Simply speaking, Pseudotumor Cerebri is a condition in which the body either produces too much cerebrospinal fluid pressure or doesn't absorb it well, resulting in increased cerebrospinal fluid pressure.

Pseudotumor cerebri literally means "false brain tumor" because it's symptoms mimic those of brain tumors. It is also (more accurately) called Idiopathic Intracranial Hypertension (IIH). The term idiopathic is applied because it's cause is not truly known. It is likely due to high pressure caused by the buildup or poor absorption of cerebrospinal fluid in the subarachnoid space surrounding the brain, but the reason for the buildup or poor absorption are also unknown. The disorder is most common in women between the ages of 20 and 50. Being overweight seems to be a a possible contributing factor, but PTC should not be ruled out based only on body weight, age, or gender.

Symptoms of pseudotumor cerebri:

• headache, sometimes daily, sometimes severe, not relieved by medication
• hearing loss
• impaired vision or eventual blindness
• memory problems
• Migraine attacks with unexplained triggers
• nausea
• pain behind the eyes
• pulsating intracranial noises
• shoulder and/or neck pain
• tinnitus
• vomiting

Diagnosis of PTC:
The only truly definitive diagnostic test for PTC is a lumbar puncture (LP) (spinal tap) with the cerebrospinal fluid (CSF) pressure measured when the needle is inserted. In some patients, swelling of the optic nerve (papilledema) can be observed in a thorough eye exam. However, it is important to note that the absence of papilledema does not rule out PTC. Not all patients with PTC exhibit papilledema. When the LP is performed, it is also essential that the protein level and cell count of the fluid be tested. The presence of protein or elevated white blood cell count indicate can indicate that inflammation or infection could be causing the elevated CSF pressure.

Secondary Intracranial Hypertension:
While PTC and IIH are idiopathic in origin, Secondary Intracranial Hypertension always has a cause. Diagnosis of Secondary IH is the same as IIH, but Secondary IH can be traced back to causes such as other conditions or medications:

• dural venous sinus thrombosis
• kidney failure
• Leukemia
• Lupus
• excess Vitamin A
• growth hormone treatments
• nasal fluticasone propionate

Treatment:

• Medications, commonly medications with diuretic actions. (Diamox is a common choice.)
• Discontinuing medications that can exacerbate the condition. (Includes oral contraceptives and some steroids)
• Weight loss
• When medications fail to control the CSF pressure, therapeutic shunting, which involves surgically inserting a draining tube from the spinal fluid space in the lower spine into the abdominal cavity, may be needed to remove excess fluid and relieve pressure.

Prognosis:
Close, repeated ophthalmologic exams are required to monitor any changes in vision. Surgery may be needed to remove pressure on the optic nerve. The disorder may cause progressive, permanent visual loss in some patients. 

From my experience:
When I began treatment with a Migraine specialist, I couldn't identify the trigger for about 50% of my Migraines, and I experience tinnitus with those Migraines. Preventive medications weren't working for me. Dr. William B. Young of the Jefferson Headache Center, my specialist, recommended that we do an LP to rule out PTC. I also have advanced glaucoma, so I see an expert ophthalmologist on a regular and frequent basis. My ophthalmologist specifically looked for papilledema when he heard this. I never had papilledema. An LP showed my CSF pressure to be above the "normal" range. Dr. Young withdrew enough CSF during the LP to put my pressure into normal range. He had me journal my Migraines and return in three weeks. That 50% of my Migraines where I hadn't been able to identify a trigger and had tinnitus were simple gone. When I returned to his office, Dr. Young and I discussed medication options with my ophthalmologist, then Dr. Young wrote me a prescription for Diamox. Not long after that, preventives began working for me.

If you suspect you may have PTC, please talk to your doctor. Don't accept it if you are told that you can't have PTC because you don't have papilledema; that's simply not true. Even if an LP shows that you don't have PTC, you have at least ruled out a physical cause for your headaches and/or Migraines.

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Resources:

National Institute of Neurological Disorders and Stroke. NINDS Pseudotumor Cerebri Information Page. Bethesda. 2006.

Pseudotumor Cerebri Support Network.

Intracranial Hypertension Research Foundation.

Bond, DW; Charlton, CPJ; Gregso, RM.  "Benign intracranial hypertension secondary to nasal fluticasone propionate." BMJ 2001; 322: 897

© Teri Robert, June 6, 2006