Amyotrophic Lateral Sclerosis (ALS)

The motor neuron disease Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig's disease, is a devastating illness for those suffering from the disease and their relatives. ALS is one of the major neurodegenerative diseases alongside Parkinsons disease and Alzheimers disease.

ALS is either sporadic or familial. 90-95 % of those diagnosed with ALS have the sporadic form while 5-10 % of all incidences have a record of ALS in their families.

Clinical features of Amyotrophic Lateral Sclerosis include the most common muscle atrophy which is a major symptom of the disease. In general the disorder involves deterioration of the motor systems on all levels.

Symptoms of Amyotrohpic Lateral Sclerosis

There are a lot of sites on the internet about ALS. Most of them offer the same list of symptoms characterizing amyotrophic lateral sclerosis or ALS.

The list of common symptoms that can be seen everywhere is repeated here:

. muscle atrophy and muscle weakness
. fasciculations and cramping of muscles
. swallowing impairment

Note 1: Muscle atrophy is the major symptom
Note 2: The section about symptoms will go into detail about symptoms

The symptoms above do not necessarily indicate ALS. They do however need to be investigated by a trained neurologist.

The symptoms above describe symptoms of ALS patients, it is just very general symptoms that can arise for a number of reasons - and people experiencing some of these symptoms, either permanently or from time to time, do definitely not need to have ALS.

Diseases and conditions with somehow similar symptoms

Benign Fasciculation Syndrome (BFS), a much more common and in contrast to ALS a benign disorder, shows to some extent the same kind of symptoms. BFS is completely harmless and most often have a duration of a couple of years with great variabilty. The symptoms above can also be caused by other diseases more serious than BFS, but still not ALS. In the section about symptoms a more detailed description of the symptoms from ALS can be found

Other and more normal conditions, e.g. stress, can also causes symptoms resembling those seen from neurodegenerative diseases. The most common symptoms of stress leading people to think of ALS and Parkinsons are tremors and cramps. Patients often mistake cramps with fasciculations. Fasciculations are tiny movements of muscle beneath the skin while cramps are more coarse.

Fear of ALS

Understandably, people tend to freak out when they get the notion that their experienced symptoms might be a prelude to ALS, but often the symptoms are caused by something else.

What is generally less known is that fear of ALS caused by lack of information is much more common "disorder" than ALS itself. On a weekly basis any neurologist will see patients that are afraid they have ALS. In most cases they suffer from something harmless or are stressed or hypersensitive. Fear of something very scary (e.g. a scary disease) may be very disabilitating to people. Fear of something that is not there may cause dysfunction of all sorts of mental functions and may prevent people from living a good life.

On this website I will elaborate a bit about ALS and hopefully provide some decent information about ALS symptoms, ALS statistics and ALS treatment.

Statistics

Statistics is a useful tool for describing the chance of something. An example is the postulate that: the average age for people being diagnosed with ALS is 65 and the standard deviation is 9 years. The average age is easy - the average age of people being diagnosed with ALS is 65 and (using the standard deviation):

In other words the chance of getting ALS before the age of 38 is neglegible. The chance of getting stress or something else letting one to think about ALS or similar diseases before the age of 38 is of course much larger.

Unfortunately the distribution of ALS within age groups is not entirely normal distributed, so the number of peolpe getting ALS in their thirties may be bit higher.

It is difficult (almost impossible) to say how old people are, on average, if they are diagnosed with ALS. It varies from study to study and the methods of diagnosis are different from country to country. However, in a study from Ireland from 1995-1997, the mean age of onset of ALS was 64.2 and 67.8 years for men and women, respectively1. In a study from Serbia (Jugoslavia) 1985-1991, the average age of onset of ALS was 56.2 years with a standard deviation of 9.8 years2. In an italian study 3, the average age at onset of ALS was 61.3 +/- 10.2 years; that study included associated risks of exposure to lead. Exposure to heavy metals and the incidence rate of ALS is also something which have been studied extensively in Japan. In a norwegian study4 the average of ALS onset was 60.9 years - no standard deviation was given.

Before you read the section about ALS statistics and real numbers about incidence and prevalence please consider this if you a younger than 40 years old:

If the incidence of ALS is 2/100000, 2 in every 100,000 people are diagnosed with ALS each year. Comparing this number to the age distribution numbers shortly outlined will tell us that the chance of getting ALS any particular year, when you are younger than 38, is about 1:165,000,000.

Now, these numbers are taken out of the blue air. The real numbers about the incidence of ALS and the risk of getting ALS must be found in peer reviewed journals. I have compiled the results of some of the studies that has lead to publication in these types of journals. This can be found in the section about statistics.

Treatment

There is no cure for ALS. Only life prolonging (palliative) treatment. In the section about treatment some of the methods to improve life-quality of ALS patients are discussed.

Sometimes ALS goes into remission and some ALS patients experience to be diagnosed with ALS and then suddenly the ALS goes into remission. An Australian guy has made a webpage about his ALS remission experience.

Videos

A lot of videos about the disease can be found on youtube. Some of those videos are very informative and excellent for both sufferers and people related to ALS patients.

References

1. Traynor BJ et al. Incidence and prevalence of ALS in Ireland, 1995-1997 - A population-based study. Neurology 52(3) pp. 504-509, 1999
2. Alcaz S et al. Epidemiological and clinical characteristics of ALS in Belgrade, Yugoslavia. ACTA NEUROLOGICA SCANDINAVICA 94(4) pp.264-268, 1996
3. Guidetti D et al. Epidemiological survey of amyotrophic lateral sclerosis in the province of Reggio Emilia, Italy: Influence of environmental exposure to lead. NEUROEPIDEMIOLOGY 15(6) pp 301-312, 1996
4. Tysnes OB et al. EPIDEMIOLOGY OF AMYOTROPHIC-LATERAL-SCLEROSIS IN HORDALAND COUNTY, WESTERN NORWAY. ACTA NEUROLOGICA SCANDINAVICA 83(5) pp. 280-285, 1991

Web resources

The ALS association
About ALS at Wikipedia
ALS Hope Foundation
NINDS - a governmental institution