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Yfeasmth

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Hello Everyone,

I have been following the forums for sometime now and this is my first post. I will try my best to describe my symptoms and tests.

Symptom onset: Lower back pain with tripping over feet (right foot). Lumbar MRI ordered (see below). (Lumbar did show some issues, but the neurologist believes the issues are minor.) Proceeded with shooting pain down my left leg, numbness in the groin region, tingling sensation in the feet, legs and hands. Symptoms persisted. As time went on I felt dizzy and generally uneasy, I was running into things and felt stiff. My legs also felt like they were shaking and I couldn't stand for long extended periods of time before having to sit.

Saw Neurologist for the first time. He examined the MRI and gave me a perfect normal neuro exam, except he noted hyperreflexia and noted the numbness. Ran a series of MRI (Head, cervical), CT of cervical, and a series of blood tests. Everything came back normal, so he suggested we go with an EMG to see if we could find anything. He tested my right leg, left leg, lumbar paraspinal and thoracic paraspinal muscles. The EMG came back abnormal, the nerve study was normal. (See below). A tremor has recently developed on both sides (started left hand, not is on the right hand) and my entire body aches with bone pain and shooting pains.

Our next step is an MRI of the Thoracic Spine with and without contrast and if that is normal a spinal tap. He said he doesn't know what is wrong with me and if these tests are normal it opens up a whole host of new questions he simply doesn't have the answer for and intends to send me to John's Hopkins for further evaluation.

My big question is, on the EMG study he suggested I have a multifocal disorder of the motor function and a clinical correlation is required. He said he doesn't believe it to be ALS, but has no explanation for the denervation in the back (he is hoping he finds something on the thoracic MRI that could explain it) and continues to ask if I remember anyone ever telling me that I had hyperreflexia. Based on my understanding, MMN is only lower neuron and not upper, whereas hyperreflexia suggests upper issues.

Any input or advice would be appreciated. Please see attachments for examinations. Family history with ALS on Paternal Grandmother. Dad and his siblings do not display symptoms.
 

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He is trying to determine if the hyperreflexia existed before your current issues, so as to consider it part of your current sx or not.

There are a lot of possibilities here, so I'd keep going through the process. There are many types of motor neuropathy that are not ALS. Hopkins is a good place for a second opinion.

Best,
Laurie
 
Thank you for the response. Have you had any experience with Johns Hopkins? I am surprised they aren't listed on the ALS association page as a certified ALS Association Centers given that they do ALS research. I had my thoracic MRI today and can expect the results within the next 48 hours and we will go from there. I've asked the neurologist repeatedly if he thinks it is ALS and he give me reasons why he thinks it isn't. The one I am clinging to is that it my symptoms do not match typical ALS disease progression. Searching for possible reasons for the denervation though and you don't seem to get a whole lot of answers, except to point you in the ALS direction. From what I've read, if it is MMN, the neurologist should be able to confirm that with a blood test, or maybe the spinal tap? I am not sure. Anyway, thank you for letting me vent, my stomach is in knots. On a side note,I will ask him for a neurologist recommendation at Hopkins and make an appointment.
 
My sister had her second opinion at Hopkins ,an MDA clinic which is equally respected as a designation. The doctors are great and very skilled diagnosticians.
I can't view your thumbnails to comment.
MMN is tricky. There is a blood test and there are findings on the NCS but not everyone with MMN shows these things. I do not believe there is a specific finding on spinal tap. I suspect you read a report I found when the thought I had MMN. When I asked I was told it was not a validated finding. My np even looked up records from their MMN patients to confirm this and said their soinal taps did not show whatever the proposed marker was
 
Thank you Nikki. Is there a way for me to make them visible?
 
I think the issue is mine not yours. Laurie seemed to see them.
 
EMG Study-

R. Tibialis An - inc insertional activity, 1+ fibs, 1+psw
R Flx.Dig.Ln - inc insertional activity, 1+ fibs, 1+psw
R. TFL - increased insertional activity
L. Tibialis An - inc insertional activity
R. MidLumbpara- increased insertional activity, 2+ fibs, 2+ psw
R UppLumbPara - increased insertional activity, 1+ fibs, 1+ psw
R. Thoracicpar - increased insertional activity, 1+ fibs, 1+ psw

Everything else on the EMG was normal.

Here is his summary: Needle EMG of the muscles indicated in the lower extremities and paraspinal muscles demonstrated some scattered fibrillation potentials in the right leg consistent with an L5-distribution lesion. However, paraspinal muscles demonstrated fibrillation potentials and high-frequency discharges at multiple levels in the lumbar as well as thoracic region. No fasciculations were seen. The configuration of motor units as well as firing pattern, interference in recruitment were all normal.

Impression: These findings suggest a multifocal disorder of motor function as indicated by the needle EMG study, clinical correlation is required.
 
Thanks. Agree with Laurie. Hopkins can help you sort it out
 
Hi Yfeasmth
With the MMN they look for conduction block in the NCS test and anti-body indications in the blood tests, I came back negative on both these but given my presentation which was purely lower motor neurone, and a history of autoimmune problems my specialist still felt trialing the Ivig treatment for MMN was worthwhile. My EMG showed denervation in all areas

They said it wasn't worth doing spinal fluid test as that wouldn't be indicative. At the end of the day I was told that they would give me the treatment and if it works I have MMN if it doesn't I have MND but a lower motor neurone variant. In the meantime they have me on riluzole too.

It's worth holding on to the hope, I am :) but it does sit you in limbo on the diagnosis. Mine is currently on probable mnd/ possible MMN until the treatment trial is finished.

I hope this helps
Wendy x
 
Thank you both for the reply. Good luck Wendy, I hope things turn out ok.
 
Just a quick follow-up question to both Wendy and Nikki. When you had an EMG done, were your results similar to mine? Given that ALS is a motor neuron disease, I would expect something on the EMG to show fascics or issues with MU amp duration configuration pattern interference or recruit. Or would this be a case where it is very early onset and thus that hasn't shown yet. I realize none of us are neuros, I am just trying to understand the EMG results and why insertional activity, fibs and psw alone point to ALS. It seems like you would need to show some sort of MU problem as well.
 
Yes I had the MUP issue as well as fibs and psws and it was very early. The MMN question arose when I had a positive antibody panel
 
Hopkins is "certified" by the MDA, not ALSA. A few centers have both. Most have one. It does not differentiate competence, only where a bit of money goes.
 
Hi
i will sort out my last EMG summary but from what I remember there were fascisculations and fibrillation noted Sorry there will be a bit of a delay as everyone is out today and the records are out of reach.

On it being too early I don't think it can be with the EMG results, I was tested in each arm and leg,rib cage, back, throat,neck and jaw. I only had symptoms in my right leg and left arm at that stage, i felt completely normal elsewhere. But the results showed abnormalities in every area tested.

I will pop the results on for you as soon as hubby gets home.
Wendy X
 
Hi
The EMG results are in a very different format, I hope they mean something to you.

Right deltoid
Occasional fascisculations, increased insertional activity. Moderate excess of polyphasic units or normal or increased duration. Units firing at high rates in isolation in a moderately reduced interference pattern to 10mv

Right Biceps
Occasional fascisculations. Moderate excess of polyphasic units of normal or increased duration. Some rather large units recruiting early to a mild moderately reduced interference pattern to 5mv

Left Deltoid
Profuse fibrillations and positive sharp waves. Single wide unstable unit firing in isolation to 6mv

Left Biceps
Profuse fibrillations and positive sharp waves. Single wide unstable unit firing to 8mv

Left Triceps
Profuse fibrillations and sharp waves 2-3 wide unstable firing in isolation to 8mv

Right Rectus femoris
Profuse fibrillations and positive sharp waves. Unstable wide units, units firing at high rates in relative isolation in a single unit pattern to 6mv

Right Tibialis anterior
Profuse fibrillations and positive sharp waves. Unstable polyphasic wide units firing in isolation in a single unit pattern to 6mv

I have just included my bad left arm and right leg and my good right arm for reference and the overall summary below. I am sorry they just tested too many areas to type it all in here and the results were fairly similar in most areas.

Summary
Small motor cmaps in the left hand and from the right foot. No evidence of dmyelation other than mild ulnar slowing around the elbow.

EMG findings confirm widespread acute denervation in upper and lower limbs and paraspinals with wide unstable motor units compatible with an ongoing denervation process seen in all muscles sampled. Masseter and tongue appear to be mildly denervated.

These findings confirm a widespread progressive motor axonal neoronopathy and would be compatible with motor neurone disease ( particularly with the extent of fibrillations seen); the severity of the denervation in some muscles ( ie very severe in left biceps and triceps) may account for the loss of reflex in these muscles.

I hope this helps
Wendy x
 
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