At Neuro Today: Clinical weakness, atrophy, abnormal EMG

[Xina535]

and confirmed fasciculations.

He diagnosed me with plexus neuritis. I asked about ALS and he said this does not fit the ALS picture because:

-I have pain, also in my upper arm (that when the pain was really bad and intense in December, that my arm felt very sensitive to the touch, it hurt like he**). I even have a post about that when that happened (viewtopic.php?f=4&t=21329)
-I have normal reflexes.
-and that ALS starts in distal limb (but I am not so sure of this??)

Clinical weakness and atrophy in my shoulder. He had my stretch my arms out on each side and he pressed down and I needed to press up. My right arm stayed up but my left arm went down. I almost fainted when that happened. Then he said he sees less muscle in my left sholder than my right and he wrote down "amyotrophic shoulder". NICE WORDING!!!!!!! I do not see any differences in the mirror.

Then he told me he saw the twitching and also the EMG of my upper arm showed damage and "spontaneous activity" - he told me of individual nerve fibers - so I expect to see "fibrillation" in his report....I am guessing. By this point, confirmed weakness, atrophy, twitching, and then abnormal EMG with word of "amyottrophic" - I was just beside myself. He assured me (or tried to) though this was not the presentation of ALS which of course I feel mixed about. I mean, my exams have EVERYTHING we all fear - but just because of pain, normal reflexes and that ALS does not start with weak shoulders?? I am not sold.

He EMGed my upper left arm, like right in the center, he said where the C5 nerve supplies. This is where abnormalities were found. Then he stuck it in the bicep and that was normal. All other strength tests were normal, including lower body.

I am to start a cortisone therapy. 100mg for 5 days, then 50mg for 3 days, then 25 for 3 days, then 12.5 for 3 days ....then 12.5 every other day. This should help with all of my symptoms. Also to start physiotherapy, to help strengthen my shoulder back up.

I am just now learning more about this plexus neuritis. I have some questions based on all of this:

Does anyone else reading this have experience with it, or similar exam results or experience with oral cortisone therapy?
Can ALS start in the shoulder area?
Can you still have normal reflexes at this stage and it be ALS?
I know there were some cases where ALS patients did have pain early on....but is that thought of more of a coincidence or very rare right? and would the pain be extreme like mine was in December and then last so long?
Also, I still have pain, not as intense, and what I learned so far with this plexus neuritis that after the initial extreme pain, the pain leaves but weakness stays. But I still have pain.

I swear if I don't get any improvement soon with cortisone and physio, I don't know what I'll do!

[Xina535]

Oh, forgot to mention - I went to him to get an opinion on my cervical MRI, which shows 2 herniations at c5/c6 and c6/c7, but no nerve compression. His opinion is that there is nerve irriation from there, which is giving me neck pain but it does not explain my arm symptoms (so not cervical radicupathy).

Also, that the strong pulsing in my arm (heart beat) is an indication of the neuritis as well.

[Yuliasir]

well. Xina, as I said MANY TIMES, clinical weakness and even atrophy is not exclusively an MND symptom.
clinical weakness and atrophy happens in fracture (after immobilisation), in case of compression traumas and in case of neuritis of course. Due to pain you definitely avoided activity, so your miscles become a bit weak.

Pain is a sign of inflammation. ALS is not an inflammatory disease as you know, ad usually pain is due to spastcis which you do not have. "amyotrofic' means literally only "with weakened muscles" so having a fear of just the word is unwise.

I hope cortisole therapy would help.

hugs
yulia

[Xina535]

thank you - I looked up this word and it is associated with pain? Does this then probably refer to the later stages??

"Amyotrophy is progressive wasting of muscle tissues. Muscle pain is also a symptom. It can occur in middle-aged males with type 2 diabetes. It also occurs with motor neuron disease."

http://en.wikipedia.org/wiki/Amyotrophy

[Xina535]

I read that this neuritis is like 1.64 cases per 100,000 per year which is more rare than ALS at 2 per 100,000 per year? Huh? Did I read this right (wikipedia)?

[Xina535]

OH - and I told him I've always had a positve ANA. He said unless that is really monitored to see how high it can get and what kind etc...that is still a sign towards something auto-immune but hard to say exactly. I am going to give blood tomorrow for a full profile including ANA. I took cortisone today. I wonder if that would affect the test for ANA tomorrow?

[Yuliasir]

I have no idea if cortisol would directly and immediately affect ANA levels ^) Just do not know.

As for amyotrophy, again. this is the same - the doctor noted your shouldre has muscle wasting, that is all. Reasons for muscle wasting are still various. having clinical weakness in painful organ IS SO NATURAL - the more pain, the less movement you will try to exert, so muscle would be weakening naturally. I do not know about shoulder plexis neuritis, but even if this is more rare than ALS, this does not nean you could not get it. Studies for almost all diseases reveal that usually disease occurence rate is always UNDERESTIMATED, and it takes time to refine the figures, and becasue plexus neuritis probably does not lead to death, hardly there were many efforts to estimate its correct rate, unlike for ALS for example. 100 times and 1000 times correction of incidence are not simething unusual in the medical stats (like with celiac disease for example).

[Yuliasir]

Also important - the article in wilipedia is a stub and it dies not have any references or citations. please conside this.

you do not have AMYOTROPHY as diagnosis buy the way, only amoitrophic (WEAKENED, in normal, non-scientific language).

so please please do not panick. you know for a while that there is something wrong with your shoulder, so here is a diagnosis, and it is not ALS. Stay on this.

[Xina535]

Thank you - it helps to hear about the wording (although scary, is not the same as ALS), and it makes sense about the incedent rate. I took 100 mg of cortisone today and that will be it before my blood test, so I guess I will see what comes of it.

It's so hard not to panic or get depressed when the clinical and exams showed everything we hope it doesn't. Once I get the official report with the exact wording in the mail, I will post it. I am not sure if or when I can accept this. I guess I will have to see how the cortisone and physiotherapy helps. I start physio this Friday.

I had previous EMG in this arm and strength test in January by a Professor Neurologist and it was normal. Another normal strength test from him in Feb. I've also had normal MEP and SEP on both arms in Jan and Feb. I am so shocked, confused, scared and questioning everything! This is SO HARD to have, on top of twitching, when you have an insane fear of having ALS.

Does anyone know if ALS can start in the shoulder or is that like a very very rare thing?
Also about pain - I know primarily it is absent, but it has been reported by some with ALS early on. Does anyone know if they have pain early on, how bad/intense it is or if it dies down or anything else?

and are the normal reflexes on the arm that has clinical weakness a good sign? He said that my weakened/abnormal muscle is one that is innervated by C5, and that there are no reflexes with that one.

[Xina535]

Also, I found this - since I also found that plexus neuritis is a differential diagnosis to ALS. Greeeeeaaaat!!

http://pn.bmj.com/content/early/2013/04 ... 00557.full

It says:
Mimics: other causes of progressive motor-only weakness

Population-based studies have indicated that nearly 10% of patients who are diagnosed with ALS ultimately turn out to have another condition (see table 117), but only a few conditions account for the majority of these misdiagnoses. Mimics can be grouped into those presenting with LMN- or UMN-only signs and those with mixed signs. LMN presentations represent the greatest diagnostic challenge. Mimics are listed below in estimated order of frequency in our tertiary referral clinic setting. Key ‘red flags’ can be identified for each of these (table 2). Conditions with sensory involvement as a core feature, for example, syringobulbia, are not considered.


So I would think that plexus neuritis would not be listed, since it causes lots of pain (which is sensory). However, it does list it and says this:

Neuralgic amyotrophy

The Parsonage–Turner syndrome26 (neuralgic amyotrophy or brachial neuritis) typically presents with a history of severe unilateral neck, shoulder or arm pain in the absence of trauma, followed over weeks by progressive upper limb weakness and muscle wasting, often involving multiple nerve root territories. Up to 5% of cases may be painless, however. Lower limb variants are much less common. There is often a history of preceding viral illness or vaccination. In 10% of cases of neuralgic amyotrophy there is a family history, with half of these linked to a point mutation or duplication in the SEPT9 gene.27 The key to excluding MND is that the process of weakness and wasting clearly arrests, with variable and slow recovery over months to years. In 10% of cases there is a bilateral pathology, and a particular catch is the involvement of the phrenic nerves, producing diaphragm paralysis with orthopnoea.28



But why would PAIN not be listed as a KEY to excluding MND???

[Xina535]

It does list this in the Table 2

http://pn.bmj.com/content/early/2013/04 ... nsion.html


Mimic disorder - Key clues


Benign fasciculations - No weakness
Multifocal motor neuropathy with conduction block - Weakness greater than wasting and predilection for finger extensors, slowly progressive
Neuralgic amyotrophy - Severe pain at onset and arrest of progression
Kennedy's syndrome (spinobulbar muscular atrophy) - Very slow progression with gynaecomastia, and often prominent chin fasciculations
Motor-predominant CIDP - Symmetrical, fluctuating, mild sensory features, slowed conduction velocity
Inclusion body myositis - Slowly progressive, predilection for quadriceps and medial forearms especially with serum creatine kinase >1000 IU/l


So pain and arrest of progression, meaning after awhile it stops progressing or does that mean it is reversible?
I am sure about the pain, I am not sure about this arresting....

[Xina535]

Update - received letter with text including EMG findings today. PLEASE HELP me determine if any of this could also be indicative of ALS, or if it maybe points away from it. I am mainly concerned with the EMG, but I posted everything in case there are questions.

It is in German so I am translating it as good as I can with help of the internet for technical stuff:

Diagnosis:
Neuralgic Shoulder Amyotrophy DD Plexus Neuritis (Side note - what does "DD" mean?)
Chronic Cervical Neck Syndrome

Patient explained symptoms as neck pain (general) for about 1 year, but with no radicupathy into the arms during this time. End of December 2014, sub-acute symptoms started: intense, neuropathic pain in neck, left shoulder and left upper arm, on the left arm extending to the entire arm. There was some weakness, but mainly pain. As the pain died down over time, there was "twitching" in the upper arm and also in the radial side of the under arm. (Side note - he saw my twitching in my under arm at the appointment, but not in upper arm.) Since then, the main complaint is the weakness.

Until now, there has not been an explanation or diagnosis for these symptoms.

Neurological Exam:
There is a mild paresis of the shoulder abduction left side with strength grade of 4+/5, also in the inside rotation of 4/5 left side, otherwise no concrete paralysis.
The muscle reflexes are the same on both sides, no pathological reflexes
Sensitivity: in the problem area of the N. Deltoideus, there is Hyp- and parethesia. Fasciculations are not found here. (Site note - what is "hyp-parathesia? - I told him that that area is very sensitive. I felt like if he touched it too hard it would hurt, and it was warm/hot feeling)

Psychiatric Exam:
The finding is normal for patient's age. (Side note - FOR ONCE a report which does NOT say psychosomatic. In my other neuro exam by a professor Neurologist, he diagnosed me with psychosomatic symptoms in left arm)

EMG:
The Musculus Deltoideus left side were individual fasciculations and positive waves. (Side note - what does individual mean here - fibrillations? Aren't positive waves a characteristic of MND? Also the other neurologist tested this EXACT muscle with an EMG and said it was normal, in January. Then, I had "psychosomatic symptoms in my left arm")
Clear fragmented muscle action potentials with an increase of the potentials in respect to chronic neurogenic damage. (Side note - I have no idea what fragmented and increase potentials means)
The Musculus Biceps left side was normal.

Summary and Assessment:
On the basis of the case history, exams including electrodiagnostic, the patient has a so called Neuralgic Shoulder Amyotrophy in respect to Plexus Neuritis with participation of the C5 nerve root, but not so much the C6 nerve root. Next to the classic case history, the diagnosis is made more congruent with the electrodiagnostic and exams.

A high dose/short term cortisone therapy is to be attempted.
The physiotherapie should be started.
If symptoms have not improved after this therapy, Gabapentin may be tried, with a goal dosage of 300mg three times per day.

(Side note - Since taking 2 doses of 100mg of cortisone - the main symptoms are reduced. The pain is very small now. The burning/tingling/stinging in my arm is also waaaay down. Twitching is still there, and I want to say less, but not much. Weakness is the same. Start physio tomorrow).

[misterjuanperalta]

An abnormal EMG can be for different conditions, not exclusive to ALS. Unless at least two medical opinions agree it's ALS, don't worry.

Hyperesthesia (or hyperaesthesia) is a condition that involves an abnormal increase in sensitivity to stimuli of the sense.

DD is differential diagnosis.

An action potential produces the nerve impulse, and in the muscle cell it produces the contraction required for all movement. If it's fragmented, then it possibly means there is an interruption taking place in signaling. That's my best estimation.

In the summary, what you have is discussed here:

Neuralgic amyotrophy is a distinct clinical syndrome with acute severe pain and patchy paresis in the shoulder and arm region.

Source: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2780663/

[Yuliasir]

Xina,
have you seen posts from our fellows, for example - from mrjuanperalta? he had 'suspected ALS' written on his papers. German2 also had, in both cases not confirmed later.
Did you see that something in the report says - 'suspected ALS", not saying even of "diagnosis ALS"? In your case ALS is not even in the list of differential diagnoses.

You have MINOR paresis (4+ and 4 points of 5 of strength are preserved) on the shoulder muscles. Moreover, you keep all reflexes normal on both sides and do not show pathological reflexes which would definitely be presentm, should you have neurodegenerative disease for 1 year and more.

As you respond well on cortisol, this means you really have inflammatory issues, ad you choose a proper root.

[Xina535]

hi Everyone. Thank you very much for your time and replies! I received my blood work today, and there are many abnormal values. I will summarize below. Does anyone know anything about these values or if this means I have more of an inflmammatory/auto-immune thing happening or does any of this indicate something MND or what?

In the meantime, I sent an e-mail with my questions to the neuro directly. I asked him about the differential diagnosis - does that mean I have this or that, or both? I asked him if my EMG showed any SHARP positive waves, or fibrillations. I asked him if any of what came out of my EMG could because from nerve compression. I asked him basically if what my EMG says has anything to do with MND or shows specific signs for a neuritis. I told him about the changes I've felt with the cortisone. I was wrong about the twitching. It's still there as normal. The pain has died down. My arm feels so weak. Even though a 4/5 in two spots, it is still clinical weakness, and it is enough to definitely notice and feel doing everything I do. I asked him in his opinion after how many physiotherapy/strengthening visits would he expect me to have improvement, and what if it gets worse instead of better. I showed him my bloodwork. I am not sure when I will have an answer, but I will share it.

Any advice on the abnormal blood results will definitely help me out in the meantime! The ANAs are still pending. Not sure if 100mg of Cortison taken 1 day before this lab has anything to do with this.

Abnormal:
Leukozyten - 19.9 (normal range is 4-10)
Neutrophile Granulozyten (is listed twice with same name, both abnormal)

85.9 (range is 40-75)
17.1 (range is 1.6-7.00)

Lymphozyten - 8.80 (range is 17-47)
Monozyten - 1.02 (range is 0.20 - 0.70)

Kind of on the bar:
B12 - 346 (range is 211-911) - when I told him my values for B12 were always in the 300 range, he said that is pretty low. He wanted me to also get the Holo-TC lab, since that has something to do with B12, and that was 69.1 (normal is greater than 50)

CRP - 4.30 (anything under 5.0 is normal). The report says that anything between 3.8 and 5.0 means a higher cardiovascular risk - if I am reading this right.

Normal:
TSH - 0.67 (range is 0.55-4.80)
Creatinine - 0.64 (range is 0.60-1.10)
GFR (n. CKD-EPI) - 117 (range is greater than 60)
GPT (ALAT) - 11 (range is less than 35)
CK Creatinkinase - 47 (range is less tan 170)

Cholesterol, blood sugar, iron, calcium, etc etc - all were normal. I have many on this list.