Status
Not open for further replies.
EMG day today ... looking forward to getting that over with.

Tokahfang, if I may ask - what age were you diagnosed at? Do you have a sense of how long things were going on before that, or how quickly they progressed? I know the accident you had complicates that history. Thanks for your advice and your story. There's not much information out there about HSP.

--Suzannah
 
I wasn't diagnosed until 2009. I had my first clinical weakness and hyper reflexes in 1995, at the time assumed to be the result of the accident. Other than a crazy time in late 2009, my progression has always either being comfortably slow (several years between different mobility devices) or plateaud (not really noticeable at all). Around 2009 I went from being a paraplegic with a few upper body symptoms to "lives in bed and needs a powerchair" in just six months. It was a big scare for us that it had turned into ALS, but no, still upper motor neurons only. ALSA permaloaned me a powerchair and I learned how to be a quad. I do ok.

My personal story gets downright weird once you hit 2011-2012, and so I have ceased saying anything publicly about my specific experience. If someone else comes up with my specific mutation of SPG7 and has a progression that parallels mine, then it might be useful to speak about. In the meantime, I had a very normal version of HSP until 2009, and then something more like "hereditary spastic quadriplegia", or PLS with complications until 2011. So when I speak here, it is in light of those 16 years of experience with a normal behaving UMN disease.

My particular HSP complications have included muscle breakdown in my wrists and jaw muscles, optic atrophy (had to learn to Braille), and sensory loss in the extremities. What complications are on the menu very much vary by the cause - mine is mitochondrial, a genetic cause that messes up your neurotransmitters instead has a very different set of complication options.

Yahoo has a pretty active HSP mailing list. I hang out on the PLS version these days, just because there is more relevant help there for a powerchair user, but they are both pretty helpful overall.
 
Wow, so I completely missed your question. I was 14 when the clinical weakness became measurably, 28 when diagnosed. That is what I get for answering pre-coffee!
 
FYI: Mine started in my lower back and R hip extending down to my toes. When I had severe calf cramps my toes curled downward toward the sole. I have hyper-reflexion, clonus, and positive Babinski. My EMG indicated UMN with LMN barely perceptible. My first major symptom was in 2006(one quick event that immediately ceased. Early 2009 is when I had my first major symptoms. My R leg is the worst, and it had the worst initial, painful symptoms. I don't know what you have going on. Could be ALS, or something else. The cramps and pain are long gone. They consider my ALS as a-typical since I am doing pretty well considering everything. I only had twitching near my R kneecap, which is long gone. My best to you.
 
Spasticity: Stretching relieves that for me. My PT gave me series of stretches to do morning and night. Laying on my back, I do 10 single knee to chest, 10 double knee to chest, knees to R and knees to L 10x, and hamstring stretches, each leg 10x. You may need to do less and should consult your M.D. and PT. It really helps me stay limber and avoid spasms.
 
So, I had my EMG on Monday and am profoundly grateful to have that out of the way. The doc said that she did not see any evidence of ALS, but she did see some very faint indictators of myopathy in both my arm and my leg. She said she didn't know what to make of that since my symptoms seem to very clearly indicate a nerve problem and not a muscle problem. I don't know what to make of that, either.

Next up is waiting to hear back from the insurance company to see if they will cover the test for HSP. Conveniently, they seem to have never received the first request, so the paperwork was refiled earlier this week. I should hear back in a few days.

@Summerguy, thanks for the suggestions for spasticity. I have a similar lower back right hip down through right foot thing going on, too. That leg is so tight and hurts so, so bad. But I had a bad hip injury a few years ago and it is hard to tell what's from what. But my left leg is now quite spastic, too.

@Tokahfang, thanks for the reply. That information is helpful. May I ask you one more question, if it is ok? I have been reading as much as I can find about HSP (which isn't much) and there's something I still don't fully understand, and that's the "paraplegia" part of it. My understanding is that the increasing spasticity is what eventually causes problems with walking and that is why people lose mobility. But along with that, do you (generic you) also lose sensation and ability to move the legs at all, like paraplegia caused by a spinal cord injury? I really can't get a sense for what really happens (and I realize that the different types present differently). And I will check out the Yahoo group.

--Suzannah
 
You can ask far more than one question. =)

I think you have the TV show simplification of what "paraplegic" means, and probably don't understand spasticity too well either. Neither of those things are surprising, and even on this relatively educated website people don't use spasticity as its proper technical term very consistently.

Let's start with your ideas about spinal cord injury. People who become paraplegics due to traumatic, sudden spinal cord injury (accidents, that kind of thing), don't necessarily lose feeling the way you think. Different parts of the spinal cord carry different messages, and it is possible to injure the motor (movement) part and leave the feeling relatively intact. The worse the injury, the less likely that will happen, but there is a range of sensory loss from total to none at all. Paraplegia itself as a term refers only to movement, not to sensation.

That whole "paralyzed from the -blah- down" thing is also an oversimplification. Different muscles in the same body part are fed signal by different levels of the spine, so it is quite possible to have control over one set of them without their antagonists. C-6 quadriplegics are the easiest example, as they have biceps but no triceps. Those normally work together to make arm movements, but you can learn to use just the biceps and set your equipment up to use the motions they can make with strength.

For someone to be called a paraplegic, they simply to have some paralysis in the lower half of their body. If they have weakness but no paralysis, that would be called a "paraparetic" instead. HSP stands equally for Hereditary Spastic Paraplegia/Paraparesis. Most normal people don't know what a paraparetic is, though, so you'll forgive those who don't bother to make the distinction I hope.

The core effect of HSP is the slow death of the upper motor neurons in a particular track of your spine. That is what all forms of HSP share, it is the same track hit by PLS and ALS. That is the "UMND" part of it. This causes hyperreflexia, spasticity, and yes, eventually often total paralysis of a muscle group. It is a death by a thousand cuts, but if signal can't get through those UMNs from your brain at all due to the extent of the damage, you will lose all voluntary control of the muscle group. My first paralyzed muscle group was the one that you use to push the ball of your foot downward. Unlike someone who becomes paralyzed due to lower motor neuron involvement, not much atrophy happens. You can't get a signal to that muscle group on purpose, but your damaged UMN's are spewing static at it. That is enough for the body not to automatically use it for spare parts. In the traumatic spinal cord world, this is considered very desirable because it keeps you from getting "wheelchair legs" and also makes blood clots and some immobility damage less likely.

Some forms of HSP also damage the nerves of one of the spine tracks that carry sensory information. Normally they do this slower than the motor part of the disease, so loss of sensation doesn't match the speed of mobility loss. What kind of sensory you lose can also vary - pressure, temperature, tactile.

Spasticity is a big enough and complicated enough topic I'll give that its own post later. I hope this helps in the meantime.
 
Status
Not open for further replies.
Back
Top