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Lou Gehrig's Disease

What is ALS(Lou Gehrig's disease)?

ALS means a disease that motor nerve cells which move skeletal muscles of the body, are dead gradually and paralysis of skeletal muscle of the entire body will be progressed. Motor nerve cells are divided in to upper motor nerve cells and lower nerve cells. ALS is a disease that affects both of these types of motor nerve cells. The reason why motor nerve cells are damaged or destroyed is not well known yet. ALS is a typical disease among the degenerative diseases of the nervous system, together with Alzheimer's disease, and Parkinson's disease.

Clinical symptoms of ALS are as follows: motor nerve cells in the brain and spinal cord are damaged. The strength of the arms and legs are getting weaker. Muscle also moves itself, regardless of their willingness. Muscles are getting dried. When motor nerve cells in the brain stem, where the medulla is located, are affected, phenomenon of being slows of speech, hard to swallow food and contracted tongue, and a symptom of difficult breathing can be appeared.

These symptoms are appeared throughout the years from several months after onset of disease. After all, the disease is progressed even in statuses of which a patient cannot swallow food so that he or she has to eat with other's help through a gastrostomy and a patient can breathe with depending on a respirator.

In other words, ALS is a disease characterized by main symptoms such as mu scle atrophy and paralysis, dysarthria and dysphagia and spasticity symptoms. Cognitive disorder also appears about 50% or more of ALS patients and its rare cases also are occurred in frontotemporal dementia, and show that personality disorder or memory disorder.

ALS causes

As with other degenerative diseases, pathogenesis of ALS is not yet clearly revealed. However, the following several hypotheses have been raised and mainly discussed so far: hereditary, excitotoxic, oxidative toxicity, protein aggregation, immune mechanisms, infections, functional abnormality of nerve micro-fibers, mitochondrial dysfunction, lack of nerve growth factor, hormonal dysfunction and environmental factors.

These are estimated to overlap mechanisms and then, to interact with each other, rather than act independently of each other. Familial ALS acounts for 5-10% of the total ALS patients. It causes turned out to be Cu/Zn-superoxide dismutase (SOD1) gene and ALS2 gene mutations. So far, all nine genes have been identified as genes relating the Familial ALS.

ALS symptoms

The symptoms of ALS are different for each patient depending on the region where the disease begins, but similar that it is begins with a symptom that one´s strength is gone. The central nervous system is divided based on motor nervous system; (1) cerebral and pars intercerebralis (2) cervical rad (3) pleural effusion (4) lumbar segment and sacral. It shows the following symptoms depending invaded parts.

  • Cerebral and pars intercerebralis (medulla oblongata)

    pronunciation disorder, swallowing disorder, respiratory failure, etc.

  • Cervical rad(arms)

    spoon and chopstick handling disorder, weakening of physical strength, writing disabilities dressing disorder, etc.

  • Pleural effusion

    there are little observable symptoms, except forhardly stand straight.

  • Lumbar segment and sacral(legs)

    walking disorder, climbing stairs disorder, etc.

In addition, it commonly shows crackling muscle symptom, muscle rigidity(becoming stiffness) and muscle atrophy (muscle is stuck). The progression of ALS varies from individuals. However, it can be expressed as brainstem symptoms (pronunciation disorder, etc.) or weakening strength of the upper limbs or pelvic limb and is spread in the body over time, finally, resulting in respiratory paralysis.

ALS diagnosis

ALS diagnosis is find characteristic symptoms through medical history and neurological examination. Because there are a variety of disorders that may exhibit similar symptoms of ALS, it is necessary to make electromyography, blood test, radiography, genetic test, etc. to find ALS. In other words, to confirm the diagnosis of ALS, the following tests shall be conducted;
In other words, go through the procedure to check a disease that may show similar symptoms to those of ALS such as connective tissu diseases, proliferative lymphocyte diseases, tumor section syndrome, heavy metal poisoning, metabolic disorders, or infectious diseases through blood tests. If it is difficult to differentiate a disease with others sometimes, nerve or muscle biopsy may be required. If it is difficult to confirm a disease in an early stage, neurological examination and electromyography are also conducted at least every six months.

  1. STEP01

    Check if any part of motor nerve cells is damaged through medical history and neurological examination, and if there are other neurologic disorders (dementia, parakinesia and balance disorder etc.) accompanied

  2. STEP02

    Go through the procedure to check if which parts were damaged to some extent, and perhaps, if other diseases were causing similar symptoms like this by conducting root thorn test and nerve conduction test on clinically suspected sites through electromyography.

  3. STEP03

    Check if there are structural abnormalities in the brain and spinal cord through a variety of radiography tests including MRI and make sure if a disease may show similar symptoms to those of ALS.

  4. STEP04

    Find the cause of ALS, and diseases that cause similar symptoms of ALS through genetic testing and blood tests.

Prevalence
Rate World US Europe Japan Korea
Incidence 1.5/100,000 120,000 5,960 4,988 3,000 960
Prevalence 5/100,000 350,000 17,500 13,500 9,000 2,500
Rate World
Incidence 1.5/100,000 120,000
Prevalence 5/100,000 350,000
US Europe
Incidence 5,960 4,988
Prevalence 17,500 13,500
Japan Korea
Incidence 3,000 960
Prevalence 9,000 2,500

The annual incidence rate is 1 in 100,000 per year and prevalence rate is 4-6 in 100,000 per year, which have increased with increase in aging population.

Also, as part of the Centers for Disease Control and Prevention Morbidity and Mortality Weekly Report, the estimated number of ALS patients in 2013 in the United States (US) was 15,908, which equates to a current prevalence of 16,415 ALS patients currently in the US. ALS is classified as either sporadic ALS (SALS) or familial ALS (FALS). 
Approximately 90% of ALS patients are sporadic, with men having a higher incidence than women; the remaining 10% of patients have FALS, which has a similar incidence between men and women. The overall population-based lifetime risk of ALS is 1:400 for women and 1:350 for men. Peak age at onset is 58–63 years for SALS and 47–52 years for FALS. Incidence decreases rapidly after 80 years of age