It is May and typically baseball season is in full swing. This month we are not going to dive into the intricacies of the sport but we will focus on the disease named after a famous baseball player, Lou Gehrig. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that attacks the human nervous system. It is also be referred to as Lou Gehrig’s disease named after Henry Louis Gehrig who was a famous baseball player for the New York Yankees. He played 17 seasons in the major league starting in 1923 and sadly ended in 1939 when he voluntarily took himself off the field when the disease hindered his performance as an athlete.
ALS is a progressive disease attacking the neurons within the brain and spinal cord. The cause of the disease is still largely unknown however approximately 10% of the cases are hereditary. Worldwide prevalence of the disease varies around the world. The most recently published review states that there are 4.1 to 8.4 cases per 100,000 persons. In the U.S., the ALS Association reports approximately 5,000 people per year are diagnosed with the condition. This is an aggressive disease. Only half of the people diagnosed live three years or more, while only 10 percent live more than 10 years. This is evidence that the disease can progress at different rates.
ALS is classified in three different categories. Sporadic is the most prevalent which accounts for 90–95 % of the cases in the United States. Familial is the genetically inherted strain that afflicts five to ten percent of the cases. The third classification is relatively rare in the general population, Guamanian, which gains the name due to the concentrated prevalence in Guam and the Trust Territories of the Pacific during the 1950’s.
The disease not only progresses at different rates, depending on the individual, but also has various symptoms associated with the progression. What typically begins with slurred speech or a small twitch in the hands or feet can lead to more life altering adherences. Tripping and falling can lead to muscle cramps or weakness further developing into difficulting walking. Weakness in the upper extremities can disrupt some of the simplest activities of daily living. Slurried speech can lead to difficulty swallowing and breathing. There are also cognitive deficients that can mirror other brain conditions like dementia. Since the disease attacks the brain, behavior changes can occurs such as laughing, yelling or crying inappropriately.
As of this writing, there is no cure for ALS. In the United States, four drugs have FDA approval to treat ALS. They are Riluzole, Nuedexta, Radicava and Tiglutik. These drugs treat some of the symptoms but does not cure the condition. There are neurotechnology therapies that may also help with the symptoms of the disease. Some evidence suggests that these interventions may slow the progression of the disease but none are proven at this point. Here are some broad categories of neurotechnologies that can be used by those living with ALS.
Communication in one area that has evolved into technology adoption. In later stages of the ALS disease, communication not only with loved ones but also with caregivers can be difficult. Electromyography or EMG can be used to connect with computers or environmental controls to allow for effective communication. The muscles around the eye seem to be those that are preserved is later stages of the disease and can be used as a means of communicating. Companies like Control Bionics and Tinkertron offer complete communication systems.
Respiratory or voluntary breathing also becomes difficult during the later stages of the disease. Ventilators are the typical line of treatment but there are other options for assisted breathing that could allow for more independence and reliability. Diaphragm pacing or phrenic nerve pacing are alternative options for breathing without a ventilator. These systems use minimally invasive electrical stimulation to allow for breathing using the contraction of the diaphragm. Commercial vendors such as Avery Biomedical, Atrotech and Synapse Biomedical offer devices of this nature.
Exercise is a means of warding off disuse muscle atrophy. There are in-clinic and home-based technologies to aid with this. Some are robotic in nature that assist with joint movement and range of motion. Most are used in rehabilitation or other therapeutic treatment centers. Another exercise modality which can be a home-based technology includes the use of electrical stimulation. This can be applied for the upper extremities such as hands, arms or shoulders while others apply to the lower extremities. FES (functional electrical stimulation) cycling can be applied to either upper or lower extremities and can provide cardiovascular benefits.
ADL assistance is another area where neurotechnology applications can aid in a variety of ways. Weak muscles make the simplest of tasks difficult to perform. This is where wearable technology can be a valuable aid. An example is an arm sleeve with embedded electrodes or a wearable arm robotic that can allow the user to pick up and hold items such as a comb or a toothbrush. Another example is the use of electrical stimulation for swallowing using devices such as the Vitalstim or Phagenyx.
These are just a few examples of the neurotechnologies to improve the quality of life for those living with ALS. Just as there are varied progressions of ALS, there are varied technologies for treatment. Neurotech Network offers free resources and a directory of devices specifically for ALS or Lou Gehrig’s disease. Check out our ALS/Lou Gehrig’s disease resource page here.
The pipeline for technology development is also vibrant. Here are a few examples of the recent technology developments that are close to becoming available for use by people living with ALS.
- C. Light Technologies is a company developing a diagnostic tool using artificial intelligence and retinal scan technology to detect small changes in eye movements that can be characterized with specific neurological conditions such as ALS.
- Eyefree Assisting Communications Ltd has completed a safety and feasibility clinical trial testing their EyeControl device which uses an eye movement-based communication device in the form of wearable glasses with connected infrared cameras that tracks the pupil and translates blinks and movements into commands.
- Researcher is Brescia, Italy have been investigating the use of transcranial direct current stimulation (tDCS) to help with symptoms associated with ALS. In a recently completed randomized, double-blind, sham-controlled study (tDCS_MND), the investigators evaluated whether a two-weeks’ treatment with bilateral motor cortex anodal tDCS and spinal cathodal tDCS can improve symptoms in people living with amyotrophic lateral sclerosis and modulate intracortical connectivity, at short and long terms.
- Another pilot study in Ulm, Germany is investigating Pharyngeal Electrical Stimulation (PES) for the treatment of dysphagia (swallowing deficit) associated with ALS. In the study, electrical stimuli are applied at the pharynx via a nasogastral tube with the aim of triggering reorganization processes in damaged brain structures. There is evidence of positive effects in people who are stroke survivors and those living with multiple sclerosis. They are investigating the use of this technology for those with ALS.
- ALS causes degeneration of motor neurons in both the brain and the spinal cord. Investigators at the VA medical center in Bronx, NY are developing an innovative method of using noninvasive cervical (neck) electrical stimulation (CES) and its potential to strengthen nerve circuits to facilitate active arm and hand movements for those living with ALS. Evidence from other studies of people with spinal cord injury suggests that activating spared nerve circuits with electromagnetic stimulation improves nerve transmission.
- Researchers at the University of Roma are investigating the use of neuromuscular magnetic stimulation to improve muscle function in people living with spinal-onset ALS. NMMS can be used to combat muscle atrophy by counteracting muscle catabolism.
These are some of the interventional studies being conducted for ALS using devices and therapeutics in the neurotechnology field. To reference those that are currently available along with other resources for ALS, check out our ALS/Lou Gehrig’s disease resource page here.
The content for this article was provided by Neurotech Network. The source of references include the ALS Association, Journal of Current Opinions in Neurology, and Mayo Clinic. Referenced trials can be found on ClinicalTrials.gov; trial ID numbers: NCT02891629, NCT03293394, NCT03481348, NCT03411863, NCT03618966.
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