Status
Not open for further replies.

GaborBP

Member
Joined
Sep 11, 2017
Messages
10
Reason
Lost a loved one
Country
HUN
State
Budapest
City
Budapest
Hi,

I understand that you're not doctors, and I sincerely appreciate the effort with which you try to answer the posts here while dealing with this terrible disease (as someone who has lost a father to ALS, I'm unfortunately quite familiar with it). I'm posting here because I'm currently in a diagnostic limbo with my neuro unavailable for some time, and kindly ask your opinion about my findings, and how do they compare to your or your loved one's experiences

I'm a 29 years old male working as a programmer, my father developed bulbar-onset ALS at the age of 54 and passed away after 2.5 years. He had non-affected parents and 8 siblings, his condition was tentatively attributed to a heavy head trauma with brain contusion 14 years prior to the illness, and possibly lifetime diesel exhaust exposure (there was a study about truck drivers more suspectible to SALS). In August 2015, I started to feel a heaviness/subclinical weakness in my left arm and hand, which was worse in cold weather, but mostly gone into remission by now. Shortly after that, I started experiencing bodywide fasciculations, for which I had exams in September 2015. The neurological exam was unremarkable, the EMG found no abnormality in the left opponens pollicis, abductor digiti minimi and tibialis anterior. I also had negative SOD1 and C9orf72 tests.

The fasciculations waxed and waned, slightly controlled by Carbamazepine and Diazepam, but somewhat worsened in the last few months, which I attributed to work-related stress. I have a hot spot over my left eyebrow which has been twitching almost non-stop for 2 years. Sometimes when I twitch, I experience an electric-shock like sensation goin down the side of my neck.

I have high arches (inherited from my mother) and I also experienced mild sensory symptoms, essential tremor, joint cracking, migrating back pain (scoliosis/sitting for long in an abnormal posture?) a spontaneously resolving right abducens palsy, and moderate trigeminal neuralgia-like episodes (every 3-4 months). Cramps were rare (once every 3-4 weeks), nightly and always happened in the calves. Prior to the onset of my disease, I had (mostly morning) nausea and vomiting for 3-4 years, which resolved appr. when the other symptoms started. I also had (and has) proctalgia for years, which is a literal pain in the ... My cervical MRI in 2015 was unremarkable (scoliosis), my cranial MRI in 2016 showed a DVA and a non specific periventricular occipital T2 lesion.

In December 2016 I had a NCS, which found a mild carpal tunnel in my right hand and mild generalized temporal dispersion suggestive of demyelination. I had a clean immune test, incl. anti-GM1. In July 2017 I had a 2 week episode of left shoulder stiffness, which was found to be not spastic in a neuro exam. I also had a 2 day neck stiffness shortly after that, which I attributed to my lifestyle and work. In the last few weeks, I started to feel bilateral knee stiffness/"wobbling"/"jelly-like feeling" (worse in the left side), which causes tremor when standing up after sitting for long. However, I was still able to hike 10+ miles in an difficult terrain even though I'm not athletic. What's more, my legs felt somewhat better after that.

A follow-up EMG in this September picked up the following abnormalities, which is summarized by the EMG neurologist as "mild abnormalities of uncertain etiology"
  • Mild amplitude reduction and temporal dispersion (worse than in 2016) in the left peroneus with decreased F-Wave persistence.
  • Increased insertional activity, CRDs when moving the needle or percussion, fibs and psw in certain needle positions in the left tibialis anterior. Mild subacute neur. lesion.
  • Incr. ins. activity, fibs when moving the needle, no spontaneous activity at rest. mild polyph. MUP in the left deltoid. This muscle was considered normal (?) in the summary
  • Incr. ins. activity, fasciculations, fibs in multiple needle positions, active partial denervation in the left frontalis.
  • Fibs and fasciculations in some needle positions in the right longissimus thoracic. Subacute neur. lesion.
  • Interference pattern/recruitment were normal.

My questions/concerns about this EMG are the following:
  • My understanding is that cranial and paraspinal denervation are highly specific for ALS. In that sense, and given the other findings, how do the "mild" and "unspecific" terms in the EMG summary compare to your EMG summaries/your opinions?
  • My understanding from the poly MUP in my arm EMG that it's indicative of reinnervation. Is it possible in ALS to practically regain all strength for months in a muscle?
  • Do the non-MND symptoms point to something or are they appear to be incidental findings? Has any of you experienced similar symptoms?

I strongly lean toward PMA/LMN-dominant ALS, but the onset of my symptoms, the lack of clinical weaknes, no athrophy (I have a dent in my right gluteus, but my neuro said it was pressure lipoathrophy, and I believe her), improvement in my arm and other symptoms seem very strange...

Thank you for taking the time for reading my rants :)
 
You have normal recruitment which is not the case with ALS
Increased insertional activity is non specific
Your findings are scattered. In ALS it starts in one muscle and spreads from there. We have had several cases of people who had multiple areas of one muscle with spontaneous activity ( at rest even not insertional) these areas cleared spontaneously and none have reported ALS

ALS does not get better

You EMG is non specific and anyway probably more likely to be an incidental finding than your actual symptoms

You lean towards PMA? Are you a neurologist or a physician of any kind? Your EMG does not support this. What did your neuro say? All EMG abnormalities are not ALS
 
Hi Nikki,

Thank you for the reassurance, I have one more question about recruitment: is normal stability with full interference pattern with some amplitude increase during voluntary activity considered "normal" recruitment? What about the polyphasic MUP?
I'm sorry if I worded the "I lean toward PMA" part wrongly or offensively (English is not my native tongue), what I meant was that given my father's disease, dirty EMG, LMN signs without UMN I'm deeply afraid of getting PMA. The EMG neuro didn't help either. he asked hypothetically what would/could I do if he said I had the same disease as my father, then after seeing my expression, quickly corrected himself, saying that the findings can point to anything, and he just wanted to point out that worrying about things I can't control will ruin my life. He's a great neuro and a nice guy, but clearly not an expert in psychology... Still waiting for my (non-EMG) neuro appointment
I would be really grateful if you could tell me the nicknames of some folks in the forum who had spontaneously resolving EMG abnormalities

Gabor
 
I can't remember the names. Please realize the volume of people who pass through this subforum. It happened again comparatively recently ( last few months) but that is many pages of posts ago

Recruitment is a separate observation / measurement

All dirty emgs are not ALS nor do they mean impending ALS

Your neuro is so right worrying does not help. This is often a problem with people who really are FALS- you are not- people with multiple relatives, identified genetic abnormalities. They worry about every twitch, every time they drop something- they let their genetic abnormality steal their lives before it has to. You are doing the same thing with less reason. I am sorry about your dad. I know the trauma of losing a parent to ALS but one relative does not FALS make
 
No worries, I'll try some Google magic to find those threads :)
You're absolutely right about the excessive worrying part, it's just that I clearly have some kind of an illness - during my last visit, my neuro mentioned the possibility of some mitochondrial disease, mostly because my mother's side of the family also has many non-specific, but not progressive symptoms like high arches, IBD, killer migraines, torticollis, allergies, kidney cysts, young onset cataracts, you name it. I'm just praying for it not to be FALS, because I've been feeling like crap for 2 years, but I'd very much like to feel the same level of crap for at least another 40 years..
 
Confusion about bulbar involvement testing

Hi,

I was wondering about the reason for testing forehead muscles during an EMG. As far as I know, bulb is the archaic name for the medulla. However, the forehead is innervated by the VII. nerve, which originates in the pons. Does anybody know about the relationship between those two regions?

Gabor
 
Status
Not open for further replies.
Back
Top