Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)
Treating Idiopathic Intracranial Hypertension
Michael Wall, MD
Department of Ophthalmology and Visual Sciences
University of Iowa
Peer Review Status: Internally Peer Reviewed
Creation Date: 1991
Last Revision Date: 2001
Treatment for patients with IIH can be divided into medical treatment
and surgical treatment. The cornerstone of medical treatment is weight
loss. It does not appear to be the total number of pounds lost. Some
patients are effectively treated by losing one pound every week or two
for several months and then maintaining the weight loss. It may be the
loss of fluid accompanying weight loss that is the significant factor
but this has not been proven.
Loss of fluid can also be obtained using diuretics
(fluid pills). Diamox (acetazolamide) is the most commonly used medication.
It is relatively safe but nearly all patients have tingling of the fingers
and toes. This tingling is a benign symptom. Patients also experience
that carbonated soft drinks taste metallic. Less commonly, kidney stones
can occur and rarely other blood disorders. Another diuretic commonly
used that appears to be effective in some patients is Lasix (furosemide).
More about Diamox.
The surgical treatments currently used are optic nerve sheath fenestration
(making slits in the optic nerve sheath or covering) and
lumbar shunting procedures (running a tube from the spinal fluid space
in the lower spine into the abdominal cavity). These procedures are
used when patients do not respond adequately to medical therapy. Optic
nerve sheath fenestration is done first by an incision into the orbit.
The eyeball is moved to the side and the optic nerve sheath is exposed.
Slits or a large hole are then placed in the optic nerve sheath and
fluid drains out, thereby taking pressure off the optic nerve.
The second surgical procedure, called lumbar-peritoneal shunting, is
done as follows. Tubing is placed in the spinal fluid space, (the space
entered during a lumbar puncture or spinal tap and tubing is then run
to the abdomen. This lowers the pressure around the brain and optic
nerve, thereby eliminating the symptoms of raised intracranial pressure.
Unfortunately, these procedures are complicated by various problems,
the most severe one being some patients have periodic occlusion of the
tubing with recurrence of symptoms and sometimes vision loss. A repeat
operation is then needed. An overview of treatment is summarized in
figure 12.
Figure 12. Treatment strategies for IIH.
Management of the pregnant IIH patient
Pregnancy occurs in IIH as often as in the general population
and in any trimester. Patients with IIH during pregnancy do not have
an increased spontaneous abortion rate. Therapeutic abortion to limit
progressive disease is not indicated. The pregnant patient with IIH
should be treated as any other patient with IIH. Also, the pregnancy
should be managed like any other. The major exception is caloric restriction
because of its adverse effect of ketosis on the fetus. Weight gain
can be limited to 20 pounds.
Use of corticosteroids has not been associated with
birth defects in humans. Acetazolamide may be used after 20 weeks
gestation; use before 20 weeks has been associated with one case of
sacrococcygeal teratoma. Glycerol and thiazide diuretics probably
should not be used in the second half of pregnancy because of the
risk of decrease in placental blood flow. There is no obstetric contraindication to surgery for those that require it.
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