What Is Pseudotumor Cerebri, aka Idiopathic Intracranial Hypertension?

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.

How does PTC relate to headaches and Migraine and their treatment?
PTC can cause headaches and/or trigger Migraines. It can also keep headache and Migraine preventives from working properly.

Also from our "What Is...? series:

• What Are Ice Pick Headaches?
• What Is Abdominal Migraine?
• What Is "Alice In Wonderland" Syndrome?
• What Is Basilar-Type Migraine?
• What Is Chronic Daily Headache?
• What Is Hemicrania Continua?
• What Is Hemiplegic Migraine?
• What Is a Migraine?
• What Is New Daily Persistent Headache?
• What Is Transformed Migraine?

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Sources:

• 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