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Amyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease that involves degeneration of the motor neuron cells with an average life expectancy of 2-5 years, post diagnosis. The incidence rate is about 2 per 100,000 with around 6,000 new diagnoses per year. This degeneration leads to progressive muscle weakness and eventual death. The problem with ALS is that there are multiple potential etiologies and no cure yet.
About 10 percent of ALS is caused by genetics. The genetics known today involve some of the following genes: SOD1, C9orf72, VCP, ALS1, TARDBP, FUS, ACSL5, ALS2, ANG, ANXA11, ATXN2, ATXN3, C21orf2, CAV1, CCNF, CHCHD10, CHMP2B, CHRNA3, DAO, DCTN1, DNAJC7, ELP3, ERBB4, EWSR1, FIG4, GLE1, GLT8D1, hnRNPA1, hnRNPA2B1, KANK1, KIF5A, LGALSL, MATR3, MOBP NEFH, NEK1, NIPA1, OPTN, PARK9, PFN1, PN1,2,3, PRPH, SARM1, SCFD1, SETX, SIGMAR1, SPG11, SPTLC1, SQSTM1, TAF15, TBK1, TIA1, TUBA4, UBLNQ2, VAPB, and WDR7.
In 90% of the cases, however, the cause is unknown. Given the uncertainty and the devastating nature of ALS, patients always often ask modifiable risk factors.
Risk factors for ALS include neurotoxins which include β-N-methylamino-L-alanine (BMAA), polyphosphate, acrylamide, smoking, metals, solvents, radiation, high intensity exercise, pesticides, and fungal and viral infections.
BMAA is found in tap water, swimming in contaminated water, food (shellfish: fish, mussels, crabs, oysters), shark fin soup, cartilage pills and can cause ALS and Parkinson’s disease in monkeys. BMAA can cross the blood brain barrier. It takes longer to get into the brain, butstays there for a long time.
Polyphosphate intake can increase the risk for ALS. Typical foods with a large amount of polyp are processed meat, ham sausages, canned fish, cola drinks and other soft drinks. Patients whose dietary phosphate intake was about the 99thpercentile died at a rate 2.37 times higher than the others.
Acrylamide, found in foods rich in carbs, which are cooked at high temperature, consumed by humans daily and is used in biscuit cereals, bread, crackers, coffee, and paper products. This toxin is involved in neurodegenerative diseases and can be linked to ALS.
Although further research needs to be done, cigarette smoking has also been reported to increase the risk of ALS.
Metals have been thought to be associated with ALS and studies suggest that cadmium, lead, mercury, manganese, and zinc could contribute to ALS etiology.
Solvents such as methylene chloride have been proposed to potentially be associated with an increased risk of ALS.
Radiation can cause an accumulation of misfolded proteins which could lead to neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, and ALS.
“The problem with ALS is that there are multiple potential etiologies and no cure yet.”
There have been some theories suggesting that there is an increased risk of ALS comorbid with an occupation that involves high intensity physical activity.
Professional athletes may be exposed to pesticides, thus increasing the risk of ALS. In addition to general pesticide exposure can increase the risk of ALS, specific pesticides include organochlorine, aldrin, dieldrin, DDT, and toxaphene.
Viruses that have been associated with ALS include poliovirus, coxsackievirus, echovirus, enterovirus A71, SARS-CoV-2, and enterovirus D68.
There has been compelling evidences of fungal infection in patients with ALS with previous reports of yeast and hyphae in their motor cortex, medulla, and spinal cord.. Candida, Malassezia, Fusarium, Botrytis, Trichoderma, and Cryptococcus have also been found in frozen neural tissue of ALS patients.
Of note, there was no significant association between alcohol consumption and ALS in previous studies.
Currently clinical trials for ALS involve the following:
Current medications given for ALS: Radicva (Edaravone), Rilutek (Riluzole), Relyvrio (Edaravone), Qalsody (Toferson)
Current devices for ALS : motorized wheelchair, eye gaze devices, ventilator, reachers, dressing aids, zipper pullers, door openers, lamp switches, key turners, pill dispensers, bathing tools, special utensils, and kitchen aids to help open jars, shower chair, bedpans, toilet seats, transfer benches, handheld showers, grab bars, hospital beds, over bed tables, patient lifts, bedside commodes, trapeze bars, lift chairs, safety rails, canes, quad canes, forearm crutches, walkers, rollators, manual wheelchairs, transport wheelchair, lightweight wheelchair, power wheelchair, scooters, oxygen, cpap, bipap, and ventilators.
Conclusion: Although there are genetic causes of ALS, the sporadic causes of ALS are unknown. Further investigation into the risk factors of ALS could help solve this neuroscience mystery.
