robc
Active member
- Joined
- Jan 23, 2007
- Messages
- 47
- Reason
- DX UMND/PLS
- Country
- US
- State
- Oregon
- City
- Silverton
Hi everyone,
I'm thinking that I'm at the end of the line in terms of ruling things out that might mimic ALS & am probably lucky if I end up with a PLS diagnosis, but I suspect it may end up being ALS.
My question is, has anyone here with ALS had an onset similar to mine, with upper motor neuron symptoms coming first.
I'm 35 years old & had originally started seeing doctors a couple years ago for exercise intolerance problems, which doctors now believe is due to some sort of metabolic disease unrelated to my motor neuron disease.
About a year ago, doctors noticed brisk knee reflexes & have since found hoffman's reflex, jaw reflex, and brisk arm reflexes. I am having trouble standing or walking for more than 10 minutes or so at a time (my legs start to feel heavy, weak & this seems to trigger more spasticity). No bulbar symptoms, no sensory problems. My gait is slow & akward I guess due to the spasticity.
Around February of this year I started to develop twitches (short burst pulses of my muscles everywhere - arms, legs, trunk, butt, etc). They last between 1 second and 20 seconds. Back in February I'd maybe have a couple an hour, now I have 20-30 an hour. The twitches are not painful, but I worry about them representing denervation.
To me, it seems like the loss of function in my legs is relatively quick (most of this has developed over the last 8 months).
I did have a biopsy back in December that showed fiber type grouping, some polygonal atrophic fibers & some mild lipid content. No other abnormalities.
I don't have any visual indication of atrophy & my short burst strength remains very good. I've had a little bit of trouble opening jars or plastic packaging, but not much. I just can't sustain strength very long.. my legs start to get weak when walking, arms start to get weak when using a manual wheelchair (I use a power chair now).
I keep reading that ALS is supposed to be painless, but I find posts from people that seem to have pain with spasticity, so I'm guessing that while many people don't have pain, some do.
I'm at the Cleveland Clinic this week & they're doing genetic testing for HSP & today I'm having a much more thorough EMG than I've had before (theirs takes 2 hours, vs the 5 minute EMGs I've had before).
So back to my original question. Has anyone here with ALS had an onset similar to mine with upper motor neuron symptoms at the onset (spasticity, pain, brisk reflexes) instead of lower motor neuron symptoms (atrophy)? And I guess one more question - is there anything else left to rule out besides PLS & HSP that would cause upper motor neuron reflexes, spasticity, & twitches?
I've had normal Brain & Spine MRIs, normal somatosensory evoked potentials, normal CK & CBC levels, normal vitamin E, B, copper (borderline normal), MMA, HLTV, HIV, normal spinal tap (just very slightly elevated protein level), abnormal lactate (too low with exercise), slightly abnormal EMG back in January (small motor unit potentials, slight increased turns).
Thanks a bunch!
-Rob
I'm thinking that I'm at the end of the line in terms of ruling things out that might mimic ALS & am probably lucky if I end up with a PLS diagnosis, but I suspect it may end up being ALS.
My question is, has anyone here with ALS had an onset similar to mine, with upper motor neuron symptoms coming first.
I'm 35 years old & had originally started seeing doctors a couple years ago for exercise intolerance problems, which doctors now believe is due to some sort of metabolic disease unrelated to my motor neuron disease.
About a year ago, doctors noticed brisk knee reflexes & have since found hoffman's reflex, jaw reflex, and brisk arm reflexes. I am having trouble standing or walking for more than 10 minutes or so at a time (my legs start to feel heavy, weak & this seems to trigger more spasticity). No bulbar symptoms, no sensory problems. My gait is slow & akward I guess due to the spasticity.
Around February of this year I started to develop twitches (short burst pulses of my muscles everywhere - arms, legs, trunk, butt, etc). They last between 1 second and 20 seconds. Back in February I'd maybe have a couple an hour, now I have 20-30 an hour. The twitches are not painful, but I worry about them representing denervation.
To me, it seems like the loss of function in my legs is relatively quick (most of this has developed over the last 8 months).
I did have a biopsy back in December that showed fiber type grouping, some polygonal atrophic fibers & some mild lipid content. No other abnormalities.
I don't have any visual indication of atrophy & my short burst strength remains very good. I've had a little bit of trouble opening jars or plastic packaging, but not much. I just can't sustain strength very long.. my legs start to get weak when walking, arms start to get weak when using a manual wheelchair (I use a power chair now).
I keep reading that ALS is supposed to be painless, but I find posts from people that seem to have pain with spasticity, so I'm guessing that while many people don't have pain, some do.
I'm at the Cleveland Clinic this week & they're doing genetic testing for HSP & today I'm having a much more thorough EMG than I've had before (theirs takes 2 hours, vs the 5 minute EMGs I've had before).
So back to my original question. Has anyone here with ALS had an onset similar to mine with upper motor neuron symptoms at the onset (spasticity, pain, brisk reflexes) instead of lower motor neuron symptoms (atrophy)? And I guess one more question - is there anything else left to rule out besides PLS & HSP that would cause upper motor neuron reflexes, spasticity, & twitches?
I've had normal Brain & Spine MRIs, normal somatosensory evoked potentials, normal CK & CBC levels, normal vitamin E, B, copper (borderline normal), MMA, HLTV, HIV, normal spinal tap (just very slightly elevated protein level), abnormal lactate (too low with exercise), slightly abnormal EMG back in January (small motor unit potentials, slight increased turns).
Thanks a bunch!
-Rob