Lou Gehrig's Disease (ALS)—Fear, Symptoms, and Statistics

The motor neuron disease, Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig's disease, is a devastating illness. ALS is one of the major neurodegenerative diseases alongside Parkinson's disease and Alzheimer's disease.

Lou Gehrig's disease is either sporadic or genetic. Ninety to ninety-five percent of those diagnosed with Lou Gehrig's disease have the sporadic form, while 5-10 % of all incidences have a record of the disease in their families.

Clinical features of Lou Gehrig's disease include the most common symptom, muscle atrophy, which is a major symptom of the disease. The disorder involves deterioration of the motor systems on all levels.

Fear of Lou Gehrig's Disease

Understandably, people tend to freak out when they get the idea that the symptoms they are experiencing might be a prelude to Lou Gehrig's disease, but often the symptoms are caused by something else.

On a weekly basis, any neurologist will see patients that are afraid they have Lou Gehrig's disease. In most cases, they suffer from something harmless or are stressed or hypersensitive. The fear of having such a crippling disease can add to the patient's stress levels, causing distress and preventing people from leading healthy lives.

In fact, at any given time, there are probably more people who fear they have ALS than people that actually have ALS.

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

                                                                                   


The list of common symptoms that can be seen everywhere is repeated here but is covered more thoroughly on the symptoms page.

Diseases and Conditions With Similar Symptoms to ALS (BFS)

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

Other, more common conditions (e.g., stress) can also cause symptoms resembling those seen from neurodegenerative diseases. The most common symptoms of stress leading people to think of Lou Gehrig's disease and Parkinson's are tremors and cramps. Patients often mistake cramps with fasciculations. Fasciculations are tiny movements of muscle beneath the skin, while cramps are coarser.

Statistics

Statistics are a useful tool for describing the chance of something. An example is the postulate that the average age for people being diagnosed with Lou Gehrig's disease is 65, and the standard deviation is nine years. The average age is easy—the average age of people being diagnosed with Lou Gehrig's disease is 65 and (using the standard deviation):

  • A tiny fraction, 0.03 %, of those diagnosed with Lou Gehrig's disease are younger than 38 or older than 92 at the time of diagnosis.
  • About 5 % will be younger than 47 or older than 83 at the time of diagnosis.

In other words, the chance of getting Lou Gehrig's disease before the age of 38 is rare. The chance of being stressed or something else leading one to think about Lou Gehrig's disease or a similar diseases before the age of 38 is of course much higher. In fact, people in their thirties are often very busy with jobs, etc., compared to both older and younger people.

Unfortunately, the distribution of Lou Gehrig's disease incidences within age groups is not entirely normally distributed, so the number of people getting ALS in their thirties may be higher.

It is difficult (almost impossible) to say how old people are, on average, if they are diagnosed with ALS. The reason for this is because the data 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 the onset of ALS was 64.2 and 67.8 years for men and women, respectively1. In a study from Serbia (Yugoslavia) from 1985-1991, the average age of onset of Lou Gehrig's disease was 56.2 years with a standard deviation of 9.8 years2. In an Italian study 3, the average age at onset of Lou Gehrig's disease was 61.3 ± 10.2 years; that study included associated risks of exposure to lead. Exposure to heavy metals and the incidence rate of Lou Gehrig's disease is also something which has been studied extensively in Japan. In a Norwegian study4 the average of Lou Gehrig's disease onset was 60.9 years—no standard deviation was given.

Before you read the section about Lou Gehrig's disease statistics and real numbers about incidence and prevalence, please consider this if you are younger than 40 years old:

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

Now, these numbers are taken out of the thin air. The real numbers surrounding the actual number of people getting Lou Gehrig's disease and the risk of getting Lou Gehrig's disease must be found in peer reviewed journals. I have compiled the results of some of the studies that have led to publication in these types of journals. These results can be found in the section about statistics.

Treatment

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

Sometimes Lou Gehrig's disease patients who are diagnosed with the disease can suddenly go into remission after the diagnosis.

Only one drug (riluzole) has so far been used in order to directly prolong the lifespan of patients with amyotrophic lateral sclerosis (Lou Gehrig's disease). Riluzole increases the lifespan of patients by an average of three months. The drug still needs further investigation, but preliminary results are positive.

Improving the Quality of Life of Lou Gehrig's Disease Patients

Most of the treatment around Lou Gehrig's disease patients revolves around relieving symptoms and improving the quality of life.

Two-thirds of patients with Lou Gehrig's disease complain about pain normally caused by muscle cramps, fasciculations, and spastic movements of limbs. Medicines, such as quinine sulphate, carbamazepine, and phenytoin are used to treat cramps and fasciculations.

Spastic movements of limbs can be reduced by baclofen, tizanidine, and tetrazepam.

ALS Research

Scientists have been trying for years to find a cure for Lou Gehrig's disease or new ways to delay the progression of the disease—besides symptomatic treatment, of course.

Below you'll find small pieces of information about the latest research. This will be updated regularly.

Antioxidants Have No Effect on the Progression of ALS

In a meta analysis study, it was investigated if antioxidants could prolong the lifespan of ALS patients. It was shown that antioxidants had no effect on the lifespan of ALS patients5.

Lithium May Delay Progression of ALS

In a study by Fornai, et al. (2008), it was shown that lithium can delay the progression of amyotrophic lateral sclerosis. Lithium was shown to induce neuronogenesis (genesis of new neurons) and remove mitochondria altered by the disease. The effect of lithium was a slower progression of the disease.

Details about this study can be found in Autophagy 4(4) 2008 pp. 527-535

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 Lou Gehrig's disease patients.

Symptoms of Lou Gehrig's Disease

The most common symptom of Lou Gehrig's disease (ALS) is muscle weakness or muscle atrophy. Muscle atrophy is a decrease in the size of the muscles, making them significantly weaker.

When people are seeking a neurologist and they mention Lou Gehrig's disease before the consultation, muscle weakness is the first thing the neurologist will look for as to exclude Lou Gehrig's disease immediately. A trained neurologist will see Lou Gehrig's disease almost instantaneously.

It is very rare that people are diagnosed with Lou Gehrig's disease without having muscle weakness, a reason why weakness and atrophy should be in capital letters and in bold in any list of Lou Gehrig's disease symptoms (see below).

If you are on this webpage because you fear that you have ALS, but you do not have any weakness or any atrophy in any of your limbs and are younger than 40 years old, you most likely do not have Lou Gehrig's disease! However, the symptoms you feel may be due to something else, so the best advice is to go and see a doctor.

There may be other symptoms not listed here, but those listed are definitely the most common symptoms. The symptoms are: cramps in muscles, spasticity in limbs, muscle weakness and atrophy, emotional sensitivity and instability, dysphagia, dyspnoea, and dysarthria.

The list contains some words that might need further explanation:

Dysphagia refers to difficulty swallowing. In bulbar onset of Lou Gehrig's disease, this can happen at an early stage. Swallowing difficulties are, however, also a common complaint from older people. The incidence of dysphagia among the elderly is relatively high.

Dyspnoea is difficulties in breathing caused by pain. Dyspnoea is the same as shortness of breath, which is a common symptom of many disorders. Those that are suffering from dyspnoea should seek medical treatment.

Dysarthria is poor articulation of words caused by the dysfunction of one or many of the subsystems involved in speech, such as the tongue, jaw, or respiration. Often dysarthria only occurs later on in the development of Lou Gehrig's disease.

Statistics About Lou Gehrig's Disease

Ethnicity is an important factor when analyzing statistics on Lou Gehrig's disease (ALS) data because some studies suggest that ethnic background may modify predisposition to Lou Gehrig's disease. However, it is widely accepted that the incidence of Lou Gehrig's disease is uniform across populations of Caucasian origin or most people in Western Europe and North America (USA and Canada). The same is the case with other motor neurodegenerative diseases.

Lou Gehrig's Disease Incidence—Per Year Per 100,000

Most studies on Caucasian populations have shown an incidence of Lou Gehrig's disease above 1 per 100,000 person years. In other words, each year there is on average a 1:100,000 chance of getting Lou Gehrig's disease. The highest reported incidence in a Caucasian population is 2.4 per 100,000 and the lowest incidence is 0.6 per 100,000. These numbers are, however, averaged numbers for whole populations and from birth to death of individuals.

According to studies based on other ethnic populations, the incidence of Lou Gehrig's disease is lower among non-Caucasian populations. This can, however, be disputed as the criteria of diagnosis and the frequency of which Lou Gehrig's disease cases are reported may be different in some of those countries.

It is more relevant to look at incidence rates for those age groups affected by Lou Gehrig's disease and not just total populations.

Secondly, meaningful comparisons between studies (meta-analysis) can only be made if the data are somehow adjusted for age and sex and compared to a population standardized to meet certain criteria. This was done in a study in Neurology by Cronin et al. (2007).

Here, all of the data were adjusted to fit into an age group from 45 years to 74 years. This seems reasonable as the vast majority of people diagnosed with Lou Gehrig's disease fall under this age range.

The study showed incidence rates of Lou Gehrig's disease in some of the world's largest countries (table 1).

Table 1. Incidence of Lou Gehrig's disease in different parts of the world in the age group 45-74 years

Country / Region Male Female Overall 5% CI
United States (WA) 5.3 4.9 5.1 4.3 - 6.1
United States (MN) 7.1 6.0 6.6 4.5 - 9.6
United States (TX) 3.4 3.2 3.3 2.7 - 4.0
Canada 6.7 4.2 5.3 3.3 - 8.1
Japan 3.4 3.2 3.3 2.7 - 4.0
Greece 3.1 1.8 2.4 1.5 - 3.7
Denmark 5.0 3.6 4.2 3.1 - 5.7
Finland 7.4 8.9 8.2 5.0 - 12.7

Note CI is confidence interval. Data from Cronin, et al. (2007)

Lou Gehrig's disease prevalence (the no. of people living with ALS per 100,000)

As a rule of thumb, only 50% of those diagnosed with amyotrophic lateral sclerosis survive for more than three years. The life-expectancy following diagnosis depends on a number of factors, such as age and physical condition. Age is perhaps the most important factor of the two, due to younger individuals generally being in better health and ability to fight disease for a longer time compared to the elderly.

If the prevalence is compared with the incidence, the higher ratio of prevalence to incidence (prevalence:incidence), the longer period of time people are living with the condition.

In other words: prevalence = incidence – average duration.

The study by Cronin, et al. (2007)6 did not consider the prevalence of ALS. It did, however, consider the mortality from Lou Gehrig's disease—that is, the incidence of death from Lou Gehrig's disease. Theoretically speaking, the mortality should be close to the incidence.

Local differences in the way deaths are reported make comparisons between mortality and incidence difficult because death reports vary among countries. In some cases, the cause of death is registered as something else than Lou Gehrig's disease—even though the condition was caused by Lou Gehrig's disease.

Regardless, data was analyzed, and it showed that the overall mortality from Lou Gehrig's disease in the United States is 1 per 100,000 a year. In Japan, it is 0.7 per 100,000; in Denmark, 0.9; and in Canada, 1.5. In all European countries the mortality was in the range of 0.7 to 1.5 per 100,000.

Web References About Lou Gehrig's Disease Statistics

It is difficult to find pages about Lou Gehrig's statistics, but here are a few.

Statistics at Wrongdiagnosis
MND alliance resource about ALS statistics (pdf)
ALS MND support forum - includes some statistics Symptoms as described by the ALS Association
ALS symptoms described at WebMD
Mayoclinic - overview of symptoms
Forum about ALS symptoms The ALS association
ALS Hope Foundation
NINDS - a governmental institution

References

1. Traynor B.J. 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 O.B. et al. EPIDEMIOLOGY OF AMYOTROPHIC-LATERAL-SCLEROSIS IN HORDALAND COUNTY, WESTERN NORWAY. ACTA NEUROLOGICA SCANDINAVICA 83(5) pp. 280-285, 1991
5. Orrell RW et al. A systematic review of antioxidant treatment for amyotrophic lateral sclerosis/motor neuron disease, Amyotrophic Lateral Sclerosis 9(4) pp 195-211 (2008) 6. Cronin S., Hardiman O., Traynor B.J, Ethnic variation in the incidence of ALS - a systematic review, Neurology 68, pp. 1002-1007, 2007


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