Researchers with the Centers for Disease Control and Prevention (CDC) says people with Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig’s disease, a deadly neurological condition is projected to increase at least 10 % by the year 2030 with the largest rise in people 66 years or older.
What is ALS or Lou Gehrig’s Disease?
ALS or Lou Gehrig’s disease is a pure motor (movement) neurological condition that slowly but relentlessly weakens the voluntary muscles of the body including the legs, arms, trunk, neck and chest wall. It destroys the nerves in the spinal cord (anterior horn cells) and brain that feeds into the muscles. This leads to severe disability including the ability to walk, swallow and speak and eventually causes death.
The term Amyotrophic Lateral Sclerosis (ALS) is Greek in origin with Amyotrophic meaning “no muscle nourishment,” Lateral or portion of the spinal cord, and Sclerosis or scarring of the nerve. In the United Kingdom it’s called Motor Neuron Disease (MND). Why it happens is unclear as the majority of cases are sporadic except for those with a family history.
It’s also called Lou Gehrig’s disease, an eponym or named after the famous Yankee baseball player who was diagnosed at age 36 with ALS at the Mayo Clinic. He held the record for the number of grand slams, and consecutive games played, and his fans called him the “Iron Man.” His first signs of a neurological condition was the loss of muscle strength, and coordination forcing him to give up baseball.
In July of 1939, over 62,000 fans gathered in Yankee Stadium for one of the most memorable farewell speeches. Gehrig said, “Today, I consider myself the luckiest man on the face of the earth. I have been in the ballpark for seventeen years and have never received anything but kindness and encouragement from you fans.”
What are the signs of ALS or Lou Gehrig’s Disease?
The hallmarks signs of ALS are muscle weakness and wasting as the nerve signals to muscles are damaged. This interrupts fine motor skills in the early stages like difficulty picking up an object or writing, foot and ankle weakness, tripping, or a foot drop. It may happen to any part of the body but most commonly starts in one arm or leg.
Symptoms may include difficulty getting up from a chair, climbing stairs, pushing off from the floor, and brushing your hair. Muscle cramping and stiffness of the legs and core or trunk increases the risk of falls. Swallowing, speech, and muscles of breathing in later stages are also affected.
One of the first signs of ALS may be the famous “split hand sign,” where the muscles near the base of thumb are weaken or atrophied (wasting away) and loose the pincer grip where the thumb and index finger work together to pick up small objects. This can be seen in up to 70 % of cases although not specific to ALS. Why this happens is not clear but can be helpful in the diagnosis especially in early stages.
It’s a progressive disease. For example, if it starts in one arm it generally progresses to the opposite arm before moving on to other body muscles. The rate of progression varies with slower progression in younger people and those who present with limb onset first. The outcomes or rate of progression is faster if targets the muscles of swallowing and breathing first.
Interestingly, half the people with ALS experience symptoms that are not purely motor, or movement related including personality and behavioral changes including apathy, disinhibition or acting out, and cognitive or mental functioning especially in advanced stages. 15 % of people with ALS may also have frontotemporal dementia (FTD), a rare disease that effects personality and behaviors.
The diagnosis of ALS requires a complete neurological evaluation by a specialist or Neurologist, lab testing, brain imaging, and nerve testing or EMG (electromyogram) that measures the electrical activity of the muscles and nerves.
How long do people live with ALS?
ALS is a progressive neurological disease that eventually results in death. There is no cure however survival time differs among people but on average from onset of symptoms is 3 to 5 years with 10 % living 10 years or more. It usually targets people between 50 to 70 years of age but sometimes younger.
Common cause of death is progressive neuromuscular respiratory or breathing failure where the muscle of the diaphragm or chest wall are so weakened that people are not able to breath on their own and may require a breathing machine.
There are some treatments to manage symptoms and medications that can prolong survival and reduce the risk of progression by a few months and even longer but there is no cure. There are also medications and therapies to help with symptoms like painful muscle spasms and cramps.
Who gets ALS and what are the risk factors?
Most cases of ALS or Lou Gehrig’s disease are sporadic or random and 10 % have a family history. It’s a relatively rare disease that tends to effect older people between age 60 to 75 years of age and is more common in men. Some sources say the average lifetime chances of getting ALS is 1:350 for men and 1:400 for women.
Other risk factors include old age, family history, and cigarette smoking. The reason why people get ALS is complex and not well understood but likely is from a combination of genes and environmental triggers. In fact, over 120 genes have been linked to the disease.
The ALS Association wants us to remember to put risk factors into context. Increased risk for a rare disease that impacts 30,000 people every year in the U.S. and an estimated 36,000 in 2030 places most of us at a less than 1 % lifetime risk of getting it unless we have a family history of ALS. So, when studies tell us a certain lifestyle risk factor may be “linked,” it impacts only a few extra people.
Outside of cigarette smoking risks factors include military services especially if you served in the Gulf War or another war (twice the risk than if you did not serve), exposure to toxins including heavy metals like lead and mercury, and brain and spinal cord injuries like severe head injuries seen in sports like football or soccer.
Any final advice on ALS or Lou Gehrig’s disease?
ALS or Lou Gehrig’s disease per the CDC is on the rise globally projected to be up 69 % by 2040 from earlier years and estimated in the U.S. to rise at least 10 % with a 25 % increase in people 66 years of age or older. The reasons for this are complex due to increased awareness, growth of our older segment of the population, and improvement in medical care. Unfortunately, despite advances in medications and therapeutics ALS has no cure and remains a progressive disease that is fatal.
It still despite the rise in numbers remains a rare disease worldwide with Lou Gehrig who put ALS on the map, famous scientist Stephen Hawkins, and more recently with the announcement by Eric Dane in Euphoria and Grey’s Anatomy diagnosed with ALS at the age of 52.
If you or a loved one is experiencing signs and symptoms including motor weakness, cramping, or fasciculations (repetitive muscle twitching) please contact your doctor as early discovery of ALS is important for management of symptoms, to delay progression of this deadly disease, and to establish a good support system.
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