Does this sound like PLS?

[bluedog]

I've posted in the DIHALS section in the past. My swallowing and speech problems seem more like what I've read here about PLS, so I am going to list my history and symptoms to see if anyone has any comments as to whether this could be PLS or anything else:

Roughly 2002 - 2004
-Stiff, heavy arms and legs
-Body wide twitching
-Postional vertigo, and constant off-balance feeling
-Numbness and tingling
-Cramping with exercise
-Dropped things
-Walking on inclines took more effort, but legs didn't feel weak
-Normal EMGs and MRIs during this time

2004 - mid-2012
Most symptoms went away except the vertigo and cramping.

mid-2012
-Problems swallowing solid food, such as sandwhiches. Food would get stuck in throat after swallow initiated and would have to wash down with water.
-Excessive yawning.

January 2013
-Feeling of spider web under eyes periodically.
-Rapid unexplained weight loss of about 15 lbs.
-Both arms and legs became stiff and heavy.

February 2013 to present the following symptoms evolved:
-Arms and legs stiff and heavy and feel weak (don't test weak clinically)
-Body wide twitching with some focal twitching in right eyelid and left elbow.
-Atrophy of right masseter and right side of face
-Atrophy of left triceps, small muscles around left elbow, and butt.
-Swallowing problem is the same, doesn't seem to have progressed in 18 months.
-Weak voice, with pitch loss and loss of modulation.
-Trouble articulating words. Started steadily about two weeks ago. Don't have the drunken sound. Increased saliva started at this time too.
-Air wasting so run out of breath when talking.
-Hands feel weak but don't test weak clinically.
-Shortness of breath on stairs.
-Insomnia
-Poor lip seal
-Thumbs feel sprained for days after carrying a water bottle while walking (thumb and index finger holding neck of bottle).
-When sitting, butt hurts from weight loss even on soft surfaces.
-Feet hurt for days after I walk barefoot. Seems to be loss of footpad.
-Positional vertigo
-Clumsy, incoordination.
-Off-balance feeling and walking into walls, doors, and other things.

I've gone to the gym regulary since January 2013 to see if I can gain strength and mass. I have gained almost 30lbs from my lowpoint and am gaining strength and mass in upper body. I don't work lower body because my hamstrings tear easily.
I walk three miles a day too, at which time my hands swell and my limbs and torso feel even heavier. I can run but ankles/calves burn. Can play basketball without too much trouble except incoordination.

EMGs in Feb and March 2013 were normal, following by 3 abnormal EMGs that showed chronic neurogenic changes in numerous muscles (large Motor Unit Potentials). No active denervation detected.

6 ALS neuros say I don't have ALS, inlcuding one two weeks ago.

Only UMN sign are brisk knee reflexes, though they never test me for spasticity. (The swallowing sounds UMN.)

ENT tests show that I am speaking with false vocal cords and have incomplete glottal closure in high pitch.

Any thoughts would be appreciated.

 

[AKmom]

You may have something going on, but PLS will start with 1 leg or foot and work its way to the other side or if its bulbar it will start with maybe some hoarseness and problems with swallowing liquids rather than solids. The fact that you mention numbness and tingling along with vertigo, as well as you have made progress by going to the gym and built some muscle back, tells me your issue is not PLS. Listen to the 6 ALS doctors. I hope you find whatever is causing your issues. You have not mentioned that you have had any work ups for Lymes, rheumatoid arthritis or Multiple Sclerosis, which could describe some of your symptoms too.

 

[bluedog]

Thank you AKmom. I had normal brain and cervical MRIs this year and back in 2003. Spinal tap in 2003 showed one faint oligoclonal band and slightly elevated protein. Evoked potentials in 2003 normal. Had negative Lyme and rheumy workups back in 2003 also.

 

[Bad Balance]

Sounds like you need to go to an ALS clinic and have an evaluation.
Sorry for your suffering

 

[bluedog]

Thank you Bad Balance. I've been to 6 ALS clinics, including Mayo, Cedars Sinai, and Barrow. They all say 99% sure no ALS. I don't get it. I think it is because I don't have any clinical weakness on exam.

 

[billbell52]

With PLS your symptoms don't get better so if it is PLS your symptoms from 2002-2004 are unrelated. PLS generally starts with your legs and progresses slowly upwards. I have Bulbar PLS (started with speech) and it started slowly descending. Moving from speech to your legs in 7 months is too fast. Although I continued to workout after getting Bulbar PLS the best I could was to maintain muscle mass. After 4 years of PLS I finally had to give up working out. I know how frustrating it is to get a diagnosis. Wishing you the best.

 

[bluedog]

Thank you Billibel52.

When maintaining muscle mass did you increase strength with the same muscle mass or did you maintain the same strength and then slowly lose strength until you had to stop lifting? I have gained strength and mass but not as much mass as expected with the strengh gains. For example, even though I have atrophy in my left triceps, I went from bench pressing 70lb dumbells two times to benching 100lb dumbells 6 times.

I also have a couple of follow-up general questions that you or maybe others can answer:

1- With PLS and ALS slurring, is there always thick, drunken speech associated with the slurring or can there be normal voice with just articulation problems? The patient videos that I've seen seem to be late stage where the patient has the drunk sound. I don't seem to have drunk sound, but have articulation problems, air wasting, and voice changes.

2- Is it unusual to have problems swallowing solids but no problems moving food around with the tongue?

Thanks

 

[vickim]

Have you looked into some forms of Muscular Dystrophy? Although I think clinical weakness is usually present. I know there are some forms that affect swallowing and hoarseness. The MDA website has lots of info.

 

[bluedog]

Hi Vickim,

I just read the MDs on the MDA site. I have symptoms of some of the MDs, like Kennedy disease. I would think that the neuros would have suggested testing for some of the MD diseases if they thought they were possible, but they haven't. One ALS specialist said he doesn't think it is Mitochondrial.

My EMGs show nerve damage, and not muscle damage (Large MUPs, not small MUPs), which I think points away from most of the muscle diseases. To me, everything points to ALS.

 

[vickim]

I had 3 emg/nvc that showed nerve damage in my feet. It was a muscle biopsy that determined I had mito. The 2 neuros I had seen didn't suggest it either and one worked in the mda clinic in a city south of us. It was the 3rd neuro at a university hospital mda clinic that figured it out and probably 5 years of progression.