Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis, or motor neuron disease - a debilitating disease of various etiologies, characterized by rapidly progressive weakness, muscle atrophy, muscle spasticity, speech problems (dysarthria), difficulty swallowing (dysphagia), and shortness of breath (dyspnea). Amyotrophic lateral sclerosis is the most common of the five motor neuron diseases. Muscle weakness and atrophy of the whole body caused by degeneration of upper and lower motor neurons. Muscles that are not able to function, weaken and atrophy. Ill eventually they may lose the ability to initiate and control all voluntary movement.
The symptoms of amyotrophic lateral sclerosis
Early symptoms of amyotrophic lateral sclerosis, as a rule, are obvious weakness and muscle atrophy. Other symptoms include muscle twitching, spasms, stiffness of affected muscles and slurred speech. Condition of the body affected by early symptoms of amyotrophic lateral sclerosis, depends on what the motor neurons in the body were damaged first.
In about 75% of the patients with muscle atrophy begins the hands or feet. Patients with atrophy of the legs may feel uncomfortable when walking or running, stumble and fall to the ground. Patients with atrophy of the hands may have difficulty in movements requiring manual dexterity such as buttoning buttons, zasovyvanie or turning the key in the lock. Sometimes symptoms distributed only on one limb over a long period of time or throughout the illness.
In about 25% of patients, the disease begins with difficulty speaking or swallowing. It may become muffled and very quiet. Other symptoms include difficulty swallowing and loss of mobility of the tongue.
In a minority of patients, the disease begins with atrophy of the intercostal muscles that support breathing.
Over time, all patients manifest symptoms of amyotrophic lateral sclerosis, as:
- Difficulty moving;
- Trouble with swallowing (dysphagia), and speaking or forming words (dysarthria);
- Muscle stiffness (spasticity);
- Hyperreflexia (including overactive gag reflex);
- Muscle cramps;
- Fleeting twitching muscles.
Approximately 15-45% of patients experience pseudobulbar affect (emotional lability), which manifests itself in uncontrollable laughter, the patient abruptly starts to cry or smile, exaggerated to express their emotions.
Although the order and speed of the symptoms varies from person to person, in the end, most patients are unable to walk, get out of bed on their own, or use their hands. The rate of progression can be measured using a functional scale of amyotrophic lateral sclerosis, is determined by the clinical conversation, consisting of 12 questions.
Progression of the disease is usually slower in patients who are younger than 40 years - the disease is limited mainly to one limb.
The symptoms of late stage amyotrophic lateral sclerosis are:
- Difficulty swallowing and chewing food, giving through the effort and increases the risk of choking or aspiration of food into the lungs;
- The weakness of the diaphragm and intercostal muscles that support breathing and lung function, such as forcing lung capacity and a decrease in pressure during inspiration.
Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms. The average life expectancy from the beginning to the death of about 39 months, and only 4% live longer than 10 years.
Treatment of amyotrophic lateral sclerosis
Treatment of amyotrophic lateral sclerosis occurs with the help of the drug Riluzole (Rilutek), which is the only remedy, a little improved and slows the progression of the disease. It prolongs survival by several months, and in the longer term extend the lives of patients with the initial stage of amyotrophic lateral sclerosis. It also extends the time before a person will need ventilatory support.
Other methods of treatment of amyotrophic lateral sclerosis are aimed at relieving symptoms and improving the quality of life of patients. This maintenance treatment, which is best provided multidisciplinary doctors and nurses capable of supporting mobile and for patients comfortable.
Health care providers can prescribe medication to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. These drugs also help patients reduce pain, depression, sleep disorders, dysphagia and constipation. Baclofen and diazepam are often prescribed to control the spasticity caused by amyotrophic lateral sclerosis, and trihexyphenidyl or amitriptyline may be prescribed when patients with amyotrophic lateral sclerosis have problems with swallowing saliva.