FAQ - Frequently Asked Questions About ALS
Amyotrophic lateral sclerosis (ALS), is a neurodegenerative disease which results in progressive loss of motor neurons that control voluntary muscles. Leading to gradual muscle weakness and loss of muscle control. There are many different types of ALS, however they all share the progressive loss of voluntary muscle control. Continue reading to learn more about ALS.
What is ALS?
ALS, Amyotrophic lateral sclerosis, is a neurodegenerative disease where neurons progressively deteriorate and die leading to the inability to voluntarily control muscles. The individual will gradually lose the ability to move, talk and eventually, breathe.
There are several different types of ALS, and the term is commonly used for the group of neurological disorders which all affect motor neurons.
How does ALS affect the body?
Neurons in the brain, brainstem and spinal cord progressively die and without them the skeletal muscle stops working.
The muscles, now lacking the impulses normally received by the neurons will eventually start to deteriorate leading to the gradual decrease of muscle strength and muscle atrophy (amyotrophy). And the neurons will be replaced with scar tissue (lateral sclerosis).
Who gets ALS?
Most patients with ALS receive symptoms when they are 45-75, however since there are several types of ALS the symptoms and the progression of the disease can vary heavily.
Around 10 percent of all ALS cases are classified as familial ALS, meaning the existence of a family history of the disease. However in the remaining 90 percent, which is classified as sporadic ALS, the exact cause is still unknown.
Common denominators for individuals with sporadic ALS have shown to be old age, smoking, relatives with neural diseases and a predominance within males. Studies have also shown that other risk factors are traumatic injuries to the head and heavy physical labor, for example athletes performing contact sports.
Up to the age of 65, twice as many men develop ALS compared to women, however after the age of 70 the difference between the genders disappears.
How many people get ALS disease?
Approximately two to four people per 100 000 inhabitants develop ALS every year and in Sweden about 220 to 250 are diagnosed yearly.
There is nothing that indicates that Sweden is more affected compared to other western countries.
In later years there has been an increase of individuals receiving an ALS diagnosis. This could indicate that more people develop the disease, however, this can also be a result of improved detection methods, redefined criteria for diagnosis and an increased knowledge of the connection between ALS and other diseases.
What symptoms does ALS give?
The first symptoms can occur suddenly but it's more common with a slow but progressive muscle weakness of the tongue, throat, hand, arm or leg. The muscle weakness then spreads to other voluntarily controlled muscles.
The muscle symptoms can vary depending on how quickly the disease develops and the distribution of neuronal damage. It can express itself with varying degrees of muscle stiffness in the affected muscles, muscle spasms, clumsiness, a decline in fine motor skills and muscle twitches.
In the later stages of ALS, the disease can give rise to emotional lability, mild signs of dementia with behavioral and cognitive changes as well as language dysfunction.
What all types of ALS have in common is the weakening of the muscles responsible for normal lung function.
How is ALS diagnosed?
Since there are many diseases which have symptoms resembling ALS it can be hard to make the right diagnosis, especially in the onset of the disease when the symptoms are more vague.
There is no specific test which can be performed inorder to set the diagnosis for ALS, but instead the criteria for ALS is instead to methodically rule out other, similar neural diseases.
Except a neurological medical examination, analyses are performed on blood and spinal fluid, a through MRI scan and neuro physical examinations which measures the function and status of the axons (the thin fiber which extends from the neuron) in arms and legs.
If there is a known hereditary risk a DNA-analysis can confirm the diagnosis.
Why is there no cure for ALS?
There is still no treatment which cures ALS but there are several medications which slows the progression of the disease.
ALS research focuses on understanding how and why ALS occurs and developing a cure which slows and stops the progression of the disease.
Why is research on ALS needed?
Medical science is only partly financed by state funds and most research projects need support from external sources.
ALS, unlike the larger public diseases such as heart disease and cancer, lacks larger external grant givers.
To ensure that the research eventually gives rise to an effective treatment a lot of resources are still required and it is calculated to cost approximately 20 billion SEK to bring forth a new drug against ALS.
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The Börje Salming ALS Foundation contributes to finding a curative treatment for ALS.
