The disease "amyotrophic lateral sclerosis" is a severe organic pathology of unknown etiology. It is characterized by damage to the lower and upper motor neurons, a progressive course. The ALS (amyotrophic lateral sclerosis) is invariably fatal. Next, we find out how pathology manifests itself and whether there is a possibility of getting rid of the disease.
General information
Amyotrophic lateral sclerosis is considered an incurable pathology. It affects the central nervous system. Pathology has some other names: Charcot's disease, Louis-Gerig, for example. Diagnosis is not always easy. The fact is that in recent years the spectrum of ailments has significantly increased, in one or another clinical manifestation of which not pathology as such, but amyotrophic lateral sclerosis syndrome is noted. In this regard, modern experts consider the most important task to differentiate and clarify the etiology of the disease.
Amyotrophic lateral sclerosis: symptoms
Manifestations of the pathology are associated, first of all, with the defeat of the lower motor neuron. Amyotrophic lateral sclerosis, the symptoms of which include atrophy, weakness, fasciculations, has signs of damage to the cortico-spinal canal. The latter are spasticity and increased tendon and pathological reflexes without sensory disturbances. In this case, cortico-bulbar tracts may be involved. This, in turn, will help accelerate the development of pathology at the level of the brain stem. Amyotrophic lateral sclerosis affects older people. Pathology does not develop until 16 years of age.
Features of manifestation
In patients with amyotrophic lateral sclerosis, muscle atrophy (progressive course) with hyperreflexion is noted in the initial stages of the pathology. This symptom is the most important clinical manifestation. Pathology can begin to develop with any striated muscle fibers. Amyotrophic lateral sclerosis can take several forms: bulbar, high, lumbosacral and cervicothoracic. The cause of death, as a rule, is the defeat of the respiratory muscles after about 3-5 years. Amyotrophic lateral sclerosis has several characteristic features. One of the most common manifestations is considered to be the progressive weakness of the muscle fibers of one of the upper limbs. As a rule, it begins in the brush. With the development of proximal located tissues, the variant of the pathology course is more favorable. At the onset of the disease, associated with the development of muscle weakness in the hand, tenar fibers are involved in the process. The condition is manifested by weak adduction (reduction) and opposition of the thumb. As a result, gripping with the index and thumb is significantly more difficult, subtle motor control is disrupted. Patients with amyotrophic lateral sclerosis feel difficulty dressing (buttoning) and picking up small items. When the leading arm is damaged, difficulties arise when writing, everyday activities in everyday life. Relatively favorable is considered the lumbosacral form.
Typical course of pathology
In this case, stable progressive involvement of the muscles of the same limb into the process is noted. Subsequently, the spread to the second arm begins before the lesion of the bulbar fibers or legs. Amyotrophic lateral sclerosis can begin with the muscles of the tongue, mouth, face, or lower extremities. In this case, subsequent defeats do not “catch up” with the initial ones. In this regard, the shortest life is considered to be with bulbar form. Patients die, remaining virtually on their feet, without waiting for paralysis of the lower extremities.
Further development
Amyotrophic lateral sclerosis is accompanied by various combinations of signs of paralysis (bulbar and pseudobulbar). This is mainly manifested by dysphagia and dysarthria, and subsequently respiratory disorders. A characteristic sign of almost all forms of pathology is the early strengthening of the mandibular reflex. When swallowing liquid food, dysphagia is noted more often than with solid. At the same time, it should be said that the use of the latter in the course of the progression of pathology is significantly difficult. There is weakness in the masticatory muscles, the soft palate begins to hang, the tongue becomes atrophic and motionless. Amyotrophic lateral sclerosis begins to be accompanied by a continuous course of saliva, anarthria. It becomes impossible to swallow, the likelihood of aspiration pneumonia increases. It should also be noted that krampi (periodically occurring cramps in the calves) are noted in all patients and are often considered the first signs of pathology.
Spread of atrophy
It should be said that it is quite selective. On the patient’s hands there is a lesion of hypotenar, tenar, interosseous and deltoid muscles. Atrophy on the legs develops in areas that perform dorsal flexion of the foot. From the bulbar muscles, tissues of the soft palate and tongue are damaged. The oculomotor fibers are considered the most resistant to atrophy.
Practical observations
With pathology, sphincter disorders are rare. One of the amazing features of the disease is the absence of pressure sores in bedridden patients paralyzed for a long time. It was also found that dementia appears extremely rarely in pathology. Only a few subgroups are considered an exception: the family form and the Parkinsonism-BA sclerosis-dementia complex. Cases with uniform involvement of the lower or upper motor neurons have also been described. In this case, damage to any one zone may predominate. For example, the upper motor neuron may suffer more. In this case, they speak of primary lateral sclerosis. Predominant damage may be noted in the lower motor neuron. In this case, they talk about anteroligar syndrome.
Electromyography
This method has special diagnostic value among paraclinical methods of studying pathology. Using electromyography, common damage is detected in the cells of the front horns (including the preserved (clinically) muscles) with fasciculations, fibrillations, changes in the potentials of the motor units, positive waves against the background of the normal speed of excitation propagation through the fibers in the sensory nerves.
Diagnostics
To conduct studies to identify pathology, the presence of:
- Signs of damage to the lower motor neuron. It is necessary, including EMG confirmation in stored (clinically) muscles
- Symptoms of damage to the upper motor neuron in a progressive course.
For diagnosis, the absence of:
- Sphincter disorders.
- Sensory impairment.
- Parkinson's disease.
- Visual impairment.
- ALS-mimicking manifestations.
- Alzheimer's type of dementia.
- Vegetative disorders.
The diagnosis is confirmed by fasciculations in 1 or more zones, EMG signs, normal speed of excitation propagation along sensory and motor fibers. Among the categories it should be noted:
- Reliable ALS. In this case, we are talking about the revealed signs of damage in the lower motor neuron and the upper in three parts of the body.
- Likely. In this case, there are symptoms of damage in the lower and motoneuron and upper in two parts of the body.
- Possible. Signs of damage to the lower motor neuron and the upper in one part of the body or signs of damage to the second in 2-3 zones were revealed. In the latter case, we are talking about the manifestations of ALS in one limb, progressive bulbar paralysis and primary form.
Treatment of amyotrophic lateral sclerosis
The first and only drug used in pathology today is Riluzol. It is approved in Europe and the USA, but is not registered in the Russian Federation and cannot be officially recommended by a doctor. The medication does not relieve pathology. But this is the only drug that has a beneficial effect on the life expectancy of patients. Medication "Riluzole" helps to reduce the concentration of glutamate (a mediator in the central nervous system) released during the passage of a nerve impulse. An excess of this compound, as was established during observation, has a destructive effect on neurons of the spinal cord and brain. According to the results of clinical studies, it was found that people taking the medication lived on average 2-3 months longer than those who used a placebo.
Antioxidants
This class of nutritional compounds helps the body prevent the damage that free radicals can cause. It is believed that individuals with amyotrophic lateral sclerosis may be more susceptible to their negative effects. Today, research is being conducted to identify, detect the beneficial effects of supplements containing antioxidants. Some of these drugs that have passed the test, have not proved their effectiveness.
Concomitant therapy
Various activities can make life easier for patients, make it more comfortable. In particular, we are talking about relaxation. It is believed that reflexology, aromatherapy and massage contribute to the elimination of stress and reduce anxiety, muscle relaxation, normalization of lymph and blood flow. Such procedures help relieve pain through the synthesis of endogenous painkillers produced by the body itself, as well as endorphins. This or that medicine or any procedure should be recommended by the doctor. With amyotrophic lateral sclerosis, self-medication is highly not recommended.