MRI-findings in idiopathic intracranial hypertension (Pseudotumor cerebri)

Abstract

Background Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a disorder of increased intracranial pressure of unknown etiology. Main symptom is headache which has a characteristic similar to other headache diseases, therefore the idiopathic intracranial hypertension often remains undetected. The incidence is 1/100 000 with the number of unreported cases being much higher. This article highlights the essential role of MRI in the differential diagnosis. Methods A literature search was carried out on idiopathic intracranial hypertension and Pseudotumor cerebri in English and German. Original and Review articles as well as case reports were taken into account. Since the main focus of the article is MRI diagnostics, some images were selected to illustrate the characteristic MRI morphological features. Results and Conclusion The diagnosis of idiopathic intracranial hypertension is based on an exclusion procedure. Most common syndromes are headache, transient visual obscurations, pulsatile tinnitus and nausea. In the presence of an underlying cause for the increased intracranial pressure one speaks of a secondary intracranial hypertension. The diagnostics include a detailed medical history, neurological and ophthalmic examination, lumbar puncture, and neuroradiological imaging procedures. MRI, in particular, has become increasingly important in recent years, since signs for changes in cerebrospinal fluid pressure are now detectable and well-defined. The therapeutic approaches are symptom-oriented and aim to lower the pressure. With a precise diagnosis and timely start of therapy, idiopathic intracranial hypertension has a good prognosis, especially with regard to the preservation of eyesight. Key Points: Citation Format

Higher cerebrospinal fluid (CSF) opening pressure in patients with idiopatic intracranial hypertension (IIH) with permanent visual impairment

“…The frequency of MRI abnormalities of nearly 80% of the cases in our study is close to what has been shown in previous studies. 23 , 24 MRI is a very useful method to aid in the diagnosis of this entity, however, its role in the prognostic assessment of IIH remains undefined.…”

The Monro-Kellie Doctrine: A Review and Call for Revision

“…Nonne's intent was to propose a state of elevated intracranial pressure that was distinct from that related to cerebral tumors: intercranial hypertension that followed a less sinister course. 11 John Foley 12 later offered the term "benign intracranial hypertension. "…”

Pediatric tinnitus: The role of neuroimaging

“…Dural venous sinus stenoses or thromboses (Figure 5) were described in patients with clinical features of IIH. It is still not clear if increased intracranial venous pressure is a cause or a consequence of elevated intracranial pressures with contradictory results in the literature 16 . Therefore, MRV or CTV is recommended in patients with pulsatile tinnitus and a clinical suspicion of IIH to evaluate for dural venous sinus stenosis or thrombosis 8 .…”

“…It is still not clear if increased intracranial venous pressure is a cause or a consequence of elevated intracranial pressures with contradictory results in the literature. 16 Therefore, MRV or CTV is recommended in patients with pulsatile tinnitus and a clinical suspicion of IIH to evaluate for dural venous sinus stenosis or thrombosis. 8 Additional MRI findings of IIH include an empty sella and evidence of papilledema, prominent optic nerve sheaths, flattening of the posterior globe, and tortuous optic nerves (Figure 6).…”

“…IIH can be managed medically by drugs such as acetazolamides and furosemide or through invasive methods including serial lumbar puncture, cerebrospinal fluid (CSF) diversion procedures (lumboperitoneal or ventriculoperitoneal shunt), optic nerve fenestration, and stent angioplasty of the stenotic sinuses 16 …”