An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management

Toscano, Simona; Fermo, Salvatore Lo; Reggio, Ester; Chisari, Clara Grazia; Patti, Francesco Paolo; Zappia, Mario

doi:10.1007/s00415-020-09943-9

Cited by 58 publications

(65 citation statements)

References 156 publications

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“…Eligibility for idiopathic intracranial hypertension cohort categorization required a diagnosis of idiopathic intracranial hypertension as per pre-established criteria, including a lumbar puncture opening pressure of $25 cm H 2 0 in the left lateral decubitus position and preprocedural clinical evaluation for papilledema by an ophthalmologist. 3 Categorization into the pulsatile tinnitus cohort consisted of several strict criteria. Patients with clinical findings consistent with pulsatile tinnitus from a venous origin and imaging findings of lateral venous sinus stenosis of at least 50% either ipsilateral to the pulsatile tinnitus or within the dominant sinus were included in the pulsatile tinnitus cohort.…”

Section: Methodsmentioning

confidence: 99%

“…Thus, blockage or severe stenoses of the dural venous sinuses responsible for draining intracranial venous circulation has the potential to limit CSF re-absorption, therefore leading to gradual intracranial CSF buildup with subsequent elevations of intracranial pressure and resultant symptomology as reported in the literature. [1][2][3][4] Although pulsatile tinnitus has been associated with idiopathic intracranial hypertension, its unique manifestation in either the setting of idiopathic intracranial hypertension or in isolation remains a clinical enigma. This vascular somatosound has been reported in the setting of venous sinus stenosis, lateral sinus wall dehiscence with associated venous sinus aneurysms, and prominent collateral circulation via mastoid, condylar, or occipital emissary veins.…”

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confidence: 99%

“…FIG3. Objective parameters implemented in recording corollary findings of both idiopathic intracranial hypertension and pulsatile tinnitus cohorts.…”

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confidence: 99%

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Dural Venous Sinus Stenosis: Why Distinguishing Intrinsic-versus-Extrinsic Stenosis Matters

Sundararajan

Ramos

Kishore³

et al. 2021

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: Dural venous sinus stenosis has been associated with idiopathic intracranial hypertension and isolated venous pulsatile tinnitus. However, the utility of characterizing stenosis as intrinsic or extrinsic remains indeterminate. The aim of this retrospective study was to review preprocedural imaging of patients with symptomatic idiopathic intracranial hypertension and pulsatile tinnitus, classify the stenosis, and assess a trend between stenosis type and clinical presentation while reviewing the frequencies of other frequently seen imaging findings in these conditions. MATERIALS AND METHODS:MRVs of 115 patients with idiopathic intracranial hypertension and 43 patients with pulsatile tinnitus before venous sinus stent placement were reviewed. Parameters recorded included the following: intrinsic or extrinsic stenosis, prominent emissary veins, optic nerve tortuosity, cephalocele, sella appearance, poststenotic fusiform enlargement versus saccular venous aneurysm, and internal jugular bulb diverticula. x 2 cross-tabulation statistics were calculated and recorded for all data.RESULTS: Most patients with idiopathic intracranial hypertension (75 of 115 sinuses, 65%) had extrinsic stenosis, and most patients with pulsatile tinnitus (37 of 45 sinuses, 82%) had intrinsic stenosis. Marked optic nerve tortuosity was more common in idiopathic intracranial hypertension. Cephaloceles were rare in both cohorts, with an increased trend toward the presence in idiopathic intracranial hypertension. Empty sellas were more common in idiopathic intracranial hypertension. Cerebellar tonsils were similarly located at the foramen magnum level in both cohorts. Saccular venous aneurysms were more common in pulsatile tinnitus. Internal jugular bulb diverticula were similarly common in both cohorts. CONCLUSIONS:In this cohort, most patients with idiopathic intracranial hypertension had extrinsic stenosis, and most patients with pulsatile tinnitus had intrinsic stenosis. Awareness and reporting of these subtypes may reduce the underrecognition of potential contributory stenoses in a given patient's idiopathic intracranial hypertension or pulsatile tinnitus.

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Section: Methodsmentioning

confidence: 99%

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confidence: 99%

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Dural Venous Sinus Stenosis: Why Distinguishing Intrinsic-versus-Extrinsic Stenosis Matters

Sundararajan

Ramos

Kishore³

et al. 2021

AJNR Am J Neuroradiol

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“…Weight loss is an effective treatment [2]. The precise pathogenesis is not known [3], and hence it is also known as idiopathic intracranial hypertension (IIH). It can manifest as a variety of symptoms, including headache, visual disturbances, and tinnitus.…”

Section: Introductionmentioning

confidence: 99%

Pseudotumor Cerebri with Blindness

Kwak

Delk

Stead

et al. 2021

Cureus

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Pseudotumor cerebri, or idiopathic intracranial hypertension (IIH), is a syndrome of elevated intracranial pressure (ICP) of unknown etiology that occurs predominantly in obese women of childbearing age. Pseudotumor cerebri literally means "false brain tumor". It is a "diagnosis of exclusion" therefore a complete work-up to rule out life-threatening causes for increased ICP must be performed through a comprehensive history, complete physical examination, diagnostic imaging, and cerebrospinal fluid (CSF) analysis before the diagnosis can be made. The authors present the case of a young woman with headache, and near blindness due to pseudotumor cerebri. The presentation, diagnosis, and treatment options are discussed.

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“…Idiopathic intracranial hypertension (IIH) is a neurological disorder of unknown etiology characterized by increased intracranial pressure (ICP) and normal composition of cerebrospinal fluid (CSF) with elevated opening pressure (OP) [1]. The pathophysiology is still ambiguous and supposed to be due to CSF dysregulation [2]. IIH commonly affects obese women in childbearing age; however, it can affect men, children, and non-obese individuals [3].…”

Section: Introductionmentioning

confidence: 99%

Diagnostic value of brain MR imaging and its correlation with clinical presentation and cognitive functions in idiopathic intracranial hypertension patients

Elmaaty

Zarad

Belal

et al. 2021

Egypt J Neurol Psychiatry Neurosurg

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Background Idiopathic intracranial hypertension (IIH) is a neurological disorder of unknown etiology and ambiguous pathophysiology due to cerebrospinal fluid dysregulation. This study is designed to evaluate the role of brain magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in diagnosis of IIH, to clarify the nature and extent of cognitive deficits, and to detect if there is a correlation between radiology, clinical findings, and cognitive dysfunctions in those patients. Results The study included 34 patients and 34 age-, sex-, body mass index (BMI)-, and education-matched healthy control subjects. MR brain imaging and Montreal cognitive assessment (MoCA) test were used for both groups. MRI and MRV sensitivity for IIH diagnosis were 85.2% and 85.3% with 100% and 94.1% specificity respectively. 44.1% had cognitive impairment, memory was the most affected domain, followed by attention, abstraction, and orientation with statistically significantly lower total MoCA score (p < 0.005). Domain comparisons reveal a statistically significantly lower memory/delayed recall (p < 0.001) and abstract scores (p < 0.007) in IIH cases versus control subjects. In comparing patients with cognitive impairment (CI) versus those without CI, there were statistically significantly higher CI in low education level, presence of diplopia, hormonal contraceptive use, abnormal MRI brain, papilledema grades, BMI, and opening pressure. Conclusions Presence of more than or equal 3 MR imaging findings, bilateral transverse sinus stenosis, and less than or equal 4 combined conduit score increase the specificity and sensitivity of MRI and MRV for IIH diagnosis. IIH had detrimental effect on different cognitive domains especially when patient have low education level, diplopia, papilledema ≥ grade III, high OP ≥ 61.5 cm H2O, and BMI ≥ 34 Kg/m2 with abnormal MRI and MRV findings.

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An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management

Cited by 58 publications

References 156 publications

Dural Venous Sinus Stenosis: Why Distinguishing Intrinsic-versus-Extrinsic Stenosis Matters

Dural Venous Sinus Stenosis: Why Distinguishing Intrinsic-versus-Extrinsic Stenosis Matters

Pseudotumor Cerebri with Blindness

Diagnostic value of brain MR imaging and its correlation with clinical presentation and cognitive functions in idiopathic intracranial hypertension patients

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