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Neurology (Expert Forum)
Myoclonus with hypotension/pre-syncope/ataxia
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Myoclonus with hypotension/pre-syncope/ataxia
by mikesgirl, Jun 09, 2002 12:00AM
Mike is a 38yo, 6'4", 186 lb man who has been wheelchair-bound for four months with myoclonus. Three years ago symptoms began with mild dizziness on waking in the morning and very mild Parkinson-type tremors that resolved quickly. A tumor was discovered on the left side of his neck that was excised, but that had no effect on his symptoms. History is sketchy here, but symptoms have diminished and reccured twice over the last three years. In late-Jan '02, myoclonus and severe balance disturbance occurred with what seemed to be flu.Testing was negative for virtually everything - MRI, CT, blood chems, CBC, cardiac ultrasound - all within normal parameters with one or two mild variations (MCV, PLT, LYMPH). Carotid - velocities are 71 right, 57 left, patent and smooth. BP averages 90/60 and pulse at 50-60 normally. Orthostatic hypotension constantly exacerbates the ataxia and myoclonus. Complaints of room-spinning and whiting out type blindness. Lying on his left side produces intense symptoms (side where velocity is lower). Horizontal nystagmus as the only other (measurable)neurological sign. Confusion on changing posture along with proprioception disturbance and increased shaking. No known history of anoxia, trauma. The question, at this point is, could all of these symptoms be induced only by the hypotension? He sees a very busy neurologist who has not ordered SPECT, MRangiogram, cerebral oximetry or any other measure of his cerebral blood flow/perfusion. Should we insist on these tests? Started on butcher's broom and caffeine today. What else can we do?
Doctor's Answer
by CCF-Neuro-M.D.-JT, Jun 11, 2002 12:00AM
His symptoms are concerning for a neurodegenerative syndrome. There is a condition called MSA or multisystem atrophy that is thought to be a parkinsonism (NOT parkinson's like Michael J Fox/Muhammad ALi, but in the family of parkinson's disease). This is characterized by profound orthostasis and autonomic dysfunction (bowel/bladder problems, swallowing difficulty), rigidity and other parkinsonian features like falling and gait instability, and cerebellar signs such as ataxia and nystagmus. It is a clinical diagnosis that cannot be made with a simple blood test or imaging study (most things will be normal). A careful history and exam are necessary.
A second possibility is Creuzfeldt Jakob disease which is a rapidly progressive dementia that can be accompanied by myoclonus. Other neurological deficits can be present also.
There are other diseases that this can represent depending on the whole clinical history and examination findings, but the symptoms you report here sound worrisome. Strongly consider a second opinion at a major clinical or academic center with a movment disorders section. Best of luck.
A second possibility is Creuzfeldt Jakob disease which is a rapidly progressive dementia that can be accompanied by myoclonus. Other neurological deficits can be present also.
There are other diseases that this can represent depending on the whole clinical history and examination findings, but the symptoms you report here sound worrisome. Strongly consider a second opinion at a major clinical or academic center with a movment disorders section. Best of luck.
10/99 Tumor was a 2.2cm benign lesion of the left parotid resulting in total parotidectomy – scar tissue from this procedure noted on recent MRI
Complaint back in 1999 was of a “head rush” – precisely the same term he currently uses to describe his symptoms
1999 Toxicology panels negative for heavy metals and organohydrocarbons
Femoral bruits noted in 2000
6/’99 Flu shot within a month prior to onset of symptoms
Childhood heart murmer
3/2002 Spinal tap – negative
2002 Extreme sensitivity to cool temps will bring on violent shivering episodes that are almost seizure-like
Dramatic weight loss and fluctuations over the course of these three years
Currently extremely slow improvement of myoclonus – movement is getting smoother and more accurate
Venous pooling in all extremities apparent on observation
I have had many of the same things happening. It is frusturating when there is not a clear diagnosis and that many of these neurological disorders can take on the same symptoms. I have been tested for many many things and like the doctor says alot of them have no black and white testing it is just my last dr. visit that he mentioned OPCA. I have not had the tilt table testing. Like so much of my tests it will probably give not clear answers.
My b.p. flucuates but can go low like 73/41 and pulse like 41. That alone makes one dizzy and unclear thinking. The confusion and thinking difficutlies can be scary for my family.
I have had bouts of bad balance that do not come completely back.
Ataxia, swallow difficulties, speech troubles, nysagimus, vision problems. My doctor has not labeled them myoclonus but I get times when I have jerking, and truncal tremors.
There is much more that goes along too.
Just know that you are not alone even though this can all be very difficult to deal with. I have read also that many doctors will not give a definate diagnosis of MSA.
Hang in there and keep the faith!
AMO
I have not been have a definite dignosis of anything yet because I do not fit in the right molds, so what else can be new!
The OPCA is one of the MSA that they catagorize. Sorry but I can not spell the whole name. As I have read about the different types they can over lap some and they used the 3 main labels depending on what area is more prominate.
As far as the b.p. and such I have not had any professional testing for it yet. He did mention to have a tilt table test done. But I have been monitoring it on my own for a few weeks ever since in the e.r. my b.p. was very high. There really isn't too much rhyme or reason to what happens although when ever I lay on my right side it drops. My breathing is very slowed also, my tempurature has a hard time regulating too. Although I haven't had the down to the bone chills lately. As far as exercise it all depends on the day and what I have done leading into then. It mostly consitsts of moving about the house and through the grocery store. I can get my pulse up to about 70+ (usually about 60)doing house workfor a 15 minute stretch and then get exausted and it is when I rest back that it takes a dive, I have taken it a few times that it was 45 and lower. I have bouts where for a few days at a time all I do is rest and mainly sleep. During those days my mind is way off and I have a very difficult time thinking. I get that freezing of movement and thinking like some of the sites read about. I am dizzy 24/7, my balance is difficult to keep. I have had p.t. a few times for 3 months at a time. Unfortunatly I do not see much if any improvement. Some days it seems to be to taxing when they up the difficulty some to where I think it does more harm than good.
I hope today is a good day for you and Mike,
take care and keep the faith!
AMO
AMO--have you had an MRI done?? I had he same syptomes as you and Mike--have I should say--and they have just found a huge disc prolapse in C5-C6 causing compression on my spinal cord. Just wondering if that is probably the reason for all these horrible feelings-if perhaps the vertabral vein is involved, and if you could be having the same problem. It has taken a year to diagnose me so far..
I have had extensive testing. I have had i think 9 brain mri, two cervical mri, one mra, and a brain biopsy which was inconclusive except to say my cells are not right, (well duh! LOL) The c-spine