General Neurology Division Chief Mark Bromberg, MD, PhD, presented at the August session of Talks with Docs.
If you were unable to attend the event, don’t worry! Dr. Bromberg has gathered your burning questions and prepared a series of thoughtful responses.
Questions and Answers
Q: What causes ALS?
A: The cause (or causes) of ALS are not known. Some have a clear genetic component, indicating an abnormality in a specific protein, but there are many different gene mutations, and the mechanism is not known for any of them. Most cases of ALS are sporadic, and there are many theories, but so far, no specific mechanism is known. There are many research laboratories working on this question.
Q: Is ALS equally common among men and women?
A: ALS is more common in men; it is ~2 times more common than in women.
Q: Is ALS related to age?
A: Yes. Onset of symptoms is rare in persons in their 20s. It is more common with advancing age, and the most common age of onset is in the mid-50 range. However, onset can be in the elderly as well.
Q: My brother has Adrenomyeloneuropathy. How similar is it to ALS?
A: This is a very different condition. AMN is an x-linked disorder.
Q: Can ALS affect the muscles in my neck? I can’t hold my head up without difficulty, but I have no noticeable problems with weakness in my arms or legs. I am on AVIG treatment.
A: This question is difficult to answer without seeing the individual. ALS can cause weakness of neck muscles and changes in voice, and ultimately affects breathing. At this stage, there is usually weakness of arms and legs. Unfortunately, I am not familiar with AVIG treatment; you may mean IVIG treatment, but this therapy has not been shown to be effective with ALS.
Q: Are genes ever analyzed in making the diagnoses?
A: The diagnosis of ALS is from the history and clinical (neurologic) examination, and it is aided by EMG studies. Gene testing is usually performed after the diagnosis.
Q: What other disorders cause fatiguability besides MG (Myasthenia gravis, a neuromuscular disease distinct from ALS)?
A: Fatigue can be defined by very rapid onset (minutes), and this is characteristic of MG. In addition, many muscle diseases can cause fatigue over many minutes. ALS can also cause fatigue over an hour or so.
Q: Can arachnoiditis produce ALS-like symptoms?
A: I do not think so; if you are referring to lumbosacral spinal arachnoiditis, it would not affect arms or speech.
Q: Is there any correlation between Lyme disease and ALS?
Q: As ALS develops, how common is it to develop twitching? At what point does that develop?
A: By twitching, I think you mean fasciculations. Everyone experiences fasciculations, but they are rare over time. In ALS, fasciculations are an early observation, and in a diffuse distribution across the body, and the size of the twitch is greater. It is not known why fasciculations are so common in ALS.
Q: Do you consider vitamin or nutrient deficiencies in treatment?
A: I do not, but I am open to anything that clearly slows the rate of ALS progression. There is an informative web page that looks objectively at vitamins and supplements: ALSUntangled.
Q: Is it possible for you to summarize the progress in the research for cure/drugs over the last 5, 10, 20, 50 years? It seems that there is a lot going on right now (as you referenced the 2014 ice bucket challenge). Is the future for remedies brightening your opinion?
A: Before (and after) the Ice Bucket Challenge, many laboratories worldwide focused on ALS mechanisms, which has led to many drug trials. There has been a marked increase in drug development companies over the past few years. There are even new drug trials designed to speed drug testing. In my time with ALS (35 years), this represents a massive increase—one result is that two new drugs have been approved in the past six years, compared to the first drug approved 29 years ago.
If you would like to view the recording of this event, please reach out to Melissa Lyons at Melissa.Lyons@hsc.utah.edu.
The next Talks with Docs event will take place on Wednesday, October 11, at 4:00 p.m. on Zoom. It will feature Kathleen B. Digre, MD, who specializes in neuro-ophthalmology and headache. You can register for it here.
Want to be notified of future Talks with Docs events in other fields of neurology? Sign up for future info here.