NORD gratefully acknowledges Bradley K. Farris, MD, Professor, Department of Ophthalmology, Adjunct Professor, Department of Neurology & Neurosurgery, University of Oklahoma School of Medicine; and Mark Robinson, MD, Neuro-Ophthalmology Fellow, Department of Neurology & Neurosurgery, University of Oklahoma School of Medicine, for assistance in the preparation of this report.
The most common symptom is often an unbearably painful headache, sometimes associated with nausea and vomiting that is not relieved by medication. The headache often awakens the patient from sleep. Some patients are treated in the emergency room where a lumbar puncture (spinal tap) is done as a last resort, to temporarily ease the headache. Measurement of the opening pressure is encouraged during these procedures.
The diagnosis is also confirmed by detecting a high spinal CSF pressure reading, usually greater than 250 mmH2O (200-250 considered borderline high) and normal laboratory and imaging studies including CT scans and MRIs. There is generally a normal neurological examination as well, although abnormal findings may be detected on eye examination. The eye findings may be subtle, and not noted in an emergency room evaluation. It is not uncommon to misdiagnose a patient with IH as simply having a refractory migraine headache, and be treated as such. Unlike Primary IH, Secondary IH patients may have abnormal scans and laboratory tests.
The high CSF pressure may cause the optic nerves to swell (papilledema). The optic nerve connects the interior of each eye, the retina, to the vision centers of the brain. The optic nerve transmits impulses from the retina to these brain centers. The earliest sign of papilledema on a visual field test is known as an enlarged blind spot. Abnormal CSF pressure can also affect the eye muscles controlling eye movements producing double vision, but this is an infrequent event. (All patients with presumed IH should have a thorough eye examination including visual field tests by an ophthalmologist or neuro-ophthalmologist).
Other common symptoms include transient altered vision, particularly on movement or bending over, intracranial noise (pulse synchronous tinnitus), stiff neck, back and arm pain, pain behind the eye, exercise intolerance, and memory difficulties.
In the idiopathic or primary type (IIH), obesity is a significant factor in women aged 20-35.
The many potential causes of secondary intracranial hypertension have been noted above. Note that in secondary IH, unlike IIH, obesity, gender, age and race are NOT risk factors, but may be present.
The mechanism by which IH occurs is not known, but several possibilities have been suggested. Most research supports the theory that there is resistance or obstruction to CSF outflow through the normal existing pathways in the brain.
The incidence if IIH in the general population is thought to be about 1 per 100,000. In obese young females the incidence of IIH is about 20 per 100,000. IIH occurs in men and children as well, but with substantially lower frequency. Weight is not usually a factor in men and in children under 10 years of age.
The true incidence of secondary IH remains unknown because of the wide range of underlying causes and the lack of published surveys on the subject. Current statistics are not available on how many people have secondary intracranial hypertension.
Medical treatment consists of using drugs called carbonic anhydrase inhibitors to suppress the production of CSF. The most commonly used of the carbonic anhydrase inhibitors is acetazolamide. A large multicenter, randomized, controlled trial published in 2014 demonstrated that acetazolamide combined with dietary weight loss resulted in improved visual field function, nerve swelling, and quality of life measures, compared to the treatment of dietary changes alone. Carbonic anhydrase inhibitors inhibit the enzyme system needed to produce CSF and control the pressure (by controlling the volume) to some degree. These drugs do not work in all cases and can have potentially serious side effects.
When medical treatment fails and vision is at risk, one of two types of surgery may be performed, optic nerve sheath fenestration or neurosurgical shunt. Optic nerve fenestration is a procedure in which a small opening is made in the sheath around the optic nerve in an attempt to relieve swelling (papilledema). Optic nerve sheath fenestration has a high rate of success in protecting vision, but usually does not significantly reduce headache. Implantation of neurosurgical shunts (internal tubes) is used to drain CSF into other areas of the body. These shunts protect vision and reduce headache, but typically have a higher complication rate than optic nerve sheath fenestration.
Studies of the treatment of intracranial hypertension, begun at SUNY Upstate Medical Center are being continued in the laboratories of:
Deborah I. Friedman, MD
Department of Ophthalmology
University of Rochester School of Medicine & Dentistry
Rochester, NY 14642
The Intracranial Hypertension Research Foundation together with the Casey Eye Institute of the Oregon Health Sciences University maintain the IH Registry in which IH patients may voluntarily register. Registrants may participate (only with informed consent) in surveys and receive almost immediate news of clinical trials in which registrants may participate.
Because IH is a rare disorder affecting so few patients a central register is key to gathering epidemiological data and to notifying patients of a clinical trial of interest.
Interested persons can receive more information by contacting:
The Intracranial Hypertension Research Foundation
6517 Buena Vista Drive
Vancouver, WA 98661
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
For information about clinical trials conducted in Europe, contact:
Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology. 2002;58:1551-3.
Digre KB. Idiopathic intracranial hypertension headache. Curr Pain Headache Rep. 2002;6:217-25.
Shin RK, Balcer LJ. New developments in idiopathic intracranial hypertension. Curr Neurol Neurosci Rep. 2001;1:463-70.
Kosmorsky G. Pseudotumor cerebri. Neurosurg Clin N Am. 2001;12:775-97.
Digre KB, Corbett JJ. Idiopathic intracranial hypertension (pseudotumor cerebri): a reappraisal. The Neurologist. 2001;7:2-67.
Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology. 2000;107:1907-12.
Corbett JJ. Pseudotumor cerebri by any other name. Arch Ophthalmol. 2000;118:850-51.
Kleinschmidt J, Digre KB, Hanover R. Idiopathic intracranial hypertension: relationship to depression, anxiety and quality of life. Neurology. 2000;54:319-24.
Zemeck G, Romner B. Seven years of clinical experience with the programmable Codman-Hakin valve: a retrospective study of 583 patients. J Neurosurg. 2000;92:941-48.
Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment and outcomes. Am J Ophthalmol. 1999;127:178-82.
Friedman DI. Pseudotumor cerebri. Neurosurg Clin N Am. 1999;10:609-21.
Brodsky MC, Vaphiades MD. Magnetic resonance imaging in pseudotumor cerebri . Ophthalmology. 1998;105:1686-93.
Friedman DI, Streeten DH. Idiopathic intracranial hypertension and orthostatic edema may share a common pathogenesis. Neurology. 1998;50:1099-1104.
Sismanis A. Pulsatile tinnitus. A 15 year experience. Am J Otol. 1998;19:472-77.
Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997;49:734-39.
Lee AG. Pseudotumor cerebri after treatment with tetracycline and isotretinoin for acne. Cutis. 1995;55:165-68.
Rosenberg ML, Corbett JJ, Smith C, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology. 1993;43:1071-72.
Spoor TC, McHenry JG. Long term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993;111:632-35.
Wall M, George D. Idiopathic intracranial hypertension. Brain. 1991;114:155-80.
Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia. 1990;10:331-35.
Corbett JJ, Thomson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol. 1989;46:1049-51.
Digre KB, Corbett JJ. Pseudotumor cerebri in men. Arch Neurol. 1988;45:866-72.
Round R, Keane JR. The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology. 1988;38:1461-64.
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