Unless you were living under a rock during the summer of 2014, then you heard about and maybe even participated in what was known as the “ice bucket challenge.” Laypersons and celebrities alike took to social media to dump ice on themselves in the name of a good cause. With an element of fun, challenge, and a healthy dose of peer pressure, it was hard to resist joining in. With the raised awareness, many are beginning to notice a potential infectious contributor.
According to the ALS Association, the disease affects roughly 20,000 people in the United States at any given time. Most of these individuals fall between the ages of 40 and 70 and are given a grim prognosis. In ALS, motor neurons in the brain and spinal cord slowly die, leading to muscle weakness and loss of control affecting essential bodily functions. Many patients do not live more than a few years post-diagnosis. At this time there is no definitive cause or cure for ALS, although certain gene mutations have been implicated in familial ALS. The majority of cases (90-95%) are caused by sporadic ALS, which has no known cause or cure.
A Possible Missing Link
An open-minded and in depth review of the research on ALS, along with the personal stories of many determined patients and the equally determined physicians treating them, points to a potential infectious contributor – one that has been gaining more and more attention in recent years for its involvement in various conditions. This idea being swept under the rug by the majority of “experts” and ALS physicians is that late-stage Lyme disease, or neuroborreliosis, due to central nervous system (CNS) infection with Borrelia burgdorferi, may cause, contribute to, or mimic ALS. Although it is not clear which of these is most accurate (mainly because of the many obstacles in the way of performing well-done, unbiased studies that account for all of the variables involved), the arguments presented by proponents of this theory are intriguing to say the least. Similarities in symptoms, patterns of geographical occurrence, higher prevalence of borrelia infection among ALS patients, and success stories of recovery, are some of the main discussion points compiled in the book “When ALS is Lyme” (Vaughter, 2014). Let’s take a closer look at these correlations between ALS and Lyme disease.
While not all of the symptoms of neuroborreliosis and ALS overlap, many of them do – particularly in the early stages of ALS. The similarities become even more apparent in light of scientific discovery that debunked the previously held view that the disease process only affects the motor neurons. Neurologist Sanjay Kalra published two papers showing evidence that 50% of patients had brain damage significant enough to be detected on an MRI, while at least 15% were so severe that they warranted a diagnosis with dementia as a co-morbidity. Altogether, an estimated two-thirds of ALS patients have generalized brain damage, causing cognitive and behavioral changes in addition to motor neuron damage. Taking this finding into consideration, symptoms that overlap with neuroborreliosis may include:
- weakness (generalized or in limbs)
- loss of coordination/balance
- stiff or tight muscles
- muscle twitches (fasciculations) or cramps
- decreased concentration/memory problems
- difficulty with speech or eating
- other cognitive/behavioral changes
The Geographical Debate
Proponents of the Lyme-ALS connection assert that cases of Lyme disease and ALS are increasing at a strikingly similar rate across the Unites States and that ALS appears to be more common in areas with endemic Ixodes ricinus ticks (which carry borrelia). Data from a study on the prevalence of ALS in Minnesota, Washington, and Texas was compared to the Lyme prevalence in those same states (based on CDC data) and the relative risks between states followed the same pattern. Other spots in the U.S. that have had “ALS clusters,” including Kelly Air Force base in Texas (which was shut down shortly after the investigation) and Mascoma Lake in New Hampshire, are located near wooded and wetland areas where tick vectors are prevalent.
While investigations into these clusters were dismissed as coincidental or determined inconclusive, it is difficult to ignore, particularly when you combine the evidence with other geographical patterns. For example, worldwide, countries where Lyme-carrying ticks have spread have a higher prevalence of ALS. Clusters among an Italian soccer team as well as a group of British players serve as evidence that playing a sport in grassy areas (especially one that involves sliding in the grass) appears to exponentially increase the risk of developing ALS – more than the general public and even more than athletes of other sports. At the very least, it points to an environmental factor as further demonstrated by the French study that identified 9 couples with ALS (8 of them sporadic ALS). This would not occur as frequently if the disease was solely genetic in origin.
A study published last year by a former CDC researcher concluded that there was no geographical relationship between Lyme disease and 4 neurological disorders, including ALS. However, the study was based on deaths from these diseases, not occurrences. The authors cited several limitations of the study. One additional shortfall that was not mentioned is the possibility that patients with ALS-like symptoms who live in Lyme-prevalent areas may be more likely to be tested for infection. In cases where borrelia is indeed involved, this would lead to more timely and effective treatment, therefore resulting in fewer deaths in those locations.
Evidence of Infection
The authors of “When ALS is Lyme” collected the stories of dozens of ALS patients who were diagnosed with or treated for borrelia infection based on positive tests. In addition, there are case studies out of Germany, Sweden, and the U.S. documenting instances in which either ALS symptoms were caused by neuroborreliosis or their diagnosis was changed from ALS to Lyme disease. There are also a few cases of spontaneous recovery that can be found in the scientific literature, which would not be possible in distinct familial or sporadic forms of the disease. This again raises the question of an environmental factor that was unknowingly removed or treated, resulting in spontaneous recovery.
One of the main physicians arguing against the link between the two conditions conducted a study on the incidence of neuroborreliois in ALS patients. The findings were interesting. Although a close examination of his results revealed that 88% were infected, his conclusions downplayed these statistics. It boils down to this: ALS patients are 1,000 times more likely to have Lyme disease than the average American! The researcher was clearly biased in his attempt to settle the debate. Another researcher asked 30 ALS patients (selected at random) to get tested for Lyme disease. All 30 were positive, and combined with the additional patients that contacted him during the course of his research, he worked with a total of 150 patients with classic ALS who were also Lyme-positive. Considering the difficulty in getting accurate Lyme tests run in the first place, the number of ALS patients infected is probably even higher than the current evidence shows.
The story that has received the most attention in the Lyme/ALS world is that of Dr. David Martz, an oncologist who had a full recovery from his own ALS after treatment with targeted, high-dose antibiotics. He went on to treat patients with ALS and other neurological disorders using antibiotics and had mixed results. Even so, it is worth noting that according to Dr. Martz, about 30% of his ALS patients improved with antibiotic treatment. Additionally, anecdotal evidence can be found of others who recovered after addressing their borrelia infection.
Some critics cite published studies where ALS patients did not improve or even declined during antibiotic use. But physicians with the most success in treating these patients say that these experiments were doomed to fail from the get-go, due to the type of antibiotics used and inadequate dosing. Some say the worsening of symptoms could also indicate a “Herxheimer (die-off) reaction,” supporting the infection theory. Unfortunately, it appears that the chance of recovery once brain damage is extensive is slim to none. This further warrants more research in this area so that if there is indeed a link, patients can be tested and treated early on in the disease process to increase their chances of recovery.
Other Potential Causes
In lieu of the mixed evidence on the effectiveness of antibiotic treatment in ALS patients, we would be remiss not to mention other potential causes and contributors that have been explored in recent years. Other pathogens such as systemic mycoplasmal infections, human herpesvirus 6 (HHV6), chlamydia pneumoniae, and retroviruses have all been found in higher prevalence in ALS patients. It is unclear whether these infections are causative or opportunistic. Other theories involve glutamate accumulation in the brain, motor neuron autoimmunity, nerve growth factor deficiency, and genetic mutations involving superoxide dismutase.
Thanks to fundraising efforts like the “ice bucket challenge,” along with relentless researchers, determined patients, hopeful loved ones, and bold physicians, we may soon have more definitive answers. It may be that different patients have different underlying causes and most likely, finding a cure for ALS will involve a multi-factorial approach that addresses both epigenetics and environmental triggers. While there are still many unknowns, one thing is clear- at the very least, Lyme disease has once again proven itself to be the “great imitator” OR at most, it is playing a major role in one of the most devastating diseases of our time. Time will certainly tell.
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