Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects

Abstract

Idiopathic intracranial hypertension (IIH) is an uncommon disorder characterized by increased intracranial pressure without radiological or laboratory evidence of intracranial pathology except empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. Persistent headache is the most common symptom. Visual impairment is a serious complication that may not be recognized by the patients. This paper reviews clinical manifestations, diagnostic challenges, and current treatments of IIH in adults. Various imaging modalities have been studied on their validity for detection of IIH and papilledema. This review also includes new studies on medical, surgical, and interventional management of this condition. Acetazolamide and topiramate are the only two medications that have been studied in randomized controlled trials about their efficacy in treatment of IIH. In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. The efficacy and complications of cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in the last 3 decades have been summarized in this review. Finally, the prospective aspects of biomarkers and treatments are proposed for future research.

Shunt Surgery in Idiopathic Intracranial Hypertension Aided by Electromagnetic Navigation

“…Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri, is a rare but potentially devastating disorder with a female preponderance and often associated with obesity [1,2,3]. It is characterized by increased cerebrospinal fluid (CSF) pressure with normal or even slit ventricles on imaging studies [4].…”

Headache in the Emergency Room

“…40 Headache is nearly always present, and papilledema is suggestive of diagnosis. [40][41] As the name implies, the process is idiopathic and diagnosis is based on exclusion of other causes of raised ICP such as mass lesions or medication-induced pseudotumor cerebri. LP is routinely performed for diagnosis, with CSF pressure >20 cm H 2 0 as diagnostic, or >25 cm H 2 0 in obese patients.…”

“…Rare reports of tonsillar herniation, however, have been reported after LP in patients with IIH. 41 Patients are treated with oral diuretics, such as acetazolamine or topiramate, as well as being referred to an ophthalmologist for formal visual field testing.…”

Efficacy and Safety of Optic Nerve Sheath Fenestration in Patients with Raised Intracranial Pressure

“…IIH is a separate entity, mainly a diagnosis of exclusion, characterized by papilledema without identifiable neurological pathology, and is common in obese women of child bearing age 2. Since the optic nerve sheath can be traced as continuation of the subarachnoid space, transmission of effects of raised ICP to the optic nerve causes disruption of the axoplasmic flow, swelling of axons, leakage of water and proteins with resultant optic disc swelling, or papilledema 3,4. Papilledema, if left untreated, causes significant and irreversible visual loss, with Visual Field (VF) defects and loss of contrast sensitivity 5…”

“…IIH is mainly treated by both medical and surgical options. Medical treatment includes Acetazolamide, Steroids, Topiramate, Frousemide and surgical treatment options include Optic Nerve Sheath Fenestration (ONSF) or CSF shunt (lumboperitoneal shunts, ventriculo-peritoneal shunts, or ventriculo-atrial shunt) 3,6,7…”