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20 year old woman with back pain, neck pain, and blurry vision OS
Digital Journal of Ophthalmology 1997
Volume 3, Number 25
September 7, 1997
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Yichieh Shiuey, MD | Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA
Diagnosis and Discussion
Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

Because of the bilateral chronic appearing disk edema the patient was suspected to have increased intracranial pressure. When the MRI showed no mass lesions, idiopathic intracranial hypertension was felt to be the most likely cause of this patients signs and symptoms. This diagnosis was confirmed by a lumbar puncture which showed a CSF pressure of > 300 mm of water ( nl < 200 mm of water). The CSF composition was also found to be normal.

Pseudotumor cerebri can affect patients of any age including children. However, the peak age is in the third decade. Approximately 90% of patients are women and most patients are obese. (Our patient was only slightly obese)

Clinical Features:
Initial symptoms in this condition include severe headache and disturbances in vision. Patients may also complain of transient obscurations in their vision lasting seconds. Double vision may also occur as a result of an associated 6th nerve palsy due to increased intracranial pressure. Any other cranial nerve deficit or neurological problem would be highly unusual in pseudotumor cerebri and should make a clinician suspicious of the diagnosis. Decreased vision in the form of substantial visual field loss or decreased acuity may occur in the setting of advanced disease.

Diagnosis of this condition requires 1) increased intracranial pressure on lumbar puncture (>200 mm of water), 2) a "normal" head imaging scan (findings which can be seen in pseudotumor cerebri, but which are often considered within nomrla limits are small ventricles, an enlarged CSF sapce around the optic nerves, and an empty sella turcica) and 3) normal CSF composition

For obese patients, treatment begins with a weight control program since decreasing weight has been shown to decrease the intracranial pressure in this condition. Medical management generally consists of acetazolamide 500 mg to 2 gm P.O. qd. Furosemide and steroids are also occasionally used. When visual field loss occurs in the face of medical management or if there is significant visual field loss at presentation, optic nerve sheath fenestration is indicated. Not infrequently, unilateral optic nerve sheath fenestration may result in decrease of optic disk edema in both eyes.

Clinical Course:
Our patient underwent optic nerve sheath fenestration within 48 hours of her presentation because of her severe visual field loss and concern that she could lose her central acuity. Over the subsequent weeks her visual fields expanded substantially OU and showed marked decrease in her disk edema in both eyes.