ptc and alternative therapies

  : : : : Two new therapies to treat ptc have been recommended to us for our adolescent daughter. Octreotide injections 3 times per day have been recommended by an endocrinologist as a possible "resolution". Also, a tyramine-free diet was recommended by a neuro-opthamologist. Our daughter is not your typical ptc patient and has already been through the gamut of surgical interventions. Do you have experience with either of these treatments or know where we may obtain additional information regarding studies that may have been conducted. Any help you can provide will be greatly appreciated.
  : : : Dear Kathy:
  : : : If you could please describe the syndrome "ptc".  The general use of octreotide or somatostatin if in patients with metastic carcinoid and vasoactive intestinal peptide secreting tumors, pancreatic tumors,
  : : gastrinoma and secretory diarrhea.  There are some off label uses such as Acromegaly, AIDs-associated secretory diarrhea, bleeding esophageal varices, breast cancer, cryptosporidiosis, Cushing's syndrome, insulinomas, small bowel fistulas, protgastrectomy dumping syndrome, chemotherapy-induced diarrhea, GVHD induced diarrhea and Zollinger-Ellison syndrom (syndrome).  These I am aware of and I don't recall any of these being given the name of "ptc".  I have asked my collegues and they have not heard of "ptc" syndrome.  If you define it for me I will get back to you.
  : : : Sincerely,
  : : : CCF Neurology:Pediatrics, MD  RPS
  : : : Sorry for my ignorance
  : Dear Kathy:
  : Sorry, but somehow your reply did not transfer.
  : CCF Neuro[Pediatrics] MD, RPS
  Dear Doctor:
  Excuse me for not making myself clear. PTC is pseudotumor cerebri. My husband and I understand Somatostatin is not a "traditional" treatment for pseudotumor but our daughter has received the "traditional" treatments - diamox, neptazane, lumbar shunt, ventricular shunt, optic nerve sheath fenestration, subtemporal decompression, and steroids. As a result of these interventions, she has developed major vision loss, Arnold-Chiari Malformation I, a syrinx, and now her adrenal glands are producing an insufficient amount of cortisol and she is taking meds to elevate her blood pressure. Medically, she continues to baffle the specialists and we are continually looking at alternatives as are her doctors. Any help you might give us as to your experiences at the Cleveland Clinic or where to search the medical literature will be greatly appreciated.
Dear Kathleen:
Thank you for responding, I am sorry that I have never heard of it described by just the letters ptc.  You did not mention your daughter's age or her weight.  Assuming that all the possible explanations (which I know that you have done this) such as infectious, adrenal insufficiency, galactosemia, hyperadrenalism, hyperthyroidism, hypoparathyroidism, menarche, pregnancy, drugs, guillain-barre, iron deficiency anemia, leukemia, polycythemia vera, protein malnutrition, and SLE you have gone through the metabolic causes of increased ICP.  I also imagine that you have had a CSF flow study to make sure there is no obstruction.  This is what I would recommend.  Talking with our pediatric neurosurgeon, he thinks that a properly positioned shunt will likely be your only answer unless your daughter is willing to undergo multiple LP for a very long period of time.  He doesn't think that somatastin is going to work, nor have I seen any large studies to suggest that it will work.  Having place many shunts he thinks that it is more than likely that the initial attempts did not get into the CSF space.  Increased ICP makes it very difficult for shunt placement.  He does his shunt by scope so he sees where he places the shunt (this is a relatively new procedure).  He has not had a failure in the many shunts he has placed other than the routine failures of shunt malfunctions that can occasionally happen.  I am not sure where you are located, but if near the Cleveland Clinic he would be happy to see your daughter.  I realize that you have seen many people and tried many different treatments.  I must say that you have made all the attempts to correct the problem.  A second opinion might be a good idea, especially someone who is taking a fresh look at all the test performed thus far.  The number to call at the Clinic for appointments is 1-800-223-2273.  There was a paper by Johnston et al. in J. Neurosurg 69:195 (1988) that talked about surgical options.  If you go to the Citation Index at a local medical library and look for recent articles citing this article, that may help you see what has been published in the last several months on this issue.  I looked on the internet on new articles on pseudotumor cerebri but found nothing new except what you already know.  I'm sorry that I can't help you further.  I could tell you that if my child needed a shunt, I would not hesitate to take him to Dr. Luciano.  Anyway, good luck.  Let me know what you decide and what happens.
Sincerely,
CCF Neuro:Pediatrics, MD  RPS