form is diagnosed in case if more than one family member suffers from it.
- 7-8% of all the cases are associated with the superoxide dismutase gene mutation that is located onchromosome 21. Mutation is the reason of 25% hereditary form of the disease and also causes 5-7% of sporadic form of ALS.
- Information exists that some agricultural pesticides increase the risk of ALS occurrence.
ALS – is an illness with unclear etiology and development mechanism. We don’t have facts about external risk factors that could contribute to the disease likelihood.
Symptoms of Amyotrophic lateral sclerosis
Illness starts gradually. Amyotrophic lateral sclerosis usually starts with the following symptoms:
- Increasing weakness and awkwardness
- Distal arms’ segments slimming
- Muscle twitching
Within some time, weight loss, weakness, and twitching will spread to the proximal (top) arms segments and additional clinical manifestations appear:
- Strengthening of tendon reflexes
- Occurrence of pathological feet and carpal reflexes
- Tongue twitching
- Reduced tongue sizes
- Speech disorder
- Swallowing violation
- Paresis of the soft palate muscles
Violent laughing or crying, strengthening of the chin reflex, the appearance of oral automatism reflexes might indicate the ALS. These symptoms are characteristic for infants, but resolve with age.
If respiratory muscles are involved into the pathological process, paradoxical breathing (abdominal inspiratory retraction and protrusion on exhalation) may be observed. A patient is not able to hold his head when the head and neck muscles are damaged.
Other symptoms can be observed among the patients who suffer from ALS:
- Hypomimia (absence or decrease in body language)
- Loose closure of the eyeballs
- Limb pain without clear localization
ALS symptoms don’t include the pelvis (urinary incontinence) and oculomotor disorders as they are characteristic only for the terminal stage. Intelectual abilty is not affected in 95% of cases, even at a stage where paralysis of almost all skeletal muscles has developed.
A patient is completely paralyzed at the final disease stage. He cannot move or speak, or eat. Death of the majority of patients occurs as a result of respiratory failure that is caused by respiratory muscles paralysis.
Amyotrophic lateral sclerosis - Diagnostics
All above mentioned symptoms are caused by the death of motor neurons. But they can die not only because of ALS.
There is a group of illnesses that manifest themselves with the same symptoms. ALS can be met rarely - just 1-2 cases per 100,000 a year. That is why, ALS is a diagnosis of exclusion.
Diagnostics is based on the clinical check and electrophysiological methods. Diagnostic criteria are:
- Signs of the central and peripheral motor neurons lesions
- Symptoms progression in dynamics
Besides, it’s important to exclude other pathologies that can cause same signs, including:
- Spondylogenic cervical myelopathy
- Diabetic amyotrophy
- Oncological diseases
- Disseminated sclerosis
- Tick-borne encephalitis
- Duchenne disease
Instrumental diagnostics methods are used for the ALS detection:
- Stimulation and needle electromyography
- MRI of brain and spinal cord
- Cerebrospinal fluid test
- Tests for infections (HIV, syphilis, Lyme disease)
- Molecular genetic analyzes
ALS is considered to be confirmed if central and peripheral motor neurons are damaged in three of central nervous system sections (brain stem, lumbar, cervical, and thoracic sections of spinal cord).
Reliable features are also the results of genetic tests that detected mutations associated with ALS.
Treatment of Amyotrophic lateral sclerosis abroad
Therapy is based on the symptomatic treatment. It includes the intake of medicines, a special diet and lifestyle correction.
Symptomatic therapy is prescribed by the doctor depending on the patient’s needs and disease course peculiarities.
Other ways of ALS treatment:
- Rilutek is a medicine that slows down the neurons death and lengthens patient’s life for a month on average. It also pushes away the need in mechanical ventilation (MV) for about several months.
- Percutaneous endoscopic gastrotomy. During the surgery, a tube is put into the patient’s stomach. It shows out through the abdominal wall. They feed a patient through the tube when he won’t be able to swallow independently.
- HAL-therapy is a new treatment method that is applied only in Japan and Germany. Patient uses the robotic suit for the lower limbs that helps him to move. This way, muscles atrophy slows down and motor functions are preserved for a longer period of time.
- «NurOwn» technology is in the final phase of clinical trials right now. It is developed in Israel and is being studied in several clinics in USA. «NurOwn» technology is based on the use of patient’s stem cells. Injections with stem cells help to protect the motor neurons form damage and also improve the neuro-muscular connections. Preliminary results showed that 87% of patients react to the stem cells treatment.
Amyotrophic lateral sclerosis - Prognosis
Average life expectancy of patients with ALS is 5 years from the moment when the disease started. Occasionally, the disease begins with bulbar disorders. In this case, the expectancy of patients’ life is from 1 to 3 years.
Except for the bulbar disorders, the negative prognostic factor is age. The older the person at the moment of diagnosis, the less is life expectancy.
It is possible that development of the new treatment methods (stem cells injection) will soon improve the existing statistics and will allow patients with ALS to live lon