muscle atrophy and weakness/clean EMG

[juliev]

Hi, I was hoping some of you might be able to provide some insight for me concerning my husband's situation. He is 28 years old and has been having fasciculations for about 3 years now. Originally, we assumed they were benign. However, 2 years ago he started experiencing mild weakness in his left hand and left leg. The left leg was clearly showing signs of atrophy as it was noticeably smaller than the right leg. At that time, we worked with a neurologist and he had an MRI and a brain scan. Both were normal. They followed up with an EMG of his left leg and it was clean. His reflex testing was normal at the time as well. Since then, his weakness is increasing in his left hand, left forearm and bicep muscles. At least he feels they are weaker. When he grips my hand with both hands, the left hand grip is significantly weaker. He has also started experiencing mild numbness in his left pinky finger. The legs do not seem to be getting weaker. He feels like he is losing some of his dexterity in his left hand as well. He is fairly sensitive to this issue because he is a surgeon and works delicately with his hands. He does not have Hoffman's or Babinski's sign right now. Within the past 1-2 months, my husband has developed the feeling like he has a lump in his throat. He has no problem swallowing liquids, but solid food gets a bit sticky sometimes. He has also been struggling with GERD for several years now and we don't know if that might be a factor in this newest symptom. We will be following up with another neurologist very soon. However, we are still fairly nervous. I think the thing we are most curious about is if an EMG can be clean when you are clearly symptomatic for potential limb-onset ALS. He was experiencing weakness and atrophy at the time of the EMG. Thank you sincerely for any insight you might be able to provide.

Julie

 

[joelc]

Sorry to hear about your husband! But welcome to the forum. Fasiculations, numbness and lump in throat are not signs of ALS. Having a clear EMG is a good sign as well. If he did have ALS, his swallowing issue would be with thin fluids, like water, not solid food.

I don't know what to recommend other than seeing an ALS neurologist and pray he get a clean bill of health.

Don't be nervous as that only accentuates problems.

 

[laurel]

Hi Julie,
I think timing with EMG and nerve conduction tests obviously is critical. When my husband who is an airline pilot/now retired, noticed weakness in his right hand while flying and manipulating toggle switches, our GP referred him to a neurologist. He had testing done and seemingly the EMG and nerve conduction tests were negative and the neurologist sent him on his way. Then perhaps a year or so later the weakness increased and GP sent him back to the same neurologist who repeated the tests. Now he was diagnosed with Carpal Tunnel and was referred for surgery for two nerve impingments--hand and forearm. He had the surgery. No improvement. He returned to the surgeon six months after surgery and the surgeon said "it takes time for the nerves to repair". Hubby waited another year and during that year he lost almost all use of the right hand, and now had major wasting of the thenar muscle and major wasting of the forearm. He could not use eating utensils or hold his coffee cup. He retired from flying. He had lost faith in the neurologist and the surgeon. So we paid for another opinion at a specialty referral clinic and saw the top hand surgeon in our province. He listened to the history and examined hubby and said "you never had any symptoms of carpal tunnel. You have a progressive muscle atrophy happening and need immediate referral back to your neurologist". We went back to the original neurologist who threw a hissy fit feeling that his judgement was being questioned which it was. He only tested one limb again (which is one he did the previous two times) and said "you have something going on but I don't think it is Lou" (Lou Gehrig's Disease). He was very sarcastic and rude and unhelpful, but he did refer hubby to a neuromuscular disease unit. He was seen fairly promptly and tested on all limbs. He did at this point have minor swallowing issues and now developed drop foot of the right foot. The neurologist didn't want to diagnose him definitely and he was given a few differential diagnosis ie. CIDP, MMN, and ALS. He was given IVIG and had good response and after about 2 or 3 treatments of IVIG, the neurologist said that she felt fairly confident saying that he had an unusual variant of CIDP. So he has been on IVIG for nearly two years. He is doing fairly well but he has permanent damage done due to lack of treatment for that 2-3 year period when he was misdiagnosed. His EMG and nerve conduction tests show that all four limbs are impaired. So the moral of the story is to get to neuromuscular disease specialist, have all limbs tested as well. I am sure if hubby had had all limbs tested in the beginning it would have indicated some system wide thing happened, and the neuro. wouldn't have chalked it up to carpal tunnel. Good luck.
Laurel

 

[laurel]

Hi Julie,
I forgot to mention that my husband had had gastro intestinal disturbances for several years, and minor diverticulitis. There is some literature that indicates autoimmune diseases start out due to GI bacterial problems and the body goes into overdrive trying to fight the bacteria. My husband also had some very brief sensory symptoms in his hand and toes with fleeting numbness. And he had and does still have fasciculations in his arm and hand.
Laurel

 

[laurel]

Hi Julie,
Another comment. I told my husband about your husband and that I had written you about his saga here. He said that the skill of the EMG technician is critical. And many EMG sites say "The skill of the examiner is paramount in conducting and interpreting needle EMG testing."
Husband said that it was like night and day the way the testing was done with the first neurologist compared to the neuromuscular disease unit. And when I read through the original reports and compared them to the properly done studies, the second neurologist even put in a placating statement in the report that she forwarded to the original neurologist--something like "the differences in these studies are likely due to blah, blah, blah." I think she was trying not to step on toes in the old boy's network. And hubby did comment about his swallowing that his issues started with the feeling like he had a lump in his throat and then he started choking on saliva.
Laurel

 

[juliev]

Thank you for the replies. They were very helpful. I am not sure how thorough the EMG was performed. We will definitely be asking for a more thorough assessment. I'm not entirely sure the doctors are taking us quite as seriously because my husband is so young and it's hard to believe he really has symptoms like this. I appreciate your information!

Julie