Amyotrophic Lateral Sclerosis (ALS)
What is ALS?
ALS is commonly known as “Lou Gehrig's Disease,” named after the New York Yankees' baseball player who died from it in 1941. The French neurologist Jean-Martin Charcot discovered the disease in 1869.
ALS is a “motor neuron disease,” meaning that the nerve cells (“neurons”) in the spinal cord that attach (innervate) to the muscles throughout the body, begin to degenerate, atrophy, and die. The cause is not known. There have been many theories that have been examined, and ALS has been the subject of multiple research efforts both in this country and abroad, but to date there has been little progress made in finding a cure or even a usefultreatment.
Most patients who develop ALS are usually between 40 and 70 years old. It is estimated that 16,000 to 20,000 Americans have this condition. Military veterans are approximately twice as likely be diagnosed with ALS, perhaps because of exposure to toxins during warfare, but exactly why that is the case is not known.
Symptoms of ALS
As the motor neurons in the spinal cord progressively deteriorate, the voluntary muscles that they innervate begin to atrophy and lose their strength, resulting in muscle weakness, difficulty moving, walking, picking things up, speaking, eating, breathing, and paralysis. Common symptoms include fasciculations (muscle twitches) in the arm, leg, shoulder, or tongue; muscle cramps; tight and stiff muscles (spasticity); muscle weakness affecting an arm, a leg, neck or diaphragm; slurred and nasal speech; and difficulty chewing or swallowing.
When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. When symptoms begin as speech or swallowing problems, it is referred to as “bulbar onset” ALS. Whether ALS starts with a “limb” onset or a “bulbar” onset, it inexorably spreads throughout the body.
Patients with ALS often die from respiratory failure within 3–5 years after diagnosis. However, up to 10% live for 10 years, and the physicist, Stephen Hawkins, lived for 55 years after his diagnosis of ALS.
Types of ALS
1.) Sporadic type. Over 90% of patients who have ALS have the sporadic type. The disease just pops-up, without any family history or other reasons for it. Anyone anywhere can get it.
2.) Familial type. Familial ALS accounts for the other 10% or so of patients who develop ALS, because they inherit a gene mutation that can be passed on from generation to generation at a 50% penetrance rate. What that means is that the familial form of ALS usually requires only one parent to carry the gene responsible for the disease. [Ref: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet]
Diagnosis of ALS
The diagnosis of ALS is typically made by a neurologist. Electromyography (EMG and Nerve Conduction) is useful in helping to confirm the diagnosis of ALS. An MRI scan is useful for ruling-out other diagnoses, but it is not too helpful in diagnosing ALS, because the MRI is usually normal.
Progress has been slow, but there are at least four drugs, Riluzole, Nuedexta, Radicava, and Tiglutik, that may be used to treat ALS.
Riluzole is a neuroprotective drug that blocks glutamatergic neurotransmission in the central nervous system (neurons and brain tissue). The drug is expensive. Without insurance coverage, the cost is about $1,200 a month. Medicare and the Veterans' Administration (VA) program cover it.
Nuedexta is a treatment for “PseudoBulbar Affect” (PBA) disorder, where patients have uncontrollable laughing or crying (often inappropriate) spells. It is also used in ALS and multiple sclerosis. It costs approximately $1,155 per month.
Radicava (edaravone) is administered as an intravenous infusion in the treatment of stroke and ALS. It works as a “free radical scavenger” to eliminate excess free radicals, thereby relieving the effects of oxidative stress to help prevent cell damage. It costs about $1,000 per infusion.
Tiglutik is a liquid oral formulation of riluzole, designed for patients with ALS who are unable to swallow tablets.
Unfortunately, none of these medications is curative. They appear to provide a benefit by slowing the progression of ALS in some patients, but they do not stop the death of neurons from continuing. For example, riluzole prolongs survival by a few months, particularly in the bulbar form of ALS, but does not reverse or stop the damage already done to motor neurons.
New Treatment Options
Potential therapies for ALS are being investigated in a range of disease models. This work involves tests of drug-like compounds, gene therapy approaches, antibodies, and cell-based therapies. For example, NINDS-supported scientists are currently investigating whether lowering levels of the SOD1 enzyme in the brain and spinal cord of individuals with SOD1 gene mutations would slow the rate of disease progression.
Other NINDS scientists are studying the use of glial-restricted progenitor cells (which can develop into other support cells) to slow disease progression and improve respiratory function. Additionally, several exploratory treatments are being tested in people with ALS. Investigators are optimistic that these and other basic, translational, and clinical research studies will eventually lead to new and more effective treatments for ALS. Id.
Another investigational treatment that has been proposed for ALS involves the protein TDP-43, which has been found to accumulate in the neurons of people with ALS and, instead of staying in the nucleus, the TDP-43 protein migrates out of the nucleus and accumulates in the cell's cytoplasm. As the TDP-43 protein accumulates in the cytoplasm of defective neurons, there is a gene called Stathmin2 (STMN2), which is involved in the growth and repair of neurons, that also becomes dysfunctional with the accumulation of TDP-43. Scientists believe that repairing the Stathmin2 gene may help patients with ALS at a molecular level. [Ref: https://www.medicalnewstoday.com/articles/324212.php]
Social Security Disability Benefits for ALS
If you have been diagnosed by a neurologist with ALS, you are likely to “automatically” qualify for Social Security Disability (SSDI) benefits under the Social Security Administration's (SSA) “Listing of Impairments” (the Blue Book). Electromyography (EMG) and nerve conduction studies may be helpful in objectively documenting your diagnosis of ALS, but the SSA does not require them.
If you have been diagnosed by a neurologist as having ALS, you may be eligible for an “expedited review” under the “Compassionate Allowances” program, which means that your application will be fast-tracked, and your case may be decided in weeks, not many months.
A diagnosis of ALS also bypasses the usual 24-month waiting period for Medicare eligibility. You immediately become eligible for Medicare coverage.
Your dependents could be eligible for benefits on top of your own Social Security disability. Eligible dependents include: a spouse age 62 or older; a spouse age 50 or older with a disability; a child under the age of 18; an adopted grandchild; and a spouse of any age caring for your child under age 16 years old.
If you have ALS, it is important for your medical care that you are diagnosed and treated by a knowledgeable and experienced neurologist. That is also important to obtaining expedited SSDI benefits for your condition.
At Law Med, we are knowledgeable about both the medical and legal aspects of helping you to get disability benefits.