What is Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease, and Motor Neuron Disease?
ALS is a Neurodegeneration having shared characteristics with Alzheimer’s and Parkinson’s diseases. These are disorders of the aging Nervous System, with a peak onset at about 60 years of age. However, young-onset of ALS is not rare. ALS affects about 2 per 100,000 persons, but at any given time there are about 4000 people living with ALS in Canada.
ALS is rapidly progressive, with an average life expectancy after diagnosis of 3 to 5 years. However, about 15% of patients have a slower course and some may live for many years. Professor Stephen Hawking is a good example of slowly progressive ALS.
Classic early symptoms of ALS consist of progressive painless muscle weakness and wasting. This usually begins on one side of the body and can cause loss of hand dexterity, shoulder function, a foot-drop or walking difficulty. In about 25% of cases the onset in “Bulbar” with difficulty articulating and swallowing. Rarely ALS presents with breathing difficulty or a specific form of dementia (Front o-temporal dementia).
What is the cause of ALS?
The cause of ALS and other Neurodegenerations presently remains elusive. It is a complex, poly genetic (meaning many genes are involved, but each has only a small influence) disease associated with yet to be determined environmental triggers, occurring in the course of an aging nervous system. It is very likely that ALS and other Neurodegenerations, begin years or decades before clinical symptoms are apparent. There is presently a major thrust at identifying “early markers” of ALS, to stop the disease from progressing. Less than 10% of ALS cases are truly hereditary. It is better to appreciate that more than 90% of cases are NOT inherited.
What can be done to help patients with ALS?
Even though there is no specific treatment for ALS, much can be done to alleviate symptoms as they develop. This is best accomplished through the Provincial multidisciplinary ALS Clinic at the G.F. Strong Rehabilitation Centre, who work and collaborate closely ALS BC to achieve the best quality of life for the ALS patient and their caregivers. Both the Clinic and ALS BC were established in 1981. As outlined in other parts of the ALS BC Web-site there are many critical aspects of care that the Clinic and ALS BC have developed. It has been well established that timely institution of BiPAP (to help breathing), PEG Tube (to maintain good nutrition) and multidisciplinary care have significantly impacted survival in ALS.
There is reason to be optimistic, with significant advances in the near future. There are a close-knit group of ALS Physician-Scientists and Clinic Personal, throughout the World, who collaborated in an open and collaborative fashion. ALS research in Canada has been the envy of many for several decades.