Amyotrophic lateral sclerosis, designated as ALS, is a severe, rapidly progressive neurodegenerative disease affecting motor neurons involved in the activation of so-called. voluntary muscles – these are all muscles for movement of paramount importance that has our conscious control. These are the muscles of the arms, legs and face. Motoneurons are located in the brain, brainstem and brain grabnachnit and serve as a control point and link between the nervous system and the voluntary muscles. The signals coming from the motoneurons in the brain and brainstem – central motor neuron transmitted to the motoneurons in front horn of the spinal cord – peripheral motor neuron, and hence the muscles performing the appropriate command, ie movement. In amyotrophic lateral sclerosis and two neurons – the central and peripheral damaged muscles and thus receive no signal for their activation. Lost its function, the muscles gradually lose their muscle strength, reduce its weight – hypotrophy and can be observed slight twitching in them – ie. fasciculation. The average incidence of the disease is approximately 3-6 / 100 000, the male sex is affected more often. The average age of appearance of the disease is 60-69 years.
What causes disease?
It is not yet detected a specific cause for the development of amyotrophic lateral sclerosis. Pathogenetically detect cases of abnormal cytoplasmic superoxide dismutase 1, leading to accumulation of free radicals and oxidative stress of cells with subsequent death. This mechanism is most pleasing in familial cases of this disease. The other theory suggests a hypersecretion of glutamate at synapses, which leads to hyperexcitability of motoneurons, with subsequent toxicity and death. Fallen motoneurons release more glutamate, which is the reason for entering a vicious circle from which there is no escape.
How is amyotrophic lateral sclerosis?
Onset of illness is often imperceptible and symptoms are often underestimated. May initially appear fasciculation – twitching muscle cramps, hardening and stiffness – spasticity in the limbs. Develops muscle weakness and reduced muscle mass – atrophy. In some cases appear difficulties in swallowing and speech, which became nasal and slightly drawling.
Many patients first notice difficulty in meeting basic tasks with their hands – when buttoning a shirt, in writing or by placing the key in klyuchalkata.Pri other first leg is affected with difficulty in walking, running and more. When initial complaints by the limbs that form is called spinal and when the first symptoms are impaired artikulatsiya- impaired speech and less difficulty in swallowing, this form is called stem.
When is it diagnosed amyotrophic lateral sclerosis?
To put this diagnosis is important to have symptoms of injury as the central motor neuron – spasticity of the muscles and reinforced tendon reflexes and peripheral motor neuron – muscle weakness, atrophy, cramps and fasciculation.
To put a definite diagnosis, however, must first and foremost to exclude all other diseases with similar clinic. The task is not easy, so the disease often remains undetected for a long time.