Mother with multiple meningiomas

[Laurie]

My mother was diagnosed last year with multiple meningiomas.  I have 6 large cafe au lait spots (upper arms, 1 into armpit, and both buttocks with some freckling into groin).  I have been doing alot of research about NF 1 and 2, there is alot of conflicting information out there about the diagnosing/complications.  Physically, I don't have many problems but I do have intermittent tinnitus that has started over the last few years.  My question is do I need any imaging or genetic testing to see if I have NF2?  My mother is the only person in her family to have meningiomas, and I am the only child (I have 4 siblings) that have cafe au lait spots.  Thank you.

[CCF Neuro[P] MD, RPS]

Dear Laurie:



Neurofibromatosis 1 or 2 are genetic condtions.  Type 1 is associated with multiple cafe au lait spots (>6, of a certain diameter), axillary freckling, optic gliomas, changes in the sphenoid bone, Lisch nodules, and neural fibromas.  Type 2 is associated with bilateral cranial nerve 8 schwannomas, meningomas, and gliomas.  Each disease is inherited as a autosomal dominant and is located on a different chromosome, 17 for NF-1 and 22 for NF-2.  By what you are telling me, I doubt that you have NF-2.  However, a simple neuroimage of your brain will tell you.  But, if your mother doesn't have bilateral CN 8 schwannomas, then you likely do not have NF-2.  But, the only way to know for sure is to have imaging of you head.



There are genetic testing in a few labs and I think that Athenia Labs does these commerically,



Sincerely,



CCF Neuro MD

[Laurie]

Thank you very much for such a quick reply, it has alleviated my worries.

[Christine Jenkins]

To CCF Neuro (P) "Doc" RPS:



First, I'm elated that Laurie managed to secure a post dedicated to Neurofibromatosis.  There hasn't been one since NF and HRT.  Thank you Laurie... and please do what Doc RPS advised.  It's very important to know exactly what type you have so you can gear research to your personal cause.  I have NF-2 and dealing with it is a task dedicated to the quality of life.



Doc?  My whole life has been strange.  GBS.  HRT providfing food for tumor growth.  A huge craniotomy, and now... Stereotactic Radiosurgery, which is the subject for discussion.



I agreed to have radiosurgery after my neurosurgeon phoned me about taking care of the schwannoma located in the deep cavernous sinus.  It was done on 11/17/99.  Prior to that, I had a pretty extensive craniotomy done on 5/20/99 for the removal of schwannoma on the left temporal lobe, including the left orbital floor and optic nerve.  Impingement was excessive.  My recovery went fine.  Pre-surgery pain was more than 75%, post-surgery pain reduced to 25%.  I was on top of the world.  Almost free of pain I began the sweet task of reducing my pain meds.  I managed to kick Ritalin out of my life along with half of the Morphine Sulphate.



On 11/17, radiosurgery was performed... and I've been in pain ever since.  It's as if I stepped back in time and am once again fighting the Battle of Pain.  After years of waiting for a glorious "new day" in the land of semi-free torment, stereotactic rads burned the newly-formed "state" right back into the very ground from whence it sprung.  I don't want to go back up on the morphine.  Morpheus is mean, and so is its focusing sidekick Methylphenidate.



Are we so advanced as to believe we know it all?  A short life minus pain is a far-cry more humane than a long one constantly at war with itself.  NF-2 is just that.  There seems to be no middle ground to speak of on the road to elephantmanism.  We push on in disfiguring pain.  For myself?  I evaluate the outcome of plastic surgeries.  If schwannoma is left untreated between the skin and bone... it grows grotesque.  If left untreated within the cranium... well Doc, you answer that one.



Back to the deep cavernous blood reservoir.  When they zapped the schwannoma something happened and now I'm in pain all over again.  My questions are:



1.)  What gives here?

2.)  What is housed in and around the cavernous sinus that could have been sparked into action?

3.)  Could the surgery have done more harm than good?  I mean, considering the slow growth characteristics of schwannoma and my age (46)?

4.)  Might this all be a result of scar tissue from the craniotomy?  I have areas of facial numbness that are "waking up"?



I see my neurosurgeon soon... and I want to be ready for it.  And quite frankly Doc, your opinion means a lot.  I sure welome the advice.  Thanks for everything,  ("My strength is made perfect in weakness.")



Sincerely,



Christine Jenkins

[CCF Neuro[P] MD, RPS]

Dear Ms Jenkins:



Since your pain was resolving somewhat before the radiosurgery, I doubt that it is a return of your old pain.  The most likely thing is that the radiosurgery was the culprit.  In the cavernous sinsue runs the cranial nerves 3, 4 and 6 with two divisions of 5.  So one could get facial pain if cranial nerve 5 was injured by the radiosurgery.  Cranial nerve 3, 4, and 6 are eye muscle nerves mostly, but cranial nerve 3 carries some parasympathetic input to the iris and lens.  So, there is a possiblility of eye pain.  I would think that the pain would be transitory but how long would be an open question.  There is a theoretical chance for inflammatory changes to persist in the sinus area and could be long lasting and irritate the cranial nerves.  



I would ask your neurologist what did the immediate MRI show (if they did one) or if there will be another MRI of the area to view the aftermath of the surgery and the schwannoma resection.  Then, they should be able to tell you the extent of the inflammatory reaction.



I am not sure what to tell you about the pain.  I think that I would go the neurontin route first, before the morphine to see if it will control the pain.  But, you might need a tad to tide you over.



Sincerely,



CCF Neuro MD

[Christine Jenkins]

Dear CCF Neuro (P) "Doc", RPS:



Please accept my deepest gratitude for your swift response, although  I've been mentally frustrated ever since.  The thinking process can be a pain all by itself, especially when it concerns the deepest part of ones brain.  Unfortunately, both matters sit side by side physiologically.



Transitory, eh?  I should hope so, but with concern focused upon a theoretical chance for inflammatory changes.  The chance is two-fold in that I control one half by what I do, such as lifting or moving heavy objects.  This surgery is really no different than the craniotomy, with the exception of visual perception.  What you can't see CAN hurt you.  During my recovery from the craniotomy, you said to me, "...slow down, life is long." (a variation of Cicero)



I did have an immediate MRI and a CT scan and intend to nab them from Radiology on the way to see my neurologist.  Better that I hand-carry them with the exception of the post MRI.  It will be sent directly to my neurosurgeon.  We'll have them all, now won't we?  Inquiring minds want to know.  Wasn't it Tennyson in Ulysses who said, "I am a part of all that I have met?"



I have consistent fluctuating eye pain in association with the intensity of light patterns (3, 4, and 6).  I believe 5 was injured during surgery.  I have to squat instead of bend-over to pick something up, and I sleep pretty-well propped-up with pillows because... in both cases, gravity is vertical and takes its toll.



Here's my Rx list:  Neurontin 300mg 3-3x day/ MS-Contin 15mg 2-3x day/ Baclofen 20mg 1-3x day/ Mexiletine 150mg 2-3x day.



Question:  Would it be wise to elevate the Baclofen in 10mg increments as needed for eye stress and muscle