About Pseudotumor Cerebri

General Information

• Patients with pseudotumor cerebri have elevated intracranial pressure unrelated to tumor, hydrocephalus or brain swelling.
• The most common form of pseudotumor cerebri is idiopathic, with no associated factors.
• Conditions sometimes associated with pseudotumor cerebri include steroid use or discontinuation, vitamin A deficiency or excess, systemic lupus erythematosus (SLE), and anemia
• Pseudotumor cerebri occurs most often in females. Obesity is a factor in up to 90 percent of cases, with the incidence in obese women of childbearing age at 19 per 100,000. Peak incidence is in the third decade.

Symptoms

• Headache, typically worse in the morning, is the most common symptom, occurring in 94 percent of patients.
• Other symptoms include dizziness (32 percent), nausea (32 percent), visual changes (48 percent) and double vision (29 percent).
• A physical exam will find swelling of the optic nerve in 100 percent of patients, sixth cranial nerve deficit in 20 percent, and an enlarged blind spot in 60 percent.

Diagnosis

• Diagnostic criteria for pseudo tumor cerebri include elevated cerebrospinal fluid (CSF) pressure, normal CSF composition, symptoms of intracranial pressure, and a normal computed tomography (CT) or magnetic resonance imaging (MRI) scan with the exception of small ventricles.
• All patients must have thorough ophthalmologic exams to document the extent of visual deficit.

Treatment

• Treatment includes discontinuing offending drugs, weight loss, fluid and salt restrictions, use of diuretics to slow cerebrospinal fluid production and steroid treatment.
• Surgical treatment is reserved for the rare patient in whom medical management fails. Persistent symptoms or progressive visual deterioration warrant surgery. Several procedures are available
  • A series of lumbar punctures to drain CSF is a simple procedure and usually effective.
  • Lumboperitoneal shunts treat intracranial pressure elevation by draining CSF from the lumbar area into the abdomen. Side effects include persistent headaches or radiating pain down the legs.
  • Subtemporal decompression involves removing a small window of bone in the temple to allow more room for the brain. The procedure can leave patients at risk for post-operative seizures.
  • Decompression of the optic nerve sheath involves opening the sheath surrounding the optic nerve to decrease pressure on the optic nerve. This has been reported to correct the visual disturbance.

The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the NeuroICU attending physician and team members direct your family member's care while in the ICU.  The NeuroICU team consists of the bedside nurses, nurse practitioners, physicians in specialty training (Fellows) and attending physicians. UCLA Neuro ICU Family Guide