eMedicine Specialties > Ophthalmology > Neurologic Disorders

Idiopathic Intracranial Hypertension: Follow-up

Author: Mark S Gans, MD, Associate Professor, Director of Neuro-Ophthalmology, Department of Ophthalmology, McGill University; Clinical Director, Department of Ophthalmology, Adult Sites, McGill University Hospital Center, Interim Chairman of the Department of Ophthalmology, McGill University
Contributor Information and Disclosures

Updated: Aug 18, 2009

Follow-up

Further Outpatient Care

• The frequency of the follow-up visits is determined by a number of factors, to include the following:
  • Initial visual function of the patient
  • Underlying disease causing increased intracranial pressure
  • Perceived compliance of the patient with respect to medical therapy
• Once the initial diagnosis has been established, investigations have been performed, and the therapy has been initiated, the patient can be observed every 3-4 weeks. However, patients who present with a significant visual function deficit or marked papilledema should be monitored daily for a week until they demonstrate some improvement and subsequent stability in their visual function. The clinician should be prepared to titrate the patient's treatment to the status of the visual function and should not hesitate to refer the patient for surgical treatment (optic nerve sheath fenestration or a neurosurgical shunting procedure) in the absence of stabilization of the visual function. 
• During follow-up visits, the best-corrected visual acuity for distant and near vision, color vision (using pseudoisochromatic plates), static perimetry, and optic nerve appearance (including the status of spontaneous venous pulsations) should be recorded. Patients who do not perform well on static perimetry testing may be better followed with kinetic perimetry testing. The spontaneous pulsation of large retinal veins generally indicates a normal intracranial pressure. If the patient continues to remark on the persistence of a significant headache despite the presence of spontaneous venous pulsations, evaluating a source other than idiopathic intracranial hypertension for the headache is important.
• When a patient appears to have stabilized with respect to visual function and treatment, follow-up visits can extend to once every 2-4 months.

Complications

• The main complication of this disorder is progressive optic neuropathy. Despite timely treatment, some patients develop an optic neuropathy related to the optic nerve edema.
• Generally, this dysfunction presents in a progressive fashion with constriction of the peripheral visual field; worsening nerve fiber bundle visual field defects; a decrease of color vision; and, in end-stage disease, a drop in the central visual function.
• Occasionally, a patient may develop an acute loss of vision due to ischemic optic neuropathy or a retinal vascular occlusion associated with the papilledema.

Prognosis

• The visual prognosis in timely and appropriately treated patients can be encouraging in cases of idiopathic intracranial hypertension.
• Unfortunately, the incidence of visual loss has been reported to be significant in some studies of this disease. Corbett et al documented visual dysfunction in close to one half of patients with idiopathic intracranial hypertension.⁹
• Since the increase in intracranial pressure tends to be chronic in nature, all patients with this disorder must be monitored for years after presentation. If necessary, medical treatment should be continued on a long-term basis.

Patient Education

• Informing patients who are overweight that weight control is a long-term factor in idiopathic intracranial hypertension is important. Asking patients about their weight loss at the beginning of each visit reinforces this concept. In addition, it may be worthwhile to mention that the loss of as little as 6% of body weight may lead to the termination of this disorder and also may significantly diminish the risk of its recurrence.
• Although the disease may appear to be self-limiting, it is considered to be a chronic disorder; therefore, once the medication is tapered off, patients should be alerted to return to an ophthalmologist if symptoms of increased intracranial pressure recur.
• If a particular agent, such as tetracycline, is associated with the rise in intracranial pressure, the patient should be educated to avoid this agent.

Miscellaneous

Medicolegal Pitfalls

• Although idiopathic intracranial hypertension typically develops in young females who are overweight, the disease does occur in males and in patients who are not overweight.  To complicate matters, some patients may present with idiopathic intracranial hypertension who have either asymmetric optic nerve swelling or the absence of optic nerve edema in either eye.
• When a patient presents with bilateral disc edema, the diagnosis is presumably an intracranial mass until proven otherwise. A lumbar puncture should not be performed until the findings on neuroimaging studies have eliminated the possibility of a space-occupying lesion. A lumbar puncture performed in the presence of an intracranial mass may result in the fatal occurrence of herniation of the temporal lobe or the cerebellum. Thus, reviewing the images prior to performing a lumbar puncture is essential to avoid this outcome.
• In addition to ruling out a mass lesion, ensuring that patients with papilledema do not have a dural sinus thrombosis is important. Although a high-quality CT scan or MRI with infusion may eliminate this possibility, magnetic resonance venography is preferable.
• If the findings on neuroimaging studies are normal and a lumbar puncture indicates increased intracranial pressure in a patient with papilledema, long-term monitoring for progressive optic neuropathy is indicated.
• Although the disease may appear to be self-limiting, it may recur and subsequently result in severe visual loss.
• Patients who are administered acetazolamide should be aware of both the precautions and the adverse reactions listed in Medication.

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