recovery from GBS

[Ana Maria Martinez]

First of all I want to thank the organizers,doctors and sponsors that make possible this forum. It is a great help for ill people and their families which try to find some answers.

My father (soon 65 years old)got GBS last April. Although he did not need a ventilator, it is a quite severe case (he was totally paralised in less than two days), and according to the doctors it is an axonal type of GBS.

I had the opportunity to contact you last June and you were very doubtfull about being an axonal type of GB, since it is much rearer. At that time you wondered how the doctors knew about it. The only thing I know is that they made an EMG about 8 days after he got the illness and another 8 days after the first one. According to the doctors the EMG showed damage in the axons. He is still going to the neurologist every second month but they did not take any more tests but just to check the development of the motion.

He is going to physiotherapy every day and still he is not able to walk, but he can move a bit the legs and the arms, not the hands either but just a little bit the fingers. His improvement is very slow, but since April it has been a kind of steady improvement

What do you think about the diagnosis? Was the EMG done in the early stages of the illness? Is the EMG the right test to get such a diagnosis? If it has been a slow improvement so far, do you think he will be able to have an independent life again? Do you have any information about GB Supporting Groups in Spain?

Once more thank you very much for your help and your time.
With the best regards, Ana

[CCF Neuro[P] MD, RPS]

Dear Ana:

Again, I am sorry that your father's recovery has been extremely slow.  The fulminant GBS types with the hyueracute course of your father's is alittle unusual in that there was no mechanical ventillation required.  The EMG should have shown markedly reduced or absent CMAP's with distal supramaximal stimulation without conduction delay and absent SNAP's.  The subsequent EMG should have shown abundant fibrillation potentials on needle EMG.  And as you have already said, a primary axonal degeneration.  These are rare cases and unfortunately the recovery is somewhat limited, as the proportion of axons have undergone degeneration and regeneration of axons is at best, not complete even in the normal GBS types.  Each case is so independent prognostication is difficult.  

The usual GBS testing is the EMG and the CSF showing elevated protein, together with the clinical history and neurological exam.  There are some types that have a specific antibody association with it, GM1, that helps in the diagnosis and differentiating it from Lambert-Eaton syndrome (also the EMG would have been distinctively different).    So, the diagnosis sounds correct.

Unfortunately, I do not know about the support groups in Spain.  

I do hope that your father has a steady recovery.  

Sincerely,

CCF Neuro MD

[Kathy, Jonathan's mom]

I wondered if anyone has heard of an unusual form of GBS in which the limbs and lungs are not paralyzed. It is under consideration for my son by Mayo. We have spent 6 weeks there now and his condition is still deteriorating. He is hypotensive and bradycardic, has now lost 80lbs. in the past year, has dilated, unequal pupils, is anemic for the first time (low HGB & HCT, elevated WBCs and lymphocytes, changes in size and shape of cells on blood smear), has gastric and small bowel transit delay, chronic fatigue, spills massive urinary amino and now organic acids, catecholamine levels fluctuate constantly, no longer sweats, has OI and OH...MIBG scan states "probable pheochromocytoma in left adrenal region and liver is inhomogenous." One test keeps coming back stating renal tubulopathy or acute liver dysfunction however renal angiography is apparently normal and most liver functions are normal except slightly decreased ALT and AST, I think. The autonomic specialist said he thinks there is the ANS component and a whole other problem going on which is metabolic/endocrine (pituitary at top size). Endocrinologist mentioned possible unusual form of GBS. Don't mean to chime in on this post but just curious....thanks for any help.

[Kathy, Jonathan's mom]

I wondered if anyone has heard of an unusual form of GBS in which the limbs and lungs are not paralyzed. It is under consideration for my son by Mayo. We have spent 6 weeks there now and his condition is still deteriorating. He is hypotensive and bradycardic, has now lost 80lbs. in the past year, has dilated, unequal pupils, is anemic for the first time (low HGB & HCT, elevated WBCs and lymphocytes, changes in size and shape of cells on blood smear), has gastric and small bowel transit delay, chronic fatigue, spills massive urinary amino and now organic acids, catecholamine levels fluctuate constantly, no longer sweats, has OI and OH...MIBG scan states "probable pheochromocytoma in left adrenal region and liver is inhomogenous." One test keeps coming back stating renal tubulopathy or acute liver dysfunction however renal angiography is apparently normal and most liver functions are normal except slightly decreased ALT and AST, I think. The autonomic specialist said he thinks there is the ANS component and a whole other problem going on which is metabolic/endocrine (pituitary at top size). Endocrinologist mentioned possible unusual form of GBS. Don't mean to chime in on this post but just curious....temperature does not go over 94 degrees and temp or hands, lower arms, feet and lower legs is in the upper 60's to low 70 degrees. Thanks for any help.

[Christine- Doc RPS?]

Dear Doc RPS:
Please ponder Kathy's post.  Jonathan is deteriorating and comforting insight is welcomed with open arms.  This being a Guillain Barre thread, I would think it appropriate for her to post here.  Many thanks. - Christine

[Jan Bailey]

Dear Kristine and Cathy, I am not sure if the replies posted by us lay folks following the questions that are answered by the neurologist are even seen by that neurologist at Cleveland clinic...This forum is usually not run via a "thread"...just one or two questions posted and answered per day by the neuro covering many topics.  Usually the doctors only answer the original question, not replies to the question.  Try posting at 8 a.m. EST (I have posted successfully between 8 and 8:20 a.m....weekends are best).  I am sure you are terribly worried and looking for any feedback you can find.  Good luck.  JanB

[Christine]

Jan,
Slide down through the posts.  Doc RPS does respond.  I have been on this Forum since 98, way before my craniotomy.  If it wasn't for Doc RPS, I wouldn't have made it through it... or my radiosurgery last year 11/17/99 to tell you the truth.  I pray that he responds to Kathy.  He has before and it turned out to be the longest post in the history of this forum.  I apologize if I sound less than polite.  Forgive me.

Please Doc RPS.  We need your input and expertise.  Jonathan is in dire straits.  Thank you.  "He conquers who endures."

With Sincerity,
Christine  (CJ)  (CJenkins)

[Jan Bailey]

Christine...I was only trying to be helpful....sorry if I posted inaccurately...JanB

[Christine]

Jan,
I know you were.  No harm done.  Just help us pull for Doc RPS to respond.  Kathy needs help.  Thanks- Christine

[CCF Neuro[P] MD, RPS]

Dear Kathy:

Sorry that things haven't gotten better.  What did Marvin N. at University of Mass say about the amino acid profile.  In addition, what does Dr. Richard Kelly say about the urine organic acids?  What did Patterson say before he left the Mayo?  It sounds like your son has a defect in energy metabolism as we talked about before.  One might suspect a ANT defect in the mitochondria, but the urine organic acids would be suggestive as the amino acids in the serum (unusually high alanine to lactate ratio).  We do see dysautonomia in mitochondrial disease.  I really think that Dr. Richard Kelly at the Kennedy Krieger at Johns Hopkins is one of the very best metabolic people in the World.  It might be worthwhile to revisit the urine organic acids and get his imput.

Sincerely,

CCF Neuro MD

[Christine]

Dear Doc RPS:

Thank you so much for responding to this "call".  Jonathan needs your help and Kathy really needs the embracement.  How about a good quote here, doc?  How about 2 good quotes?

"The strong, sound mind is a mind that embraces all things, large and small."

and...

"In life there are meetings... which seem like fate."

With deep and sincere thanks,
Christine Jenkins

[Jonathan's mom]

Hi, Dr. RPS:

Thank you so much for taking the time and interest to respond.

I never heard from Dr. N. Dr. K said the organic acids indicated an unusual lack of salicylate metabolism." I don't know or nor have had any contact with Dr. Patterson. Is there anything more to be made of the changes in the CBC (i.e., low HGB and HCT with normal iron and iron binding capacity, elevated WBCs and lympocytes and leukocytes, and changes in the size and shape of the blood cells? Or the temperature problems in light of what I am told is a normal MRI of the brain x2? Should there not be a hypothalamic problem?

I am awaiting copies of everything from this last two week visit and will undertake your advice.

Can't thank you enough. You have never failed to steer us in a more accurate direction and I am seriously considering taking Jonathan to Cleveland Clinic if answers are not soon forthcoming. There is a Diagnostic Specialties Center at our corner here at home where they perform MRIs, CTs, etc., and it is affiliated with Cleveland Clinic. Interesting.

God bless.