SEVERE atrophy/pain in left quad/knee area, been struggling for 5 years.

[Emil77]

My info: 25 year old male, 5-10, 215lbs.
Problem: SEVERE atrophy in left quadriceps/Severe knee pain.
<Due to lack of space I had to make this short>
History: June of 1997 I had a patellar-tendon transfer knee surgery. Medical name is Roux-Goldwaith procedure. After the surgery, I start the physical therapy, etc. After 3 months of physical therapy there is no new development in quadriceps muscle. The orthopedic doctor says "It will take time, continue to do physical therapy."
2 years go by, again no development in muscle. I go to a different orthopedic doctor. He does x-rays, says "everything is ok." He sends me to a Neurologist for an EMG test. Results are "normal."
Jan 02-Develop major pain again in knee. New orthoped does MRI/Xrays, everything normal. Says there is pressure in kneecap, preventing developement of muscle. Performs surgery to relieve pressure. I go to physical therapy. Again, NO muscle growth. July 16, 2002, I am sent to pain doctor. He performs a Left lumbar sympathetic nerve block. Again, I go to therapy and NO results. Pain is still present in the kneecap, that whole area hurts. November 6, 2002-New neurologist, EMG/NCV tests are again mormal. MRI of brain/spine to rule out ALS. Dec 4, results are given. Says there is no evidence of ALS. In 2 weeks,I am going to UCLA medical center for another opinion. I am at the end of my rope with the pain. I can not run, go up stairs normally.
Pain is always there. I had a whole 3 page post written with doctors quotes but there is not enough space to write it all. I used to be very active. Any ideas?

[CCF-Neuro-M.D.-JT]

I know your space is limited for the post, but more information would be helpful such as the circumstances around the surgery in 1997(was it for ACL reconstruction, was there trauma involved, was there direct injury to the quads, what complications there were, etc...). There appear to be 3 issues in your post and I'll try to address them that way: 1)atrophy of the quads 2)severe pain 3)question of ALS.

1.According to my orthopedic colleague, you shouldn't have atrophy in the quads if it was just a routine ACL reconstruction/repair which is what patellar-tendon transfers are usually done for, unless there are other circumstances such as direct trauma to the quads that was not stated above.  And it's surprising that the EMG is completely normal if there is severe atrophy as you report, unless they did not sample the quads. Unfortunately, the details of this procedure are out of the neurologic arena and would best be answered by your orthopedist.

2.Again, the problem appears to be ortho-related rather than neurologic. Steroid injections directly into the knee may be of help rather than sympathetic nerve blocks. Neurontin has also helped patients with nerve type pain. A pain management specialist may have more ideas.

3.The symptoms do not sound like ALS as they appear to be localized to the knee and have been for 5 years. Also, the normal EMG helps make ALS an unlikely possibility.

If you would like to list more details regarding the quadriceps atrophy in a comment rather than a new post, I will be happy to answer on this string. Sorry for the wait.

[Kit1]

Pain lasting this long with no apparent basis in tissue damage could be a condition called RSD (reflex sympathetic dystrophy), also called CRPS (complex regional pain syndrome). That might be why you were sent to the pain doctor-- lumbar sympathetic blocks can sometimes relieve this pain, but not always (and often not permanently). (Did it help?)

The pain is caused by an abnormal feedback loop between your spine and the nerves in your extremity so that you feel pain in the extremity. You may also have muscle atrophy, poor motor control of the limb, and various physical changes like swelling, discoloration, temperature changes, and unusual hair growth. (Not all of these may occur).

If you go to read literature about RSD, you will be distressed because doctors sometimes write that it is a psychogenic condition or that it is more common in people with "dependent" personalities. DON'T BELIEVE IT. It can happen to anyone, even young athletes who want nothing better than to run again.

There is hope. In many patients, the pain fades with time-- although to be realistic, it may never completely go away. You will learn your own strategies for getting what exercise you can. You can also try acupuncture, heat or cold treatments, stretching, massage, etc.

I'm not saying this is what you have. But you might look into it. Take care, and please keep trying.

[Emil77]

Hi Kit,

Unfortunately with the limited space we have, I was not able to type out my full report. :-)

Yes, the doctors have mentioned it could be RSD or CRPS. I hate that diagnosis. Why should I have it?
The lumbar blocked helped, but now I am getting that itchy feeling again. Your right, I do have some color change, swelling of the knee and poor motor control. Sometimes my knee gives out and I fall, I can't raise my leg straight up, or extend it from a sitting position. The pain is INTENSE when I do that!!! I am thinking about trying Prolo-Therapy. Ever heard of it?

I have an appt on the 20th to see a specialist at the U.C.L.A. Medical Center.

Thanks,
Emil

[Annika]

Dear Emil,

Before trying Prolo-therapy, do some serious research.  The little that I do know about this treatment, which involves the injection of caustic/irritating substances around the spine, sounds extremely concerning.  This topic comes up a lot among patients with pain of spinal origin.  In general, inflammation in and around the spine/spinal nerves/spinal cord is more often linked with causing these chronic pain syndromes than it is with curing them.  Based on the substances that are injected, there is a  risk that such a treatment might just make a patient worse.  

Unfortunately, one has to be vigilant in avoiding further harm from poorly studied treatments.  The treatment of spinal-origin pain has a long history of previously-tried invasive procedures, all well-meant, that only served to injure the spine further.  I don't mean there will never be a successful treatment for these disorders, but rather that extreme caution is necessary.  The problem is that sometimes the bad effects of treatments take a while to show up, so careful research is essential in weeding out the potentially harmful therapies.  Those kinds of studies, to my knowledge, have not been done with Prolo-Therapy.  On the other hand, there is plenty of research on what happens when other pro-inflammatory foreign substances are injected around the spine.  One of the big risks is scarring in the various spaces around the nervous system structures.  This kind of scarring can have serious consequences, and be difficult to treat once it has formed.  

On the other hand, there is a lot of information about how to manage the various spinal-origin pain disorders, if that's what this ends up being.  I'd be happy to write more about this later.

Good luck,
Annika

[Emil77]

Hi,

Is it normal to wait 3 days for a response from a doctor? I see other questions that have a reponse the next day.


Thanks,
Emil

[Emil77]

Wow, so I am not going to get a response? Not even a reponse from a doctor saying "We can't help you?"

I just wanted to say thank you to the 2 people who did take the time to respond. So.....Thank You!.

Emil

[Annika]

Hi Emil,

I don't know what's going on.  I'm sure it's not the nature of your question, but that somehow your question's been missed (one doctor thought another on had already responded... that seems to happen occasionally). I don't know what to do about it except wait another few days, and then maybe post a note saying your question wasn't answered.

Kirsten

[Emil77]

Thanks for the tips Kirsten,

I guess I am going to have to get up at 5 am again to post another question. :-)

Take Care,
Emil

[Emil77]

Hi Doc,

Thanks for taking the time to respond. I am going to copy and paste my whole 3 page thing here. It will turn into a 3 or 4 comment reply by me.
Before I do, a little more history. I had no trauma that caused me to get the surgery. It just happened, one day I started having massive knee pains. I went to the doctor, and he said I have a dislocating knee cap problem. According to him, the patellar tendons in each of my knees is crooked. He "fixed" the left one, I have not touched the right knee. I have pain in the right knee, but nothing to the extent of the left. One more note of interest, my left thigh diameter in the middle of the thigh is 57cm. My right thigh, in the middle of the thigh is 63cm!

Thanks,
Emil

[Emil77]

History: June of 1997 I had a patellar-tendon transfer knee surgery. Medical name is Roux-Goldwaith procedure. After the surgery, I start the physical therapy, etc. After 3 months of physical therapy there is no new development in quadriceps muscle. The orthopedic doctor says "It will take time, continue to do physical therapy."
2 years go by, again no development in muscle. I go to a different orthopedic doctor. He does x-rays, says "everything is ok." He sends me to a Neurologist for an EMG test. The quote from the EMG paper says "22 year old male with subsequent atrophy of left quadriceps. The impressions (results) are No electro diagnostic evidence of left femoral neuropathy. EMG of muscles of left femoral innervation demonstrated no acute or chronic denervation. There was no evidence of generalized or regional nerve disorder. Please bear in mind that disuse atrophy yields normal results on needle EMG."
So, the new orthopedic doctor says "nothing is wrong, continue to do exercises.
<A brief step back in time: After the therapy sessions ended, I continued, and still continue to this day to do my work outs. Up to 1 year ago I was able to leg press 500lbs! BUT the muscle in my left quad, NEVER NEVER ever gets sore. It does not develop. BEFORE the knee surgery, I was an active runner. I ran 5 miles a day. I played 1 year of high school football in the 9th grade.>
Fast forward to Jan of 2002. I am in the attic putting down plywood for a floor. As a result of my constant bending, my left knee swells up.My mom finds another orthopedic doctor. We go to him; he says "The nerve that controls the muscle growth in your quad has pressure on it. That’s why it isn't allowing muscle growth." He orders MRI's and X-rays of my left and right legs. Everything on the MRI's is "normal" according to him. He says next step is to have surgery to relieve the pressure.  March 18, 2002 I have knee surgery. Quotes from my medical records. Preoperative diagnosis: Patellofemoral pain with patellar hypertension.  Postoperative diagnosis: Patellofemoral pain with patellar hypertension and synovitis. Operation performed: 1) Patellar arthoplasty. 2) Synovectomy."

[Emil77]

Basically, while I was awake, they introduced pressure into my kneecap, causing me tremendous pain. So the patellar test was determined to be positive and I was put to sleep. Then I had a W shaped pattern of 4 cracks through 2 holes in the patellar to relieve the pressure. After the surgery, I go to therapy, again NO development of muscle. During therapy, I had an NMES machine, 2 electric pads put on my quad to stimulate the muscle. That didn’t work either.
July 16, 2002, the orthopedic doctor basically gives up and sends me to a doctor for pain management. This new doctor says again, the nerve is somehow not working, thus no muscle is developing. He orders MRI’s of my spine and knee again. Again the results are “normal”; there is no pressure on the nerve that controls muscle development. He says next step is to do a “Left lumbar sympathetic nerve block.” Again quotes from my medical records. “Preoperative diagnosis: Complex regional pain syndrome, left lower extremity. Postoperative diagnosis: Complex regional pain syndrome, left lower extremity. Operation performed: Left lumbar sympathetic nerve block.
So after this procedure, again I do therapy. Again, there is NO growth of muscle.
The pain doctor sends me to another neurologist.
November 6, 2002. Some quotes from the neurologist’s medical records “Deep tendon reflexes have hyperreflexia throughout even at the left patellar jerks, he had hyperreflexia. There are adductor signs. Toes were going down bilaterally. He has some patchy distribution of sensory changes on the left side around the kneecap area and the lateral aspect of the lower thigh and even at the mid-shin area as well, but does not appear to be in any dermatome distribution. He also has a mild degree of sensory changes in the kneecap area in the right lower extremity in patchy distribution. The etiology is unclear at this point. I am impressed by his hyperreflexia despite almost non-existing quadriceps muscle. Based on such information, motor neuron disease process or upper motor neuron disease needs to be ruled out as well as the peripheral entrapment neuropathy.”
So he says I have to do another EMG test, which is performed on November 13, 2002. <The summary of the EMG test is as follows: This is an abnormal EMG/NCV study. NCV study was completely normal in left lower extremity except absent f-waves. In the absence of other abnormality its clinical significance is uncertain. Even in normal subjects with active physical activity such absence can be seen. In EMG study, quadriceps muscles show no significant voluntary activity without any resting insertional abnormal findings suggestive of denervation. Such findings may suggest possible femoral neuropathy but absence of denervative change certainly is a typical feature. Central pathology causing muscle atrophy should be ruled out. In comparison to previous EMG/NCV study done 3 years ago, only different finding in this study is absence of muscle activation in quadriceps muscle. Such observation may indicate that this could be very slowly progressive monomelic amyotrophy. >
So, after that he sends me to get MRI’s of my brain and spine. The MRI’s were done with and without contrast. He wanted to rule out Lou Gherigh’s disease. So the results of the MRI’s are normal according to him. Now I am waiting for word from a doctor at U.C.L.A. Medical center. My neurologist wants me to see him to make sure it’s not some disease.

What is I am looking for? Well this pain and atrophy has in a sense ruined my life. I had finished firefighter classes and obtained my EMT certification. But this problem has shattered those dreams.
I can not run; can not go up stairs normally, I can not stay on my feet for more than an hour. If I do, I have SEVERE pain at night. My kneecap is the place that hurts, it’s very tender. If my 2 year old cousin accidentally puts pressure on it, I experience major pain.

Any idea what could be wrong with me? It seems every doctor I go to can’t figure out my problem. My neurologist is saying I might have Complex regional pain syndrome. So am I supposed to live with that? My social life has gone down hill; I am in to much pain to go out. I have become irritable; the smallest things make me mad. I don’t want to live like this. Have you heard of prolo therapy? Do you recommend I try that? Is there ANYTHING you recommend I do, or do you have an idea of what might be wrong with me?

*****I have been to 3 orthopedic doctors, they all say it is not an orthopedic problem. I don't know what to do anymore***

Thank You,
Emil

[Annika]

Dear Emil,

Your story sounds extremely frustrating.  

I just wanted to say that in interpreting the EMG, it's extremely important to understand that the EMG will only look at damage to the lower motor neuron.  This is the neuron type that makes up the peripheral nerves - the nerves that leave the spinal cord and travel to the actual muscles themselves.  

The other part of the nerve chain is the "upper motor neuron", which carries the message to move from your brain down through the spinal cord.  The EMG cannot "see" these nerves, so if there is damage anywhere along their path in the spinal cord, the EMG will still be normal.  Patients with weakness from MS, for example, which affects the brain and spinal cord, tend to have normal EMG's.  In this way, it is perfectly possible to have nerve damage affecting motor function while still having a normal EMG.  

When there's damage to the upper motor neurons, another classic physical change is that the reflexes in that part of the body become increased - this is what "hyperreflexia" means.  There will generally be atrophy, although sometimes not as dramatic as when there's damage to the lower motor neuron.  Usually the patient will also notice that the affected muscle is stiffer, and that the tendons seem wound tighter.

I don't mean to say that I think you have damage to the upper motor neurons - I have no idea, but since this issue has come up in your care, wanted to pass on some basic information.  Complex regional pain syndrome, although it's poorly understood and seems to have many causes, is found with a fair frequency among people with known damage to the fibers within the spinal cord (from illness, trauma).  Again, you may not have this syndrome.  This is just another piece of information about the diagnoses that have been brought up.  A negative MRI of the spine cannot rule out all dysfunction within the spinal cord, although it can rule out many of the scary things (like a tumor, which is what I had), especially if done with gadolinium contrast.  Again, I'm also puzzled by your story and am not trying to say this is where the problem lies.  I just think it's important to understand what the tests are able to show, and what they can't.

The doctors on this site usually don't write back about follow-up questions - I don't know why this is, but assume it's the basic policy of the site (I've never seen a follow-up questions answered for anyone).  Just so you don't feel ignored if you don't get a response.  I completely understand how stuck you feel, and would feel the same way myself.   One of the hardest things is when you have no real diagnosis to explain why things are happening the way they are.  I wish you the best of luck in getting more answers to your questions, and in finding something to treat the pain and muscle dysfunction.  

Sincerely,
Annnika

[Emil77]

Hi Annika!

Thanks for your support. It's nice when somebody understands. Some of the doctors I went to, sort of hinted that I am not doing the physical therapy well enough! I was thinking to myself "OH ya, I don't do the physical therapy, so I can suffer more pain and pay more doctor bills!" Ugh, some people.

I posted all that stuff cause the doctor mentioned "If you would like to list more details regarding the quadriceps atrophy in a comment rather than a new post, I will be happy to answer on this string. Sorry for the wait."

The upper neuron motors part you mentioned is interesting, I will be sure to bring that up when I see the doctor on the 20th.

Thanks again for your support,
Emil

[Emil77]

Opps, I forgot some parts.
Is there any way to fix "upper neuron motor damage?" Also, are there any tests that can be done to see the upper neuron damage?

Thanks,
Emil

[Annika]

Hi Emil,

I didn't see that note from the doctor... that sounds very helpful.  I hope he writes back.

I know exactly what you mean about the "not doing your physical therapy" comment.  It can be very frustrating.  I don't mean this in a "poor me" kind of way, but I think most people/doctors intuitively relate a patient's complaints to their own experiences of ordinary aches and pains, unless there's some clearly visible, dramatic problem, like a big wound.  When you've got some problem that lies outside the experience of the average person (as is often the case with neurologic/pain disorders), people really have a hard time correctly judging the extent of a patient's disability.  Only when it happens to you yourself does this really sink in.

I still don't mean to say that your problem is necessarily neurologic, since I'm hardly the expert on orthopedic issues.  I do have personal experience with muscle wasting in the quads from upper motor neuron damage (in my case, related to scarring from my spinal tumor removal).  Unfortunately, upper motor neuron damage tends not to reverse, once it has really settled in.  There's some controversy now about different studies that have shown regeneration in these nerves, but on the whole upper motor neurons regenerate much less than do peripheral nerves/lower motor neurons.  

Good luck working with your doctors on investigating this.  There are so many other factors in your history that many of the things I have told you about my own experience may turn out not to apply at all.  I'm still wondering about how this whole thing started for you.  One little bit of informatioon, which again may not have any bearing in your case, is that when I had the damage that affected the muscles I my quads, my kneecaps started tracking in a different way.  This was because the muscles in my thighs were imbalanced as a result of the weakness.  I wear knee braces now, to prevent the grinding on the joints that happens due to my thigh weakness.  So sometimes an orthopedic problem can be found on physical exam that seems to explain the patient's pain or dysfunction, but it's actually secondary to a whole other issue, not the source of the problem itself.  

Sincerely,
Annika

[CCF-Neuro-M.D.-JT]

Although I read your post in detail, I'm still not exactly sure why you have quads atrophy unless it really is just disuse atrophy from such severe chronic knee pain. Still doesn't sound like ALS, despite the brisk reflexes as there haven't been any other symptoms/signs in the last 5 years. I suppose if you want to pursue this further and your docs suspect a rare or uncommon cause of the atrophy (depending on your full clinical history and exam), a muscle biopsy could be he helpful. But again, this should be taken into clinical context.

As for the prolotherapy, I found some research studies in the alternative medicine literature (nothing in the mainstream journals that are held in high regard like the New England Journal of Medicine) demonstrating some improvement with regard to pain, joint stability, and flexion range of movement with the injections of growth factors/stimulants.  One study looked at 38 knees with severe arthritis that were injected with prolotherapy.  After 6 injections over a one year period, there was a 44%  decrease in pain and 85% decrease in knee buckling. No significant side effects/complications were reported. But as I am personally unfamiliar with this procedure and was unable to find any information in a well respected mainstream journal, I'm not sure I can recommend it. But I haven't found anything to say that it causes significant harm or permanent damage either. Perhaps, talk to an orthopedist or bring it up at your next appointment at UCLA.

Finally, I did run across an article from the Plastic surgery department from Johns Hopkins (a very well respected institution) that talked about "selective denervation" of the knee for patients who had chronic debilitating knee pain due to prior surgery or intervention. After their procedure, 77% patients (13 total) had good to excellent results.  The primary author of the journal was Dr. Nahabedian, but that's as far as I know. Best of luck to you.

[Emil77]

Thank you so much for taking the time to respond again. I really appreciate it. It's amazing how nobody can figure out what's wrong with me! I hope the appointment at U.C.L.A. will do something good for me.

Thanks again, and happy holidays.

Sincerely,
Emil

[Emil77]

Well an update from me.
Today I went to U.C.L.A. Medical Center. After meeting with a "specialist' for an hour, she said that she can not explain what is wrong with me. She has no idea. Sigh, once again I am back to square one.
Oh well.

Thanks for your help,
Emil

[stormcloudfyre]

Hi,
having had my right patella removed,   Theonly way I an get any  Quad developement is to  CYCLE  20 to 100  km ( up to 60  miles ) non stop  but being an avid cyclist this is an easy  ride done in 1/2 to 3 hours . Its inrte nsity that builds the muscle. not just sitting and  peddling  easy .
I do lots of leg preses in the Gym  And Work up to 450 Pounds  on a  Global gym  machine !
  Physio c an be a wimpy situation and building muscle is  A task thats not wimpy. you may hae tro  put up with some pain initially but that will  go with  continuous  work ! OVer a few months.   Ultra sound and other  physio techniques  are  a  waste of time.
c
Try some  heavy duty effort!  


Peter

[Emil77]

Ummm, maybe you didn't read the whole post. I have had this problem for 5, FIVE years, and gone through extensive physical therapy and work outs. I am not sitting at home, not working the muscle, hoping it magically comes back. So, your suggestion of heavy duty effort has a been there, done that response from me.

[To CCF Neuro MD From Pokey]

Wow, sure sounds like you have been through a lot.

Something you mentioned in your post about the size differences between the thighs - 6cm - quite a bit of difference. Have you been checked for a blood clot, or a compartment syndrome?

Did the Doctor at UCLA have anyone that she could refer you to? Maybe that would be a good re-starting place. I hope you find out what is going on soon. Take care.

[Emil77]

Hi Pokey,

None of the doctors in the past 5 years have mentioned a blood clot problem. I've never heard of compartment syndrome, what is it? The doc at UCLA basically said "I don't know whats wrong with you, wait a couple more months, then do another EMG."
There was a post here about major medical facilities. The doc mentioned a hospital in San Franscisco. I might go there.

Emil