Amyotrophic Lateral Sclerosis is also known as Lou Gehrig’s Disease or Charcot Disease. It is a progressive, neurodegenerative disorder, which affects motor neurons in the brain and the spinal cord. (Amyotrophic Lateral Sclerosis, 2015). The translation verbatim from Greek Amyotrophic means no muscle nourishment. The word Lateral indicates the affected areas in the dorsal cord.
The main reason, which causes the disease, is the death of motor neurons. The muscle movements of the human body are initiated by the brain, which sends impulses to the concrete muscles through the dorsal cord, by means of the nerve cells called motor neurons. The ALS leads to the death or degeneration of the motor neurons, located in the brain, and those that are located in the spinal cord, which in its turn reduces the brains ability to initiate and control a muscle movement. Without functioning, the muscles languish and waste.
This disease causes a huge amount of serious disabilities. Suffering from ALS, a person loses the ability to move his/her limbs, as practically all the muscles under voluntary control are paralyzed. It is considered, that most patients with the ALS effect do not lose the ability to see, to hear, to smell, moreover this disease usually does not influence a persons mental abilities.
The earliest symptoms of the disease are cramps, muscle weakness, motor dysfunction, nasal speech, difficulties in chewing. These primary symptoms then may develop into atrophy.
Depending on which muscles are affected, may be distinguished the so-called limb onset, when the symptoms begin in arms and legs, and the bulbar onset, when the obvious problems with speech are observed.
In the course of the disease, muscle degeneration is observed all over the body, regardless of the primary affected part. Some patients may have some memory problems. In the last stage of the malady, the respiratory apparatus is affected, so a person has problems with breathing. Practically, the patient loses the ability to breathe and becomes completely dependent upon assisted respiration.
ALS is very difficult to detect. Now there is no precise test to definitely establish the ALS diagnosis. The primordial diagnosis is based on primary symptoms and complete examination of the full patients medical history. For the precise diagnosis the following procedures as electromyography (EMG), nerve conduction study (NCS), magnetic resonance imaging (MRI), muscle biopsy, blood and urine studies are conducted.
Neurology specialist Richard Rison as a case study gives the example of a 78-year-old Caucasian. He was presented to the clinic complaining of weakness in the arm and shoulder. Rison (2015) pointed out that the patient had attributed his issues to a prior rotator cuff injury, but in a course of time, he had noticed a progressive shrinking in the muscles of his left arm and hand with decreased grip strength (para.5). When the patient was examined by means of the neurological examination and electrodiagnostic, fasciculations were observed in his arm, but there were no problems with his bulbar region or lower limbs. His electromyography and magnetic resonance imaging were clear, and the results of his hematologic study were negative for any abnormalities. The established diagnosis was flail arm syndrome because in the lower limbs of the patient there were no any symptoms. In several months, the patient felt weakness in his legs and there were problems with his speech and swallowing. The further diagnosis was ALS.
ALS is an incurable fatal malady, which is very difficult to diagnose. Bradley, Daroff, Fenichl and Jankovic (2004) state that “death, usually from respiratory compromise, occurs approximately three years after onset of symptoms” (p.15). The only awareness of the fact, that this disease is fatal may cause serious depression of the patient. Though there is no treatment, the progression of this disease may be diminished. Todays contemporary technologies help the scientists to learn more about this disease, but still there is a lot of unknown about ALS.
Reference List
Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. (2015). Web.
Bradley, W., Daroff, R., Fenichl, G., & Jankovic, J. (2004). Neurology in Clinical Practice: The Neurological Disorders. Volume II. Philadelphia, US: Butterworth Heinemann.
Rison, R. (2015). Amyotrophic lateral sclerosis-motor neuron disease, monoclonalgammopathy, hyperparathyroidism, and B12 deficiency: case report and review of the literature. Web.