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Suzannah

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Hi all,

I usually post in the PLS section since I've been diagnosed with a pure upper motor neuron disorder, but I wanted to post this here. The disorder I've been diagnosed with, hereditary spastic paraplegia, typically affects mostly the legs and generally does not cause atrophy, a lower motor neuron symptom.

However, I have been having problems with my hands and coordination for quite a while. Last December I had a neuropsych exam done, and my motor skills/strength scores put me in the "profoundly impaired" range. My neuro has noted weakness as well.

A couple of weeks ago a friend (an occupational therapist hand specialist) pointed out that my left hand is starting to atrophy. Mostly on the top of my hand between my thumb and first finger, and around and at the base of my thumb. My last two fingers are starting to be curled a lot of the time, and my thumb keeps extending backwards (like hitchhiking sign) and staying that way for long periods of time.

I went to see a hand specialist last week to see if it was maybe something mechanical, since I figure there's no law that two things can't be wrong with me. I played dumb and did not mention the neuro issues (oddly, he didn't check any reflexes. That would have given me away immediately). He noted the atrophy and found that I had 0/5 strength in my little fingers.

He said most likely radiculopathy or damage to the ulnar nerve. He rx'd an EMG and nerve conduction test, both of which I was have on Thursday morning. Incidentally, he described the tests as "sending little tickles down your arm and inserting small acupuncture like needles." Um, no. I have done those tests three other times, and they hurt like a ****. Especially for someone with severely spastic muscles.

Not to sound too much like a DIHALS-er, but I'm pretty worried about this and have been obsessively reading about hand atrophy and it's implications. I know some people here initially presented to the hand specialists before realizing it was neurological. Were the docs able to tell right away, or was it the EMG or something else that clued them in?

I'm worried. Thanks for listening.
Sincerely,
--Suzannah
 

lgelb

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Hi, Suzannah,

You're right -- HSP shouldn't cause what you describe. But we shouldn't jump to ALS (though I can understand why your mind would go there). Statistically, radiculopathy is more likely so I'd run w/ that until Thursday, anyway. To answer your question, I don't see how a hand specialist could differentiate confidently between a myopathic and neurological process, for example, without testing, esp. w/o doing reflexes!

But it's less than productive to have the EMG/NCT done by an examiner w/ an incomplete history. I would unmask, as it were, for that, esp. since they should compare Thursday's results with your last arm results (I'm presuming you had one or both done during the HSP process), with which you would need to provide them. And they should know that in advance to help focus the nerves selected. Because unavoidably, esp. given the length of time you say your hands have been questionable, this is somewhat about stress-testing the HSP theory as well as looking at other possible dz.

If you're going to experience discomfort for this test, I'd make it count. So I'd make a call, send your records and then stay off the net for the rest of the week. Let us know how it goes.

Best,
Laurie
 

Suzannah

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Hi Laurie,

Thanks for your nice, calming response.

I looked back through my medical records from my visit to Dr. Appel's clinic last summer (2014) to see if I had any weakness then, and apparently I did. Some 2's and 3's out of 5 on wrist and finger strength. Also full-blown UMN symptoms noted in my arms - hyperreflexes and clonus in all three arm reflexes on both sides. So I guess the weakness has been going on for a while.

I do plan to give the EMG doc a head's up for the reasons that you mentioned, but also because it will be immediately apparent that something neurological is wrong the second they start jabbing or shocking me (my muscles react pretty violently due to my level of spasticity). Also, I had some botox injections in my arms about six weeks ago that I will need to tell him about because that might cause some kind of error or misinterpretation.

As for staying off the internet, ha, yes, I should do that. I"m pretty sure I've been invoking minor signs of carpal tunnel by spending too much time on the computer looking this stuff up lately (yes, irony duly noted). Among other things, I am also a fourth year PhD student in public health and epidemiology, so not only do I have access to all the medical journals I want, I also know how to read and interpret them. A little knowledge can be a dangerous thing!

Thanks for your support. Test tomorrow. I'll report back.
--Suzannah
 

lgelb

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Sounds like a plan! BTW, I got my MPH at UTSPH Houston.
 

Green Queen

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Suzannah I hope that the testing goes well.
Thinking of you.
God bless, Janelle x
 

Suzannah

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Okay, back from my test. It wasn't too bad. After having 22 EMG-guided botox injections in one sitting last month (including 6 in my adductor/inner thigh muscles and four in the bottom of my feet) the regular EMG felt like a breeze. Mostly.

The EMG doc was very nice. Right at the beginning, he said, "So I assume you've probably never had this test before," And I said, "Well, actually ..." and told him the whole story. I told him that I was trying to differentiate between a mechanical problem, a lower motor neuron problem, or just a continuation of my upper motor neuron issues.

He did not find any evidence of carpal tunnel, ulnar nerve damage, radiculopathy, or lower motor neuron dysfunction. The only thing he saw was reduced recruitment, which is what my prior EMG said. Even though EMGs can't assess upper motor neuron dysfunction, reduced recruitment is consistent with UMN issues. I specifically asked if he saw any deinnervation or reinnervation, and he said no.

So, it doesn't really clear up much of anything, but at least I can stop worrying that I have LMN damage. Hopefully that is the last EMG I will have to do. Four in one lifetime is plenty.

Thanks for your support!

Sincerely,
--Suzannah
 

lgelb

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Great to hear. I'm sure you are relieved.
--Laurie
 

Nikki J

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Very happy to hear it. Hope you get some clarity at NIH
 

Green Queen

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Suzannah that is good news about the LMN.
All the best as you continue the search.
God bless, Janelle x
 

Suzannah

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Yes, I am very relieved.

The two main things I wanted to know were: is this a mechanical problem that I can fix, or, is this a LMN issue. I did get answers to both of those questions, so even though I didn't get an actual answer, I guess I can rule two more things off the list.

Today I saw the movement disorder neuro who I saw this past summer who told me I might have a leukodystrophy (I don't). She recently sent me for a DatScan (tests for dopamine deficiency), which was normal. Today she said, "I have no idea what's wrong with you. Come back in four months for a followup." Sigh.

Nikki, yes, very much looking forward to going to NIH next week for the diagnostic study. Hoping for some insight.

--Suzannah
 
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