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What is Lou Gehrig's disease?
Lou Gehrig's disease is a degenerative disease of the nervous system, and it is called motor neuron disease (MND). Motor neuron disease refers to a disease in which motor neurons located in the spinal cord, brain stem, and cerebral cortex of the nervous system show degenerative changes due to some cause. It is a systemic disease that progresses slowly and involves both the upper nerve (UMN)* and the lower nerve (LMN)*.
*Superior neurons: Motor neurons with cell bodies in the cerebral cortex, which are responsible for issuing motor commands.
*Subneuron: Motor neurons with cell bodies in the brain stem or spinal cord that transmit signals directly to muscles.
In official Korean terminology, it is called 'amyotrophic lateral sclerosis', which means that it is a disease in which muscles lose and the edges of the spinal cord become hard.
The exact cause of its occurrence is not yet known, but the main pathophysiological characteristic is that the sensation is still felt, but only the motor nerves change.
According to epidemiological surveys in Korea, it occurs in 1.2 out of 100,000 people per year, and the age of onset of symptoms is 60.4 years. The incidence ratio in males and females is 1.6 males to 1 female, and the prevalence rate is 3.44 per 100,000 people. Lou Gehrig's disease accounts for 50% of all motor neuron diseases, and it is characterized by a generally poor prognosis.
The public policy within the ALS management system in Korea is the Rare Disease Management Act, which provides multidisciplinary treatment, but there are limitations such as non-reimbursed treatments such as radical treatments, and there is a lack of caregivers and specialized medical facilities.
What are the symptoms of ALS?
Lou Gehrig's disease causes a variety of symptoms. Characteristically, a symptom appears throughout the body. Muscles dry and tremble, and the body becomes weak and stiff. In addition, you may experience frequent sore or slurred speech, all of which can be difficult to notice at first unless you are sensitive, as all of these symptoms progress slowly.
How is Lou Gehrig's disease diagnosed?
Just because there is only a symptom of muscle bouncing does not mean that it is ALS, and on the other hand, there is ALS in which there are no symptoms but only muscle abnormalities. Lou Gehrig's disease is diagnosed when there is an abnormality in both the upper and lower nerve sources.
The diagnostic criteria for ALS consist of four stages.
Phase 1, medical history, neurological examination
The patient's medical history will be examined. Symptoms of ALS can start on the tongue, mouth, and arms, and the onset and speed may vary from patient to patient.
Step 2, electromyography
Electromyography will be performed. Electromyography is a test in which a needle is pricked into a muscle to observe the pattern of the muscle.
Step 3, Imaging Examination
An MRI will be taken.
Step 4, all other checks
Blood tests, cancer tests, cerebrospinal fluid tests, and cognitive function tests are performed.
Stages 3 and 4 of all are tests that are performed to identify other diseases other than ALS. Although there are symptoms of ALS, there are many different cousins that must be differentiated from ALS, so the above tests must be performed to accurately diagnose ALS.
If I am diagnosed with ALS, what are the 3 steps I should take?
Step 1
In the early stages of ALS symptoms, it is necessary to assess the situation and life, and accept the disease.
Step 2
The symptoms are already advanced. You can get a disability rating, but we recommend getting it when you need it. The most important thing at this stage is to maintain weight.
Step 3
The symptoms are somewhat advanced. Determine whether to wear a brace, gastrostomy*, or respirator.
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