Hi,

My father recently was afflicted by rapidly progressing peripheral neuropathy. Our healthcare provider has failed to provide anything useful, and it continues to progress as far as we can tell. Any help or input from people who know a lot about the subject would be greatly appreciated.

Male/Age: 55/Caucasian (English/Irish/Scottish/German mutt)/Blonde/Blue/5'6" ~210~ lbs, Diet heavy on meats and starch, but all around not tooo bad

What happened:

He was recently afflicted with what appeared to be a 24hr stomach flu (some vomiting.) A week later he began to notice weakness in his arm, an inability to lift it far up, and muscle pain, especially while sleeping, with tingling and partial numbness to follow. He described the feeling as being punched for the entire night. This feeling then started in his legs, then affecting his groin area as well. His walking became less steady.

Key Affected Areas: Arms (especially right arm should and elbows,) waist/groin, and both legs

3 days after onset he was admitted to the hospital having some wobble in his walk. After 5 (total, +2 from the hospital) he came out barely able to walk, worse all around. Pale in the days before hospitalization (but regained colour during/after,) exhausted by small attempts at movement, sweating.

The tests that were done in hospital are as follows:

Numerous Blood Tests: Doctors reported nothing on these, not sure what all was tested

HSV Test: negative, though there was possible exposure around 3 weeks prior to this episode to a dormant individual

EMG (Electromyography Supposedly ruled out Gullain Barre, reported no response in the deltoid muscle.

Failed 3 attempts at lumbar puncture in a row, could not get spinal fluid they said. He was scheduled to have an anesthesiologist do a higher puncture, but that was cancelled after the EMG.

MRI: Nothing to report

A doctor said they thought it was HNNP (Hereditary Nerve Pressure Palsy,) but there was no mention of this diagnosis on his discharge papers, they simply said all tests were inconclusive.

I wish I had the actual information, but I only have what comes down second hand from doctors.

Other factors:

Around 2008, my father and I both had a virus that caused arm pain. It was just some weird annoying virus to me, but for him, his left arm became quite weak and hard to lift, it took around 1 to 1.5 years to fully recover if I recall. He says it felt about the same as this, but without pain, tingling, or numbness, just inability to control the muscle. Interestingly, this is the arm he can still use now with decent functionality, his right arm is now barely usable, ie he has to move the arm with the other to a location, but then can seemingly use his hand from that position well enough.

His mother was a Polio survivor with a paralyzed arm.

Had some positional vertigo issues the week before, supposedly due to dislodged calcium in the inner ear, he still believes this to be correct and unrelated.

The rapid progression is quite scary, and I fear it will be too late to really help him (if there's anything to be done) by the time we can cut through all the red tape of the HMO. Definitely going to be looking to get a Peripheral Neuropathy expert's opinion ASAP.

The whole thing still sort of sounds like Guillain Barre to me, his spinal fluid was never tested, it doesn't seem like there are focused pressure points as with HNNP, though I'm no expert. So many causes have nearly identical symptoms, figuring out what's what is extremely difficult.

Thanks for reading
--Sam

--my first thought with a rapidly ascending neuromuscular disturbance would be Guillian Barre syndrome, or one of its variants, including a possible attach or re-attack of chronic inflammatory demyelinating polyneuropathy, an autoimmune waxing/waning entity that is related to Guillain Barre (it's often considered, the "slower", more chronic form of that condition).

The recent prior history of infection also supports that idea, as a prior infection can often set off the immune molecular mimicry that leads to the neurological attack and symptoms.

Don't know why the EMG results should preclude the lumbar puncture in a case like this either--especially if the EMG showed nerve compromise.

I'm assuming MRI's were done to rule out obvious spinal cord issues--was one done of the brain as well? Often, in cases like this, another possibility is MS or another central nervous system demyelinating disease, such as acute disseminated encephalomyelitis (ADEM--these are distinct from demyelinating conditions of peripheral nerve such as Guillain Barre).

It really sounds like you need to get him to a (better) teaching hospital. The problem now, though, is that with Guillain Barre and similar syndromes the initial attack may be over and there may be less evidence to find, even if there are residual affects; has he improved?

Welcome to NeuroTalk:

There is a bacterium that comes from poorly cooked chicken.
Campylobacter is the name. It results in an autoimmune PN in some people. Treatment is IVIG. This condition was illustrated on the Discovery series, Mystery diagnosis. You might be able to find the episode online. PN follows a bout of stomach "flu" therefore from this food borne infection.

Also, you are intuitively very perceptive. Vaccines can also cause a GBS in sensitive people.

The biggest problem I see is finding a doctor who will understand this progression and at least try the IVIG for your father.

Sometimes also the drugs that people take, can cause problems. What does your father take, can you tell us.

Even blood pressure drugs are not 100% safe. I am getting over a drug induced lupus from lisinopril, and ACE inhibitor.
This type of lupus does not typically raise ANA levels, so when tested this might be "normal".
Here is an article on the subject:
http://www.lupus.org/webmodules/weba...=377&zoneid=17

So people with drug induced lupus may fall thru the cracks of traditional medicine!

I would get testing done for B12 and Vitamin D. This really is the beginning for all PN type situations. Both of these nutrients can commonly be low in many people. Get the numbers of the B12 test, because doctors will accept LOWS from outdated lab ranges still as normal. So even getting testing EVALUATIONS may fail. I find this pretty disturbing, but it happens all the time, so patients have to take a proactive stance in their treatments and testings where PN is concerned.
This is our B12 thread:
http://neurotalk.psychcentral.com/thread85103.html
You will want to have at least 400pg/ml in US testing units for the lowest normal result. Most of us here keep our levels at 1000 or above.

When there is an injury to the nerves, and healing elements are not present, then the healing becomes impaired and the symptoms linger. Damage can be to the myelin that insulates the axons of the nerves, or to the mitochondria in the cell bodies. Sometimes the injury is to the dorsal ganglia in the spine. B12 is a cofactor in remyelination. So if low, then healing cannot take place. If your father uses statins for cholesterol...these drugs impair normal remyelination as a side effect, and can cause PN. One is not likely to heal therefore when these drugs are continually taken.

Here is a site concerning HNPP which you mentioned:

http://www.hnpp.org/

It is the opposite of CMT1A. HNPP has a deletion and CMT has a duplication. Just thought that you might to explore the site. Hope you find an answer.

There is a genetic test for HNPP, have they done that? Your father's elbow problems sound a lot like mine, but I simply have pinched nerves in addition to hereditary neuropathy. HNPP comes and goes, my symptoms are pretty much permanent. There may be more than one thing going on with the diversity of symptoms you have described. It must be very frustrating to have to wait for more aggressive diagnostics, and you are right, time may be essential to recovery.
I hope that you get more satisfactory answers soon. The questions that the others have asked are an important place to start. Finding a cause for PN requires that you be a detective, and even the most seemingly insignificant event or medication can be important. Toxins, antibiotics, cholesterol medication, tick bites, alcohol, ruptured discs, vitamin deficiencies, and many other things can all be causes, so keep meticulous notes. Doctors tend not to want to assign blame to any medical treatment, but unfortunately many of them do carry a certain risk, which is why so much of the research has to fall on the patient and his family.

He has supposedly slightly improved today, but I think it's most likely the comfort and quiet of home as opposed the the smorgasbord of opiates, tv watching roommates, and interns jabbing his spine with needles at the hospital.

Thanks for the info on CIPD, sounds like it could be a possibility, I'll definitely research it more.

I would assume an MRI was done to check for spinal chord injury and brain tumor, but I don't know what images were actually taken and what was checked. MS was "ruled out" in that it was quickly diagnoses as "Peripheral Neuropathy." How that would be diagnostically separated by a doctor in his office from central neuropathy, I do not know.

No genetic test for HNPP was done.

I'm going to try and get HMO to get him a referral to the UCSF Neuropathy Center. Prolly gonna have to pull out the pliers and blow torch to get them to do it.

@mrsD He's wasn't taking medication of any kind at all, nor has he for basically his entire life. I completely agree I need to get my hands on his B12 counts, regardless if this has been caused by B12 deficiency or not, making sure his body is populated with the necessary materials for neurological health is essential. AFAIK he didn't eat any chicken prior to his stomach "flu," but I'll check. There was potentially some under cooked chicken like a ~week~ prior to his stomach flu. At any rate he did not even slightly suspect the chicken due to the the latency and food poisoning usually being quite rapid after ingestion.

@Susanne C. I guess what I meant with his elbows and shoulders is they seem to be the joints about which movement is difficult, like he "sets" his elbow to the right angle and can leave it there, but not really move it, not much range in the shoulder to lift either. Does this sort of rapid, progressive, and broad onset strike you as odd for HNPP? There's a bit more evidence related to this that I'm posting

Four other things I should mention:

He had a sort of episode with his wrist I want to say approximately a year ago that took about 5-6mo to go away. He had some pretty intense pain when applying pressure with his palm, or rotating it certain direction. The exact distinction I don't know. He said it felt almost like a bone fracture, but it could have been neural.

When he was 20 (~35 years ago,) he went on a backpacking trip, and had a strap too tight on his left shoulder, apparently this led to some extreme weakness and numbness following the trip, from which he recovered, but he said he always felt like that arm was weaker (also potentially double jointed.) This is the same arm that had the episode in 2008, but is now mostly functional (left.)

Keratosis on the skin (for some time now) but apparently almost all 50+ white people do.

He had a dental implant (titanium post deal) about a year ago (with bovine bone graft.)



Again thank you all very much. I'm quite touched by the number of detailed and thoughtful responses.

--Sam

http://www.hnpp.org/varieties.htm

Scroll down to "Progression of HNPP". As you can see it can be slow for some and rapid for others. Not saying he has this but it might be worth having DNA testing for it. Actually, the whole site on HNPP is very interesting.

Not saying at all that he has this but it would be worth checking out. And to have a neurologist who knows about this.

Nor was I saying that he does not have it. While it does say that it can be rapid and some of it sounds right, none of this has been "activity based" at all as far as I can tell. It just seems strange that it would hit with this, to a large degree, body wide wombo combo all at the same time.

He is feeling somewhat better today, and he said the tingling in his abdomen is better.

The genetic test would definitely be worth having done if we can make the HMO do it. More information is never bad. And he could potentially have a mild case of HNPP with something else interacting heavily.

the bacteria mrsd mentioned normally takes 2/5 days for symptoms, but may take as long as ten.

Irelia, lots of information in the thread. I am glad you are researching for your father. I hope you can get to a really good doctor that is more helpful. I know your research will help for sure.