The PTC Primer...
Chapter Three - Update

Medications, Treatments, Research, Etc.

Interested in the "big overview" of ways to treat PTC? Read on...

Pathway 1: Pharmaceutical

The first level of treatment is usually drugs (pharmaceutical). There are several strategies (or combinations thereof) that your doctor may "choose."

The most common class of drugs used are the carbonic anhydrase inhibitors (CAI's): diamox (acetazolomide), neptazane (methazolamide), daranide (ethazolamide). These are pump inhibitors and reduce both the spinal fluid production from the choroid plexus in the brain's ventricles and the eye's aqueous production from the ciliary body (in other words, they reduce cerebrospinal fluid production in the brain and in the aqueous fluid in the eye). It was determined in the early 1970's that acetazolomide can reduce cerebrospinal fluid production by about 50%, and lower head pressure. Acetazolomide can also help control transient visual obscurations (or TVO's, which are seconds-long blackout of vision), headache and diplopia (which is horizontal double vision).

Another option is diuretics, such as furosemide (lasix) or chlorthalidone. Diuretics reduce fluids from the body; the goal with using diuretics is to reduce the cerebrospinal fluid (or CSF) level as well, which in turn relieves pressure in your head. The problem with diuretics are that side effects are common and can be severe, to the point that the drug cannot be tolerated. Common side effects are nausea, vomiting, dizziness, blurred vision, a metallic taste in the mouth, and itching or tingling of the fingers and toes.

Steroids are used after infections or inflammations, especially in children and in patients with high CSF protein counts. They are best used for short periods of time, since long-term therapy can aggravate high blood pressure and weight gain, both which make PTC riskier.

Digoxin is a medication used as treatment for heart conditions. It is also used for PTC. Lanoxin is an example of a brand name of this drug. Digoxin comes from the digitalis plant. Interestingly, a Welsh pharmaceutical book from the 11th century suggests using the digitalis plant in the treatment of headaches. Digoxin reduces the amount of CSF (remember, that's cerebrospinal fluid) produced in the body. Side effects can include toxicity to the body (digoxin levels need to be monitored), visual disturbances, nausea, fatigue and mental confusion.

Cytoxan is a drug that thins the CSF. Cytoxan can reduce cerebrospinal fluid protein in patients with cerebrospinal inflammations and secondary pseudotumor from clogged arachnoid villi or cerebral veins. An example where doctors would prescribe this for PTC patients is because the CSF "gunks up", having a semi-gelatinous consistency. If a patient has a shunt in the body and the shunt clogs with CSF, shunt operation stops and head pressure goes up (along with increasing headache). Cytoxan is a pretty potent drug (it is also used for cancer treatment). It is toxic and generally used in extremely serious cases. Common side effects are: nausea, vomiting, diarrhea, flushing, sweating, temporary hair loss, and darkening of skin/nails. It's important to drink lots of fluids while taking this medication. This allows the kidneys to remove the drug from your body quickly and can diminish the side effects.

So far we've discussed drugs that treat the physical aspects of PTC. Now let's briefly look at pain management drugs. These are called "symptomatic therapy." There are the obvious over-the-counter options such as aspirin, acetominephan (Tylenol, etc.), ibuprofen (Advil, etc.) and so on. Heavy use can have various side effects, including stomach upset and analgesic rebound, which is a lessening effectiveness of the drug. Analgesic rebound can also trigger headaches.

Since over-the-counter drugs frequently do not address the intensity of PTC headache pain, there are other options. Beta blockers such as Inderal, Corgard, Timolol and Labeto can be effective. Calcium channel blockers like Verpramil and Eilteazen can also help. Narcotics like Darvoset, Talwin and Percocet can alleviate pain, but need to be used with discretion to avoid addiction. Depakote is an anticonvulsant which can be effective with headache. Clonidine patches (a patch applied to the skin once a week and kept in place) enhances the effectiveness of pain medication and can also lower blood pressure. Another patch called a Duragesic patch (applied to the skin every three days) is also effective for managing intense pain.

The decision of which drug to prescribe for pain management is a carefully orchestrated dance that you and your physician need to engage in. Just as in real dancing, effective pain management is a partnership where two people- you and your physician- need to be comfortable with each other, give feedback, and know the proper steps to take. You need to let your doctor know how effectively the medication is working and if you are having any side effects. In turn, your physician needs to monitor interactions with other drugs and keep up-to-date on new pharmaceutical advances.

Special note: We have received reports of people being prescribed Ultram for pain medication. McNeil Pharmaceuticals, manufacturer of Ultram, has cautioned against the use of Ultram for anyone who has increased intracranial pressure (that's you, PTC people). For more information about this drug, contact McNeil Pharmaceuticals at 800-542-5365.

Pathway 2: Surgical Intervention

When drug intervention is either partially effective or ineffective, your doctors are going to recommend surgical options. Unremitting headaches and a steady loss of vision need a more aggressive approach. There are two commonly used methods to divert the pressure of the spinal fluid from squeezing the top of the optic nerve and causing lack of circulation:
1) having a shunt placed in the body to divert cerebrospinal fluid, or
2) having a surgery called an Optic Nerve Sheath Decompression (ONSD). Tiny slits are cut into the sheath covering the optic nerve. ONSD surgery relieves the pressure on the optic nerve by allowing CSF to leak through (aren't you tired of all these abbreviations!?!). ONSD surgery has had good results in stopping vision loss and even in restoring some or all of previous vision.

There has been much discussion over which should come first- the shunt or ONSD. Because of shunt failure rates in the past, ONSD is becoming more popular and recommended as a first-choice operation. It should be noted, however, that the goal of ONSD surgery is to save and/or reverse vision damage; this surgery does not always address the issue of high pressure headaches. If you are considering the ONSD procedure, find a qualified neuro-ophthalmologist who has experience with this surgery.

If a shunt becomes necessary, do some research on both the surgeon and the type of shunt to be used. The most popular type of shunt used for pseudotumor cerebri in the last 20 years is a lumbo-peritoneal (or LP) shunt. This shunt connects to the spine and drains CSF into the abdominal cavity. Other shunts that have been used in the past are the ventriculo-peritoneal (VP), cisterno-atrial (CA) and cisterno-peritoneal (CP) shunts.

Having a shunt placed in your body requires serious thought and the knowledge of both pros and cons. A shunt can alleviate and prevent high pressure headaches. It can also stop and even reverse vision loss.

On the "con" side are a number of factors to consider. There is a 50% shunt failure and revision rate due to infections, dislocations, inflammations and occlusions. Over-filtration (too low a pressure) can cause low pressure headaches, and more rarely subdural hematomas and hygromas. Over-filtration or low pressure headaches appear to be most common with LP shunts, probably because the spinal fluid is made in the head (where the pressure is higher) and drains from the lower back (where the pressure inside the abdominal cavity- or peritoneum- is lower. The abdomen pressures rise in pregnancy, obesity, constipation, and horizontal positions. Sitting or standing allows gravity to speed the flow out of the lower end of the tube; it can cause a sudden "rush" of headache from the stretch on the arachnoid coverings of the brain.

A low pressure headache for one or two seeks after insertion of a new LP shunt is normal. Slowly raising the head from flat to 30 degrees to 90 degrees day by day is the way to readjust. If the pressure is too low, a re-operation is needed to change the size of the tubing or add a different valve to slow the flow. Long-term low-pressure may lead to an acquired Arnold-Chiari I malformation. This causes the brain cerebellar tonsils to fall out of the base of the skull and put pressure on the lower brain-stem .

Pathway 3- Diet

Yes, Diet. The big "D" four-letter word. I know the hairs are raising on the back of your neck, but D I E T is a non-invasive treatment option that is important to consider. First of all, there are no drug side effects. Secondly, dieting is not nearly as invasive (or inconvenient) as surgery. Last of all, there is a good chance of success in relieving or eliminating symptoms.
(Editor's note: I'd like to add here that I'm not a thin person. If you're obese (as I am), and you're thinking about murder if one more thin, hyperactive person blabs on and on about how easy it was to lose weight and how their entire life has changed for the best ever since, I'm right there with you. There are people who believe PTC is connected to an endocrinological disorder. If this proves to be true, this could predispose you towards obesity. But the truth is overweight people with pseudotumor cerebri have lost weight and gotten better. )

If you lose weight, are you guaranteed to get rid of your PTC? No. In addition to the successful cases mentioned above, there are also numbers of people who have lost vast amounts of weight and had no change in symptoms. And there are thin people who have PTC of unknown origin.

No guarantees here. Only a chance of better health if weight is lost.

Some patients have tried gastric stapling surgery as a way to achieve weight loss. This is an idea that several doctors have suggested. Be aware that this is serious surgery; there are definite risks involved. Talk to your doctor about your situation.

Pathway 4- Alternative Treatments

There are several ideas to consider that "traditional" medicine may not recognize as viable options, but could be effective for you. (Editor's note: Jennifer and I would like to stress one thing: always remember that your body belongs to you and you have the right to search out treatment options that may benefit your body. We feel you need to be in control of your health and destiny.)

Here's a possibility to consider: Dr. John Upledger of Palm Beach Gardens, FL (next to West Palm Beach), is a trained osteopath who developed and practices a treatment called "CranioSacral Therapy". This treatment is applied to poorly-understood malfunctions involving the brain and spinal cord and chronic pain. Dr. Upledger was featured in the June Columbus Monthly '96 in an article about OSU diver Mary Ellen Clark and her battle with vertigo.

The Olympic medalist's career seemed over and a worldwide search for treatment was fruitless until she went to Dr. Upledger. He states: "Her symptom was vertigo, but her problem was in the membrane system surrounding her brain." (See the similarities in info with PTC?) Ms. Clark was treated, resumed her career, and went on to win the Bronze medal at the Atlanta Olympics.

Dr. Upledger is interested in working with PTC patients who have visual disturbances. Consider going to qualified massage therapists and physical therapists that have trained in this technique.

Other treatment options to consider would be acupuncture, massage therapy, rolfing, meditation, Therapeutic Touch, herbal therapy, nutritional/natural diet therapy... and the list goes on. There are several options that may be valuable to you; we urge you to talk to others (if you have a success story, tell us- we'll share your info!), do research, and go to a reliable practitioner.

PTC Chronic Pain Primer

"Pain is probably the most common symptom today in the field of medicine and all its allied disciplines. Ironically, it's the least understood. Many physicians, and various medical professionals, have had relatively little training in the management of pain, per se." Dr. Jerome Meers, PhD Psychologist, FMS/Ohio Newsletter

Pain is a daily fact of life to most people. There's minor aches and pains, occasional headaches, bumps and bruises, and other stimuli that we react to and either treat or ignore. Acute pain occurs suddenly and usually signals a physical injury or tissue damage. Chronic pain, however, goes on and on without relief. The relentlessness of chronic pain will wear down a person both physically and psychologically. Chronic pain can encourage other symptoms to emerge, which aggravates the pain. It's a vicious cycle.

Where does the pain come from?

All right, what does pain look like? Where is it located? Although your first response may be, "it's in my neck, or my head, or where my shunt is", pain actually exists only in your head. (For all of you with chronic headaches, you may think I'm stating the obvious, but bear with me.) Pain is actually a psychological phenomenon. It exists in your head, or more specifically, your brain. Until you get a signal into the brain from the source of pain, you have no pain. Your back may feel like it's aching, but until this signal of pain registers in the brain, there is no pain.

What's the point of this? It's a reminder that psychological and physical symptoms coexist. The mind and body are intertwined; they are not separable. It's important to remember this fact when you approach your pain management.

A moment on the psychological aspects

Chronic pain that cannot be physically viewed is both a private and frustrating experience. You alone knows what the pain feels like. You cannot adequately communicate that to family members and friends. You look OK, so they do not understand either the intensity of the pain or the duration. As supportive as they try to be, there will be many times when they just don't "get it".

Think a moment about how you were taught about dealing with pain as you grew up. Was it acceptable to talk about it? Could you complain and collapse? Were you encouraged to carry on quietly? Were you ignored or nurtured? Your attitudes about pain that were developed from your family can greatly influence how you handle the pain you experience today.

Pain can be eased by outside stimuli. When you're happy or excited about events in your life, you have things to focus on beside pain. Positive experiences allow the pain to be tolerated more easily. On the other hand, lack of enjoyable experiences in your life leave more time to focus on the pain. Stress, depression, anxiety and feelings of helplessness can increase your pain perception.

Pain Management

Pain management is a good term for chronic pain sufferers. Just as an office manager attends to the many details of office life and strives to make things run more efficiently, the "pain manager" works to efficiently manage the details of pain with a goal of a fuller life. The pain does not go away; it is just better managed. You, the patient, have more control of your life.

The ideal situation involves you and your physician, working together to use whatever strategy necessary to minimize your pain. Unfortunately, there are doctors who are not open to this philosophy and insurance companies who refuse to pay for anything except drugs. Logic doesn't prevail here, since alternative or supplemental therapies are often drastically less expensive. If you have a less-than-ideal situation, your first step may be to find a supportive physician and/or a better insurance company. Finding a physician is probably the easier step. If you are not comfortable with your doctor, find a replacement. Ask friends, neighbors, family and other patients for a referral. Be specific about what you're looking for in a doctor.

Medicine is often the first line of defense. A variety of medications are available; some with possible negative side effects. Work with your doctor to develop a strategy of medication that works for you. Keep up on new pain medications and talk about them with your doctor. Develop the attitude that this is a partnership between you and your physician.

Unfortunately, some people stop with taking medication and don't explore other options. It can take a lot of mental energy to become your own strongest advocate and actively search out pain relief when that actual pain leaves you exhausted.

Find those who will support you in your cause and persevere; the benefit of reduced pain is well worth the effort. A brief listing of options to explore include:

A TENS unit (transcutaneous electrical nerve stimulator), which is a machine that sends electrical signals to confuse your brain from correctly interpreting your pain signals. There are a variety of models and a doctor's prescription is needed. Some people benefit greatly; others don't.

Diversion can work and it's free. Focusing on interests can distract your brain so it perceives less pain. Watching funny movies, needlepoint, gardening, watching sports, reading a good book, and attending a play are a few examples of diversionary techniques.

Producing endorphins and enkephalins in your body naturally will produce an analgesic effect and benefit you by increasing your pain threshold. Three ways to do this is to physically exercise your body, obtain a deep state of relaxation and drink warm milk.

Relaxation allows your muscles to relax. Many people in chronic pain don't even realize their muscles are tense. Learning progressive muscle relaxation benefits you by releasing lactic acid in your muscles, a waste product created when muscles are tense. Your total pain level is lowered since your muscles are no longer sending out pain signals. It's not possible to be tense, in pain or anxious and be relaxed at the same time. This is an important technique to learn. Relaxation can be aided with soft words, soothing music, a darkened room or visualization of a pleasant memory.

Biofeedback machines can be used to monitor what's going on physically in your body, such as blood pressure, skin temperature, muscle tension, etc. By using a biofeedback machine, you can learn to alter these physical changes at any time, without the use of the machine. Controlling your physical responses will indirectly allow you to control your pain.

Self-hypnosis is defined best as "focused arousal". You are aroused mentally and so focused on something that you're not aware of other stimuli around you (like your pain). Self hypnosis can be taught, is safe and potentially very helpful.

Massage is a physical way of relieving muscle tension and pain. It's also a nice way to make yourself feel good! Find a masso-therapist that will work with you and be sensitive to your levels of pain; the goal is not to hurt more, but to feel better. Many massage techniques can be taught to your partner for a long term, less costly approach.

Cognitive behavior technique is a medical way to describe how we think and how we feel. If you understand how you are feeling, you can then recognize how you are reacting to your pain. Working with a therapist in either individual or group counseling can give you a way to restructure your experience of pain.

These are just a few tools available to help you with your pain management. Open your mind to different options and explore whether they will work for you. We encourage you to share with us your successes (and failures, too!) in your job as "pain manager."

PTC Research and Theories

(Ed. note: As most of you are aware, The PTC Support Network is constantly trying to find out information regarding development of treatments and research being done for pseudotumor cerebri. We invite all physicians involved in the research of PTC to call or write to us so we may share.)

Why do people get pseudotumor cerebri? The "easy" PTC patients can have their disease traced back to a specific cause (drug interaction, overuse of vitamins, head trauma, etc.). Usually, these patients can be treated and their PTC will go into remission or disappear. However, the tougher PTC patients have PTC from an unknown cause, and the simple answer as to why those patients get PTC is "I don't know".

This disease has a lot of "I don't know" answers. There are theories, and limited research has been done ("limited" because there are few funding dollars for the pseudotumor cerebri disease; there are not enough people with PTC nationwide to allow much interest or money). The following offers a few doctors and their theories. Your physician may concur with one of them or may disagree with all of them. In research as in treatment options, there are no easy answers.

Dr. Rekate (Phoenix, AZ) believes that PTC is caused by a strain on the heart when the patient is obese. He advocates weight loss and/or gastric stapling surgery as treatment. When the patient is not obese, he believes there is a problem with the ability of the brain to drain blood out of the head. In this case, he advocates a shunt or ONSD surgery.

Dr. Corbett (Jackson, MS) believes that PTC is linked to obesity. Many of his patients have had improvement with 25-30 lbs. weight loss.

Dr. Streeten (Syracuse, NY) believes PTC is a systemic water retention problem and advocates a low salt diet to help this condition.

Dr. Deborah Friedman of the SUNY Health Science Center in Syracuse, NY is conducting a clinical trial for PTC. She theorizes that PTC is a systemic disease of fluid leaking throughout the body and is doing research into this idea.

Dr. Harvey Sugarman of the Virginia Commonwealth University Hospitals in Richmond, VA believes that "the high failure rate for lumbar-peritoneal shunts is due to shunting the cerebrospinal fluid from one high-pressure system (the brain) to another high-pressure system (the abdomen)." Dr. Sugarman has performed gastric-bypass surgery on 24 overweight PTC patients instead of inserting a shunt; most patients have had relief of PTC symptoms. (Ed. note: complications did arise from the gastric bypass surgery with some patients.)
(Refer to the magazine Neurology, September '95 and August '97 for articles by Dr. Sugarman regarding this option.)

Dr. Susan Benes, a neuro-ophthalmologist at OSU states: "At Ohio State University, research has suggested that the use of somastatin analog (SAS, Sandostatin or Octreotide) may have a role in the therapy of some patients with pseudotumor cerebri. This was noted in a 14-year-old boy with precocious puberty, tall stature (6'7"), elevated growth hormone and somatomedin C levels, and a blinding condition with chronic papilledema and abnormal vessels growing around both optic nerves. The boy's opening pressure was 420 mm prior to the SMS therapy, which was given to reduce growth hormone levels and halt the growth of the abnormal vessels in his retinas. A week later the vision was improved, the vessels stable, and the opening pressure was only 160 mm.
"The boy used SMS for 18 months, during which time his vision was stable and his headaches rare, but because of abdominal gas and gall bladder symptoms and progressive dental decay, he discontinued the drug. Fortunately, the condition seemed stable for the next 3 years off medication, but in the last 6 months, his sight has deteriorated and headaches have returned.
"OSU researchers wonder whether the SMS might have stabilized this patient through endocrine hormone actions, neurotransmitter brain actions or vascular bed shunting of blood flow. At OSU an additional 12 patients have used SMS for various ocular conditions, and a study for its role in PTC has been proposed. Because of the cost ($200 per bottle), mode of administration (daily 3 injections) and potential side-effects, the process of study approval has already taken 3 years.
"A newsletter from a New York PTC support group has alluded to a breakthrough therapy, which will not be disclosed until they publish the results of their first patient. However, this group advocates the use of a tyramine-free diet in the treatment of PTC. The tyramine may reduce some of the migraine headaches that PTC patients may also experience.
"These research approaches should give PTC patients hope that they not being ignored and that for some, there will be some successful treatment options."

Meds FYI

Consult with your doctors if you think the following information may be of help:

· Several members have observed that caffeine relieves their pain from low-pressure headaches.

· Midrin has been reported to us to help high-pressure headaches. It is primarily a migraine medication.

· If you have a shunt, be sure to take antibiotics before any dental work to prevent infection or bacterial complications.

· Zoloft helped one member with relief from pain and depression ; another member had an adverse reaction of extreme depression.

· Catapres-TTS-2 (Clonidine) is a transdermal patch produced by Boehringer Ingelheim Pharmaceuticals. The patch is applied to your skin once a week; medicine is released slowly through the skin during the week. The medicine contained in the patch helps your pain medication work more effectively. An added benefit is that it can also control high blood pressure. (Jennifer has used this effectively for a long time now.) For more information, talk to your doctor or contact Boehringer Ingelheim Pharmaceuticals at 203-798-9988.