Progression of UMN symptoms

[Suzannah]

Hi,

This is a question for the folks with UMN disorders. Could you tell me a little bit about the timeline of how your symptoms have progressed? What started where, and when/how things shifted to other parts of your body?

I was provisionally diagnosed with HSP (hereditary spastic paraplegia) last summer, which is a pure upper motor neuron disorder that is sometimes difficult to distinguish between PLS. From what I understand, it typically does not affect the arms (though it can) and usually affects limbs fairly symmetrically.

I have UMN symptoms in all four limbs, and have also experienced a significant increase in spasticity in first my right leg and now my right arm in the past year or so. None of that really seems to fit the typical patterns for HSP. The only way to definitively diagnose HSP is through genetic testing, which I'm in the process of pursuing. I'm not fully convinced that's what I really have.

For those of you with PLS, is the progression similar to ALS? One limb, then the next, then the other side? Or slowly ascending/descending through the body symmetrically/asymmetrically? What has been your experience?

Thank you,
--Suzannah

 

[Clearwater AL]

First, ALS is rare, PLS is a rare form of ALS. For most (90%), PLS starts with a leg, then the other, then the trunk, arms and finally speech. Generally there may be an abnormal EMG but not a direct indicator of ALS. So, if it starts with speech... that person has cornered the rare of the rarest. Progression (for the most part) is very slow compared to ALS. As a Neuro once told me, "Use it or lose it... sooner" which is not the case for those with ALS. The criteria for diagnosing PLS is 3 to 5 years of exclusion and observation. There are some unspoken little secrets qualified Neuros look for during that 3 to 5 years. Remember, Neuros do not have to tell you everything they know and observe, his final diagnosis is a 3 to 5 year (or longer) summation of many things conclusively condensed into three words, "You have PLS". So, sadly, meeting the criteria... you may have already out lived someone diagnosed with ALS.

 

[Suzannah]

Hi Al,

Thanks for the info.

My neuro (a well-respected neuromuscular doc) has been pretty straightforward with me about things so far. She told me on my first visit that she thought it was HSP or PLS and that we needed to do an EMG to rule out ALS. After doing the EMG, which showed no signs of LMN dysfunction, she sent me to Houston Methodist to see Dr. Appel, who is the one who diagnosed the HSP.

But I saw her again last week, and she agreed that the progression wasn't typical of HSP, but that I have some other issues (cognitive) that aren't associated with PLS, either. My symptoms don't really fit very cleanly into either category. I'm not sure it's all that important, since there's nothing that can be done about either one, but I guess I would like to have at least a vague sense of what's in store on down on the road ...

--Suzannah

 

[Clearwater AL]

From what I have read here Dr. Appel is one of the best.

Tokahfang (Beki), one of the Moderators here has been diagnosed with HSP. Hopefully she can give you some better insight to what is going on.

 

[IhavePLS]

Suzanne, from my perspective, the reason that your sx. don't fit either PLS or HSP cleanly is that both are variants of ALS -- all of us reside somewhere on this ALS spectrum, some of us closer to the pure LMN end of the spectrum, others to the UMN end, some of us in the middle.

I can't tell you -- with any kind of precision anyway -- when or how my sx. began, nor can I tell you how they progressed necessarily, as I had two major back surgeries that "clouded" the neurological problem.

 

[Trippy]

Suzannah,

All of my limbs are affected to some degree but I do not have profound weakness or abnorml EMGs to date. My neurologist at Johns Hopkins still thinks I have PLS (just saw him again in December) but he also suggested HSP, saying there are many variations besides the 'pure form.' It is too expensive to get the genetic testing for me (10k) so I won't know if it's HSP. There are some good articles on the internet outlining all the HSP variants, and cognitive dysfunction is a symptom of several variants. Also my Dr. even tried me on a parkinsons drug for my 'slowness' but it didn't do much so i stopped it. I don't take any prescription meds right now.

I have had noticeable symptoms for at least 2 1/2 years, starting with left finger dexterity problems and jelly feeling thighs, but weird stuff happening for over five years, so who knows. I've progressed significantly in the past year and a half, but still get around on my legs, and use both hands. My left hand is still pretty strong but still lacks dexterity.

It's frustrating not knowing and always wondering. Hang in there. I keep having to remind myself to just take one day at a time.