The PTC Primer...
Chapter Two - Basics

Pseudotumor Cerebri... The Big Picture

Ok, just what is the disease pseudotumor cerebri?

You've received the diagnosis, heard a lot of medical jargon, have probably been told that it's a rare condition...
and you have a lot of questions.

Let's go 'back to the basics' to cover all bases. Here goes:

What's the problem behind PTC?

Simply put, there is too much cerebrospinal fluid in your head, and there's no place for this fluid to go. Cerebrospinal fluid (CSF) is the liquid that protects the brain and spinal cord from injury. Since your skull is an enclosed unit, the increased pressure from your excess CSF puts pressure on your brain (as well as your pituitary gland, optic nerves, and all the other things in your head). What you get is a true pressure headache.

Why is there so much CSF? One theory is that your body has increased the formation of cerebrospinal fluid. Another theory is your body has a decreased ability to absorb CSF. Something has changed with the circulation of cerebrospinal fluid.

Normally, CSF is absorbed by tiny sponge-like areas on the highest surfaces of the brain called the arachnoid villi. These drain into tinier veins, then larger and larger cerebral veins, and leave the head by only two veins: the right and left jugular veins. Jugular veins drain best when you are upright (with the help of gravity) into the superior vena cava in the chest, and then into the right side of the heart. High pressure in the head results from obstructions along this path.

What causes PTC?

There are two groups of patients who have pseudotumor cerebri. The first group has PTC connected to a specific cause (usually use of a drug or vitamin A). Once the cause is removed, the PTC frequently disappears. This group is referred to as secondary PTC because there is a known primary cause of the raised head pressure.

The much larger second group develops pseudotumor cerebri and a direct cause cannot be found. The doctors have no idea what causes the primary idiopathic PTC. The disease is frequently chronic in this group and needs long-term treatment. For people with chronic pseudotumor cerebri, theories and speculation include:

· Belief that PTC is linked to obesity.
· PTC is caused by a strain on the heart when obese.
· Problem with the ability of the brain to drain blood out of the head.
· PTC is a systemic disease of blood vessel inflammation (vasculitis) causing fluid leaking throughout the body.
· PTC is a systemic water retention problem.
· PTC is connected to hormonal problems (which can also cause obesity).

What are the Symptoms of Pseudotumor Cerebri?

· By appearance, a person looks well.

· PTC can appear spontaneously. It can also appear with pregnancy.

· Initial diagnosis often occurs upon an eye exam, which shows swollen optic nerves and ruptured blood vessels in the eye (papilledema). Since this can be a symptom of a brain tumor, a CAT scan or MRI is needed to eliminate this possibility (hence the name pseudotumor). To confirm the diagnosis of pseudotumor cerebri, a spinal tap must be done to measure the spinal fluid pressure. Normal spinal fluid pressure is 70 mm to 200 mm; in PTC spinal fluid pressure is elevated, usually between 250 mm and 600 mm. The protein level and cell count in the fluid has to be normal for the PTC diagnosis. White blood cell counts over 3 and/or protein over 45 indicate there is an infection or inflammation causing the abnormalities.

·The main symptoms are headache, dizziness and impaired vision. Although it is rare, a person can have PTC without vision problems and/or headaches.

· Headache is often worse on awakening, and can be intensified by coughing, laughing, bending over, crying, and increased physical activity.

· Impaired vision shows up in several ways. It can be visual blurring, brief moments of dimming or loss of vision called transient visual obscurations (TVO's), small specks appearing or vision distortions (items moving up or down). Blindness can occur. Enlarged blind spots are found in virtually all patients with papilledema (swollen optic nerves and blood vessels in the eye).

· Other symptoms affecting PTC patients include: shoulder/arm pain, neck pain, memory problems, awkward coordination, muscle weakness, fatigue, back pain, and depression.

· Possible symptoms include dizziness, hearing loss, ringing in the ears or noises within the head called tinnitus (a study indicates this could be due to stretching and constricting of brain stem due to changes in cerebrospinal fluid pressure).

· Regarding headaches: a study showed 92% of patients interviewed had headaches; 93% of those with headaches said it was the most severe headache they had ever experienced. The head pain was described as a pulsing headache that kept increasing in intensity. Another description was a "pressure" headache, resembling a percolator. Seventy four percent of those with headaches had it on a daily basis.

What are the Characteristics of Pseudotumor Cerebri?

· PTC can last for months or for years. It can go into remission. For those in remission, PTC can re-occur 5% to 10% of the time.

· 80% of PTC patients have some positive response to treatment. (This means that the PTC symptoms improve, not that the PTC necessarily goes away.)

· 10% have ongoing problems
(Jennifer's note: This statistic is based on published medical information, which is very limited on Pseudotumor Cerebri. I highly question this statistic, as I have personally talked with many PTC people who live with daily symptoms and pain caused by this disease.)

· Pseudotumor Cerebri most commonly appears in women of child-bearing age who appear healthy. However, this disease also occurs in children and teenagers (both male and female) and adult males.

· For women of child-bearing age, there is often a history of menstrual problems. Frequently these women are overweight and/or have had a recent weight gain. Although physicians recommend weight loss, there are patients whose weight loss has not affected their PTC.

· Can experience neck stiffness or nausea.

· A number of PTC patients have had a previous history of sinus problems.

· Many patients are light-sensitive; bright lights bother them and fluorescent light will fatigue and bring on confusion.

· Endocrine studies can appear normal.

· Often PTC is complicated with high blood pressure.

· PTC does appear to have a relationship to adrenal hormones.

· Secondary PTC has appeared with use of oral contraceptives, prolonged use of corticosteroids, large doses of vitamin A, use of tetracycline , nalidixic acid, nitrofurantoin, sulfa drugs, lithium, indomethacin, phenytoin. Rapid recovery often occurs when drug use is stopped. Chlordane toxicity (an insecticide) can also cause PTC. Lyme disease has been shown to trigger PTC.

· PTC has been connected as a manifestation of another disease: systemic lupus erythematosus.

*Courtesy of Dr. Eric Singman, M.D.,Ph.D., a neuro-ophthalmologist who wrote a previous newsletter article- here is his listing of possible causes, symptoms, etc:

"There are a multitude of PTC presenting signs and the list of risk factors grows yearly. Following is a laundry list of etiologies, presenting signs and symptoms, and laboratory findings associated with PTC..."

Possible etiologies:
Tetracyclines (including doxycycline and minocycline, antibiotics for chlamydia and acne)
Lithium carbonate (a mineral salt to treat bipolar or manic-depressive disorder)
Addison's disease (a condition in which the adrenal glands hypofunction)
Cushing's disease (a condition in which the adrenal glands hyperfunction)
Prednisone (and other steroids, possibly by any route including topically, nasally, orally, etc.)
Hypo-thyroidism
Hyper-thyroidism
Pregnancy
Obesity
Head Trauma
Irritable Bowel Syndrome
Empty Sella Syndrome (condition in which the pituitary hypofunctions)
Licorice (the inciting ingredient is glycericic acid)
Vitamin A or its derivatives (used in bone marrow transplantation patients and to treat acne)
Respiratory infections
Sleep apnea (a condition in which the patient snores and has headaches upon awakening)
Norplant (a contraceptive that is implanted under the skin)
Growth hormone therapy
Climacteric (the start of menopause)
Kidney disorders and kidney transplants

Possible symptoms:
Difficulty reading
Double vision
Headache (often in the back of the head)
Blurred vision
Reduced peripheral vision
Darkening or blurring of vision with bending over
Tinnitus (ringing in the ears)
Eye pain
No complaint (patients might not notice a visual field loss!)
Facial twitches (a condition called myokymia)
Nausea

Possible signs:
Convergence insufficiency (a decrease in the ability of the eyes to turn inward)
Facial twitches
Anisocoria (pupils of unequal size, a presentation we discovered and hope to report)
Nystagmus (fine or gross uncontrollable eye movements)
Vomiting
Papilledema (but this need not be present)
Lack of spontaneous venous pulsations on the optic nerve (but this need not be present)

Possible laboratory/ radiologic/therapeutic findings:
Normal brain imaging study, with small ventricles (the fluid filled spaces in the brain)
Elevated opening pressure (above 200-250 mm), although normal pressures can occur.
Elevated levels of Anti-cardiolipin or Anti-phospholipid antibody in the blood
Beneficial response to Diamox

PTC People... Who Are You?

Look in the mirror. What does a person with pseudotumor cerebri look like? What are your problems and your symptoms? What do you have in common with other people who have pseudotumor cerebri?

In past newsletters we included a detailed questionnaire for PTC people. It has been interesting assembling this data; while seeing common characteristics, it has also been a reminder that this disease affects each person individually. The information on these questionnaires offers a peek at what other people with PTC are experiencing.

Here's the breakdown of the group of 96 respondents:

· Seven are male (4 under the age of 15, 2 in their forties, 1 in his thirties). The remaining 89 are female. Nearly two thirds of the group (60) have experienced vision loss ranging from 2% to 70%, with one having complete vision loss in one eye. Fourteen out of the 96 are in remission from the disease.

· Women include 8 in their teens, 23 in their twenties, 33 in their thirties, 18 in their forties, 5 in their fifties and 2 in their sixties. Nearly half the women had menstrual irregularities. Since being overweight is a common factor, we asked about this and if weight loss helped their symptoms.
teens: 4 overweight; 1 said weight loss didn't help
20's: 20 out of 23 overweight; 8 say weight loss didn't affect PTC
30's: 31 out of 33 overweight; 14 say weight loss didn't affect PTC, 4 state it did help
40's: 13 overweight; 12 say weight loss didn't help
50's: all overweight; 4 out of 5 said weight loss didn't help
60's: both overweight; one said weight loss didn't help

Other Than PTC, How Healthy Am I?

Over 3/4 of the entire group have balance problems (80 out of 96), mood swings (86) and chronic fatigue (84). Most have healthy livers, kidneys, gallbladders, adequate sexual appetite and good blood pressure (the few reported incidents of gallbladder problems and higher blood pressure were mostly in older persons; not atypical from the general population).

One third have problems with recurrent or low-grade fever, with itching, and with diarrhea. Sixty-four persons have problems with sleeping. The areas of the body where people report experiencing pain were mainly:
· the head/neck/ shoulder area
· the face
· and the hips/legs area.

How's Your Headache?

Forty-four report their heads hurt "all over". Others have pain in the back (18), the front (30), the sides (23) and the top (22). Of those who responded to the question, 20 said the pain was "constant", 26 said it was "daily", 10 said they had headaches several times a week, 1 said pain occurred only at night, 8 have headaches once a week, and 5 said they have headaches 1-2 weeks a month.

Not surprisingly, another frustrating area is memory/concentration. Nearly 3/4 of respondents (69) report memory problems (mostly short-term) and difficulty with concentration.

Is It Hot In Here, Or What?

Most of you can't stand the heat; 74 out of 96 report heat intolerance. Other conditions that aggravate symptoms are noise (58), mental stress (66), physical exertion (48), posture (30), weather (33), cold (22), and caffeine (8). (Interestingly, 31 people report that caffeine actually helps their symptoms.)

Nearly 2/3 (57) report frustration with tinnitus, or noise in the ears. The noise is described as whooshing, sloshing, roaring, ringing, "the ocean" and a heartbeat. Two report tinnitus as their "most aggravating symptom."

The Eyes Have It

Light bothers PTC patients. There are difficulties with fluorescent lighting (58 out of 96), bright lights (75) and diminished vision in the dark (61).

Descriptions of vision problems include:
· temporary dim-outs or blindness (TVO's)
· double vision
· gray circles in vision during the day
· yellow light circles at night
· blurred vision
· tunnel vision
· white outs
· dark spots
· wavy lines
· "floaters" or "stars"
· holes in vision
· bright "light flashes"
· dimness
· depth perception problems
· distorted or curved vision
· transparent objects that blink on and off to the pulse rate.
· lights streaking
· blackouts and flickering vision
· pupils that do not dilate at the same time
· crossed eyes or lazy eyes
(Note: a symptom of crossed eyes is dangerous; see a doctor
ASAP)
· wavy lines in peripheral vision
· color changes

Respondents' Tips For Coping
· "Drink caffeine".
· "Wear sunglasses whenever you're outside".
· "Ice packs/hot packs on my head help a lot".
· "Lie down to equalize the pressure."
· "Listen to soothing, soft music and focus on your breathing- in and out, in and out."

Clearing Up Myths About Pseudotumor Cerebri

· About PTC being a short term disease- a study of 13 patients in 1982 showed them needing spinal taps 5 to 41 years after being diagnosed with PTC.

· About repeated spinal taps- the only benefit is short term relief of severe headaches. Multiple spinal taps may contribute to chronic low back pain. A study in 1974 showed spinal taps had a short-lived effect on cerebrospinal fluid In one case the pressure returned to its previous level after only 82 minutes. Also, repeated invasive procedures to the body can develop a secondary painful condition called arachnoiditis (chronic inflammation of the arachnoid lining in the head and spinal cavity).

· About PTC being a 'benign' disease: there is nothing benign about going blind or living with chronic pain.

· "You've got it because you're fat"....... Your doctor may have told you this. However, there is the thought that whatever is causing the PTC is causing the individual to be overweight. What the "that" is, is unknown (hormones? endocrine system? phase of the moon? misaligned body clock?). Some people get relief of PTC symptoms by losing weight. Some people do not. We consistently hear from people who state they are overweight, but do not eat large amounts of food. (A thought to bear in mind here: Some people consume many calories as Pepsi or Coke drinkers, so carbohydrates may be part of the problem, rather than the amount of food itself.) We also hear from people who say their weight gain happened inexplicably just prior to having PTC symptoms, or shortly after their symptoms started.

Our question to the professionals: If you get PTC because you're overweight, then why is pseudotumor cerebri such a rare disease? There are many, many overweight people in this country.

This and That.....

How many people have PTC?
Answer: We estimate there are 12,000 people in the US with pseudotumor cerebri.

Statistical Stuff
How we did it: Although there have been studies to determine the percentage rate of the general population who have PTC, there have been no studies or accounting of actual numbers. The generally-accepted percentage rate is 0.9 per 100,000 overall. The rate increases to 19.3 per 100,000 for females 20% or more above their ideal weight. The female-to-male ratio for adults is 8:1.

If you use the .9 per 100,000 for overall population and apply that to the 1993 total US population of 257,908,000, you get an answer of 2,321 people with PTC. However, a closer look proves interesting.

Of the total 257,908,000 1993 US population, the number of people 18 years and older is 181, 447,000. Of this 18 year and older segment, 29.6% of the males are 20% above their normal weight; and 25.6% of females are 20% above their normal weight. The actual number of the female ratio (the number of women who are 20% above their normal weight) is 46, 450, 430 women. When we apply the 19.3 per 100,000 for PTC, we get an astounding figure of 8,975 women with PTC!

When we divide the 8, 975 by 8 for males (using the 8:1 ratio), our answer is 1,122 men with PTC. Next, apply the .9 per 100,000 ratio to the 76,461,000 people under age 18 and the answer is 688 children/minors with PTC. Last, subtract the women who are 20% over their ideal weight and over age 18 from the total of people over age 18 and apply the .9 per 100,000 (181,447,000 minus 46,450,430 divided by 100,000 times .9) for a total of 1215 people over age 18.

A total of these four numbers (8, 975 + 1, 122 + 688 + 1,215) comes to 12,000 people in the US with pseudotumor cerebri.

Now this is by no means complete or even totally scientific. I'm sure individuals qualified in statistical analysis would arrive at a different number. But the point of this exercise is to show, that based on accepted population ratios, there's a lot more people with pseudotumor cerebri than the general public (and I think even the medical community) is aware of ! This "rare" disease isn't quite so rare.

(By comparison, some authors estimate there are 250,000-350,000 people with multiple sclerosis and 38,000-129,000 people with lupus erythematosus within the USA.)

Treatment options that have been used for PTC

· medicine: diamox, neptazine, lasix, steroids in children after infection
· frequent spinal taps to "bleed off" excess spinal fluid
· surgical insertion of a shunt: lumbar-peritoneal (drains off fluid from spine into abdominal cavity) cisternoatrial, cisternoperitoneal, ventriculoperitoneal (drains off fluid from brain into peritoneal cavity)
·surgery on the optic nerves to prevent blindness or further loss of vision (called either Optic Nerve Sheath Decompression-ONSD, or Optic Nerve Sheath Fenestration- ONSF).

Shunt Negatives

· Shunts have had a significant rate of failure. There are problems with the opening of the shunt becoming clogged and not working properly. This can result in significant pain for the patient in the area of the shunt opening.

· Arachnoiditis (pain caused by chronic inflammation of the arachnoid lining in the head and spinal cavities) can be a complication post-operation.

· A study in 1991 showed 37 patients having 73 lumboperitoneal shunts plus 9 ventricular shunts. Only 14 patients were "cured" after a single surgical procedure. Average time between shunt insertion and shunt replacement was 9 months, although 64% of shunts lasted less than 6 months.

· The two most common causes for reoperation was shunt failure (55%) and low pressure headaches (21%). The vision of most patients either improved or remained stable after their operations.

· The number of shunt operations averaged 2.2 per patient, ranging from one shunt to seven shunts.

Optic Nerve Sheath Decompression Facts

· This is surgery on the covering of the optic nerve in the eye. Small cuts are made to allow drainage of cerebrospinal fluid, so there is less pressure pinching the tip of the optic nerve on the back of the eye. This is becoming the most popular option for acute PTC in order to prevent blindness.

· Although recently gaining popularity, the first optic nerve sheath decompression was done by De Wecker in 1897.

· A study of 53 patients who had optic nerve sheath decompression resulted in: 35 patients with improved visual function. Of 18 patients with chronic papilledema, only ten had improved visual function. Several patients had to have a second or even a third decompression after initial successful results.

Pregnancy and PTC

· A case of a woman who developed PTC 3 weeks following eclampsia (medical condition requiring immediate attention) brings up questions about correlation of PTC and changes in estrogen, progesterone and prolactin.
· Successful pregnancy can be accomplished having PTC. We recommend women use a high-risk obstetrician who can monitor the development of the pregnancy and confer with the patient's neuro-ophthalmologist as needed.
(for more information about PTC and pregnancy, see our Newsletter 2001 web page.)

Misc./FYI
· 2 girls in Galveston, TX developed specific antibodies to silicone in their recent implanted shunts. This finding suggests that humans can develop a specific immune response to silicone. (10/92)

Words to Know
· Diplopia- a result of increased pressure, this is most commonly 6th nerve palsy; almost always horizontal, rarely vertical

· Empty Sella- an enlargement of the space in the brain above the pituitary tumor. The enlarged space is filled with CS fluid under high pressure. The stretch on the visual fields can give the same abnormality as a brain tumor. Thus, a head scan is necessary.

· Papilledema- a swelling of the optic nerves and optic disc; it may be accompanied by swollen and ruptured blood vessels in the eye

· CSF- cerebrospinal fluid

· Transient Visual Obscurations (TVOs)- seconds-long blackouts of vision. They may occur while changing body positions or sitting quietly.

· Arachnoiditis- a progressive inflammatory disorder that can be a complication of PTC due to a mixture of blood with the spinal fluid, common after repeated spinal taps or shunt revisions. The arachnoid is the middle membrane surrounding the spinal cord and brain. Symptoms are severe headaches, stiff neck and pain when bending forward, vision disturbances, dizziness, nausea and vomiting (same symptoms as PTC). When the spine is affected, weakness and paralysis can develop.

· Med Fact: Cerebral blood flow was studied in 9 patients with PTC. Globally elevated blood flows were found in all 9 patients averaging 149% of normal flows.

· Med Fact: A study of 177 PTC patients found only 26% of patients had low protein in their fluid.

· Med Fact: Some form of vision problem will occur in about 50% of PTC patients, with serious visual loss in 25% of patients.

· Med Fact: Contrary to earlier data, recent studies suggest that the incidence of menstrual problems are not significantly higher than normal population.