Rare Motor Neuron Disease Destroys Muscles
Lou Gehrig’s Disease, also known as amyloid lateral sclerosis (ALS) is a rare motor neuron disease that attacks all the voluntary muscles in the body, so that eventually the person cannot chew, walk, talk, move or breathe. The disease got its name from Lou Gehrig, the famous baseball player for the New York Yankees, who died from ALS at the age of 38, although typically the disease strikes at people who are over the age of 50. The muscles become atrophied and paralyzed. The disease is progressive, and the symptoms get worse over time. Early symptoms of ALS usually include muscle weakness or stiffness, which can be a sign for other less serious diseases. Gradually, all voluntary muscles are affected, and individuals become very weak and lose the ability to speak, eat, move, and even breathe. When people lose the ability to breathe, most of them die of respiratory failure, usually about three to five years after the first symptoms appeared.
However, about 10% of people afflicted with ALS will survive for 10 or more years. The late British scientist, Stephen Hawking had a very slow early onset kind of ALS and he died at the age of 76. Until now no cure has been found, but two recent animal studies may lead to a breakthrough to treatments that can put an end to this terrible disease that has afflicted 15,000 Americans.
Supplementing a Unique Protein can Protect Mice against ALS
Results of a new study published July 12, 2018, in Cell Metabolism by researchers of Case Western Reserve University School of Medicine show that a unique treatment can set back the progress of ALS in mice by improving the muscular atrophy and paralysis of the disease. The researchers noted that a protein mitofusion 2 or Mfn2, normally found in the spinal cord, is usually missing or reduced in people afflicted with ALS. Nerve degeneration, muscle atrophy and paralysis were prevented by increasing the amount of Mfn2 in the spinal cords of mice models of the disease. In fact, the onset of the disease was delayed in mice by 68 days. Damage to the sciatic nerve was also helped by Mfn2.
Israeli Researchers Used a Treatment that Helped ALS Mice to Walk Again
Results of a Study by Researchers at Tel Aviv University in Israel were published June 13, 2018, in the Journal of Neuroscience. The study was led by Dr. Eran Perlson of the Department of Physiology and Pharmacology at the Sackler Faculty of Medicine at Tel Aviv University.
The researchers discovered that ALS causes a reduction of MicroRNA (the miR-126-5p) in the muscles and this leads to the muscles secreting toxic molecules that attack and destroy these same muscles. When they treated the mice, that had been generated to have ALS characteristics, with MicroRNA not only did the mice improve, but in fact regained the ability to walk.
Skilled Nursing and Palliative Care
ALS qualifies as a disease for which palliative care can be given and Medicare, Medicaid, private long-term care insurance and the Department of Veteran Affairs may pay for palliative care. Palliative care is extra care that can be given alongside regular treatment. As ALS progresses, there may be a need to seek long-term care and palliative care in a skilled nursing and care facility like Royal Suites Healthcare and Rehabilitation in Galloway Township, New Jersey.
These studies will hopefully lead to a cure for this terrible devastating muscle wasting fatal disease. However, there will need to be human clinical trials to see if and how they can work and if they will be as effective and safe in humans as they were in mice.