Expertise needed

[Struminscott]

All,
I am looking for a little input from you kind folks. I will try to be brief.

I am a 55 year old male currently in hospital suffering with a NMD which is yet undefined.

History: 9 weeks ago I noticed faciculations in my left calf and a few days later it was in right calf. I was already having some issues with my right hand (like using keys for a few days). I blamed that on my neck as I previously had, 3 months earlier, numbness in the ring and pinky finger and pain in arm and back which came from cervical disc degeneration according to the X-ray.

Within a week though I could not type and could barely write my name. I had terrible tremors in both hands along with cramping that bent my pinky of the right hand up. I lost all my balance and could not walk a straight line or stand on one foot. I had no feeling off and on in my right toes. I had some vertigo as well. I could not even touch my nose with my index fingers as I would completely miss it and hit my cheek instead. I went to urgent care. The Dr suspected MS or ALS and did lots of blood work and ordered an MRI. The MRI was clean. I had an EMG and NCV the next day with a neurologist which didn't show any denervation or renervation and only some neuropathy from the disc narrowing in my cervical spine. He called it "essentially normal". I started to recover and got my balance back and the ability to type and write but the tremors in hands and fasiculations in legs stayed.
I had complete insomnia for the first three weeks. I had fasiculations in right arm but only when hand was extended open. When I made a fist they stopped.

A couple weeks ago I got a terrible rash that hit my thighs. They biopsied it thinking it was vasiculitus but instead they said it was an autoimmune response to a virus or infection. That cleared up with steroids. Dr thought maybe it was the cause of the neuro issues.

About 10 days ago the faciculations spread all through my entire body along with some myclonic jerks when I sleep. I had tingling in lips and tongue and my voice was horse. I ended up in hospital and they did lots of tests including another NCV EMG which was about the same as the first one 7 weeks earlier. Only difference was she thinks I have a nerve pinch in my right elbow. Fasiculations are really bad but they sent me home saying there was nothing else to test for.

Now a week later I'm back in the hospital with the same rash and some serious muscle atrophy in hands, legs, back and arms which has probably been going on since the beginning of this. I can walk and balance but the muscles are definitely atrophying and most of them are twitching. The Drs see this but don't know what is causing it. They twitch but still work. The doctors put me on 5 days of IVIG (this is day 2). I didn't have enough markers in blood or spinal fluid for difinitive GBS, or one of it"s variants. Can walk and balance and type but I am really concerned obviously.

So my question for you all would be does this match any of your experiences? Im eating like a horse to maintain weight and now with the atrophy I get bad cramps in legs and hands. I'm hoping maybe someone here has had the same experience and could shed some light. The Dr says I should repeat EMG NCV in a month to check for changes. I could atrophy to zero by then. If it was ALS does it match anything you have heard or experienced? It seems very rapid in advancing on so many fronts at once doesn't it?

I appreciate any input you may have.

Thank you so much from a fellow sufferer with neuro-muscular issues.

Blessings,
Scott

 

[KimT]

Scott,

I'm no expert but it sounds a lot more like autoimmune than ALS. I say this because you showed improvement after taking steroids. Have you actually been diagnosed with anything? What diagnoses, according to your doctors, are still on the table? Did they suggest a muscle biopsy or other tests? Have you been seen by a neuro who specializes in neuromuscular disorders?

I'm very sorry you're going through all this but your EMG points away from ALS.

 

[lgelb]

Agree, this all speaks not to ALS but more to an inflammatory myopathy such as dermatomyositis, for which steroids are usually first line. You're in a big city so would make sure you are seen at an MDA-affiliated clinic or the equivalent, e.g. UCSD.

Best,
Laurie

 

[Struminscott]

The rash improved with clobetasol which is a steroid cream I believe. The other conditions such as balance and dexterity improved on their own but the fasiculations and muscle atrophy continued and sped up. I'm at Sharp hospital right now doing the IVIG. Have had extensive testing for cancers, metals, most neurological diseases that have definitive markers etc. EMG said all F waves were normal. Super frustrating. I appreciate your input so much. Will ask about what you mentioned.
Blessings to you.

 

[KarenNWendyn]

Hi,
I'm a retired rheumatologist, now with ALS, so I'll do my best to provide input here.
I agree with others that ALS is not likely. Per my readings and experience, ALS doesn't generally progress at the pace of your symptoms and shouldn't be associated with tremors or loss of sensation. We're your reflexes brisk or absent? The latter might suggest more of a neuropathy.
If there is truly muscle atrophy, it's hard for me to believe the EMGs would be negative -- twice. The idea of dermatomyositis was raised. This generally involves proximal muscles (upper arms, thighs, neck, and trunk) and doesn't generally go along with fasiculations or sensory symptoms. Other types of myopathy may be possible.
Something like vasculitis is possible but would likely involve foot drop and involvement of at least one other organ system (e.g. Lungs, kidneys), and skin biopsy of the rash might have shown more.
IVIG is good for chronic demyelinating polyneuropathy. Also for Guillan-Barre. Were they thinking either of those?
Other considerations include some type of infection (Lyme disease is in that category but not likely in San Diego) and malignancy.
You need close followup, possibility a biopsy of muscle or nerves, and a good evaluation for multi system disease.
Best of luck.
Karen

 

[Struminscott]

Karen,
My reflexes have been zero in arms and normal to light in legs. No hyper reflexes. Had full body CT scan but it was clean as was MRI of head, neck and lumbar other than the degenerative disc issues. Was checked for West Nile, hepatitis, Lyme and lots of others.

I just looked up the NCV EMG on follow my health and here is the report verbatim
"Clinical Data: Right upper and lower extremity NCV/EMG study is essentially normal, only showing some sporadic reinnervation changes in a few upper and lower extremity muscles, no active denervation. Could be evidence of prior right C7/8 radiculopathy, prior ulnar neuropathy, and prior peroneal neuropathy vs L5 radiculopathy. No evidence of motor neuron disease, myopathy, peripheral neuropathy or neuromuscular junction defect. See report."

The latest one says basically the same thing " no evidence of active denervation"

Sorry I did not read the report till now. I just believed what the Dr said when he said no denervation or reinnervation...I suppose he meant active.

Scott

 

[KarenNWendyn]

Hi again Scott,
Sorry you are in such a diagnostic quandary. It sounds like they've worked you up pretty thoroughly and have ruled out a lot of bad stuff, yet no one has any definitive answers for you. That is so frustrating. Very likely they'll get you stabilized, send you home, and give you followup with one or more doctors. They need to keep looking. Sometimes things take a while to declare themselves medically. I wish you the best of luck and eternal patience.
Karen