Dear John:
If you had bulbar signs with ALS, then the EMG would be positive for the muscles involved in swallow,chewing, etc. It would show
normalNormal saline flush sensoryNumbness and tingling conduction, abnormal motor conduction, with muscle signs of
fibrillationsAtrial fibrillation/flutter
Implantable cardioverter-defibrillator
Ventricular fibrillation and fasciculations. However, to meet the criteria of EMG diagnosis, one needs three different areas to be positive. However, if the EMG were positive and the clinical exam fits the diagnosis would be ALS and your neurologist would have given you the diagnosis of ALS. We find that with bulbar type ALS, the parasaggitals are usually also involved. It would very, very unlikely that you would have only selective bulbar signs, without EMG findings and have ALS. In fact, you would probably be the first.
Unless there is EMG evidence, fasciculations are not diagnostic for any disease that we know the etiology of. Benign fasciculations occur without EMG changes or muscle weakness. They go away on their own (most of the time) and do not increase your chances of coming down with ALS.
If you truely have ALS with bulbar onset, the average lifespan is less than 1 year. So the onset of other signs on EMG would be quick. But, frankly, there would be other EMG changes in other muscle groups. People would notice the drooling, difficulty with chewing and swallowing and would have changed their diet to soft so they could chew. There would be episodes of choking-like occurances due to poor swallowing etc. The neurologist usually would have a feel for ALS upon the clincal examination.
I hope I answered your question.
Sincerely,
CCF Neuro MD
I think what the doctor meant about difficulties making the dx of ALS was that there are certain criteria (El Escorial) for making a dx of ALS; namely upper and lower motor neuron involvement in three spinal areas. So at onset if a patient had only bulbar symptoms and the other areas of their body were not yet affected, the dx of definite ALS cannot be given. Especially when other diseases like Kennedy's can present this way.
There are less strict criteria for making a diagnosis of possible or probable ALS. For example if you only have UMN and LMN signs in one area or UMN signs in two areas the diagnosis of possible ALS can be made.
However I am sure no doctor likes to give a dx of ALS for a patient unless there is absolutely no other possible cause.
I have a small question that is related to Johns, so I hope that it won't be violating the rule about secondary questions. Since the EMG is the standard tool for diagnosing or ruling out MND, what is considered a thorough EMG, in terms of numbers of muscles(or root levels) studied and number of insertions or MUP's per muscle.
Thanks so much.
john
You already had these questions answered by ccf under about 4 other names, why keep asking the same thing over? If you need that much reassurance, please check the archives and re-read your answers so other people can ask theirs.
questions and answers should be avoided!!!! Archives are a great source of information people.
You are correct in that I am not suppose to answer your posting. Actually, upper motor neuron disease is usually only inferred from the EMG as only muscle spasticity is present. However, the astute EMGer would pick up a strictly UMN. For the EMG diagnosis of ALS, three different muscle groups need to be affected with muscle dying. Kennedy's disease would be infered from the clinical exam and not be a problem with an EMG diagnosis. Also, the triplet repeat would be found.
Each lab sets their own number of muscles tested for a routine exam. So, the number various between labs. We have a baseline routine exam of number of muscles tested depending on the disease expected. For an ALS workup we do about 10 different groups and more if needed. The number of insertions per muscle varies according to the EMGer. At least 2-3 are necessary to define irritability of the muscle.
I hope this helps.
CCF Neuro MD
If the EMG is normal then the twitching or fasciculation you see or feel is not pathological. Therefore, it cannot be ALS.
CCF Neuro MD
CCF Neuro MD
CCF Neuro MD
Thanks for taking the time to answer my question. I would appreciate one small clarification if you have time. Regarding number of insertions wher you stated: "At least 2-3 are necessary to define irritability of the muscle." Do you define a single insertion as the actual needle insertion through the skin irregardless of the number of directions tested after insertion? Or does testing in multiple directions in the muscle after insertion through the skin count as multiple insertions.
The reason I ask is it seems like thorough EMG's are hard to come by, even at places like MDA clinics.
Thank you for your time and expertise.
Dennis
Insertions can be either complete new insertions or just movement of the needle in a new direction. Yes, many places the EMG is not done thoroughly. It is often due to Rehab people doing the EMG or people who are not trained in EMG.
I hope I have helped you.
Sincerely,
CCF Neuro MD
I've had it. I've "twitched" in most of my limbs for MONTHS following spine surgery (herniated disc). I can't take it anymore. Two neurologists in New York have dismissed my symptoms but I remain anxious about ALS, uncomfortable from the twitching, unable to resume my old exercise routine, and seriously depressed. I'd gladly come to Cleveland to see someone but I need to know who, where, and to know that I can come there and get this all behind me. I'm convinced I could handle almost any diagnosis but i can't stand the pain in my leg and foot, twitching, and anxiety. What to do? Please respond...
You can call 1-800-CCF-Care to make an appointment. I would see one of our muscle experts, such as Dr. Levin, or Shields.
CCF Neuro MD
Thanks for your reply. I really appreciate your taking the time to answer my questions.
Kindest regards,
Dennis
CCF Neuro MD