MRI-Findings in Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) MRT-Befunde Bei Der Idiopathischen Intrakraniellen Hypertension (Pseudotumor Cerebri)
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Published online: 2021-05-12 Review MRI-findings in idiopathic intracranial hypertension (Pseudotumor cerebri) MRT-Befunde bei der idiopathischen intrakraniellen Hypertension (Pseudotumor cerebri) Authors Julia Juhász, Johannes Hensler ,OlavJansen Affiliation tions, pulsatile tinnitus and nausea. In the presence of an un- Department of Radiology and Neuroradiology, University derlying cause for the increased intracranial pressure one Hospital Schleswig-Holstein – Campus Kiel, Germany speaks of a secondary intracranial hypertension. The diagnos- tics include a detailed medical history, neurological and oph- Key words thalmic examination, lumbar puncture, and neuroradiological pseudotumor cerebri, idiopathic intracranial hypertension, imaging procedures. MRI, in particular, has become increasing- secondary intracranial hypertension, MRI, MR-venography ly important in recent years, since signs for changes in cere- received 31.10.2020 brospinal fluid pressure are now detectable and well-defined. accepted 03.03.2021 The therapeutic approaches are symptom-oriented and aim to published online 2021 lower the pressure. With a precise diagnosis and timely start of therapy, idiopathic intracranial hypertension has a good prog- Bibliography nosis, especially with regard to the preservation of eyesight. Fortschr Röntgenstr DOI 10.1055/a-1447-0264 Key Points: ▪ ISSN 1438-9029 The idiopathic intracranial hypertension is an important © 2021. Thieme. All rights reserved. differential diagnosis for unspecific headache and impaired Georg Thieme Verlag KG, Rüdigerstraße 14, vision ▪ 70469 Stuttgart, Germany Overweight women in childbearing age are particularly affected Correspondence ▪ The most important component in diagnostics is MRI Dr. Julia Juhász Citation Format Radiologie und Neuroradiologie, Universitätsklinikum ▪ Juhász J, Hensler J, Jansen O. MRT-Befunde bei der idiopathi- Schleswig-Holstein, Arnold-Heller-Str. 3, 24105 Kiel, Germany schen intrakraniellen Hypertension (Pseudotumor cerebri). Tel.: +49/4 31/50 01 65 01 Fortschr Röntgenstr 2021; DOI 10.1055/a-1447-0264 [email protected] ZUSAMMENFASSUNG ABSTRACT Hintergrund Der Pseudotumor cerebri, auch idiopathische Background Pseudotumor cerebri, also known as idiopathic intrakranielle Hypertension genannt, ist eine Erkrankung mit intracranial hypertension, is a disorder of increased intracra- erhöhtem Liquordruck unklarer Genese. Leitsymptom sind nial pressure of unknown etiology. Main symptom is headache Kopfschmerzen, dessen Charakteristiken anderen Kopf- which has a characteristic similar to other headache diseases, This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. schmerzerkrankungen ähneln, weshalb die idiopathische in- therefore the idiopathic intracranial hypertension often re- trakranielle Hypertension oft unerkannt bleibt. Die Inzidenz mains undetected. The incidence is 1/100 000 with the num- wird mit 1/100 000 angegeben, wobei die Dunkelziffer we- ber of unreported cases being much higher. This article high- sentlich höher liegt. Dieser Artikel weist auf die essenzielle lights the essential role of MRI in the differential diagnosis. Rolle der MRT-Bildgebung in der Differenzialdiagnostik hin. Methods A literature search was carried out on idiopathic in- Methode Es wurde eine Literaturrecherche zu dem Syndrom tracranial hypertension and Pseudotumor cerebri in English IIH und Pseudotumor cerebri in englischer und deutscher and German. Original and Review articles as well as case re- Sprache durchgeführt. Berücksichtigt wurden sowohl Origi- ports were taken into account. Since the main focus of the nal- und Übersichtsarbeiten als auch Fallbeispiele. Da der article is MRI diagnostics, some images were selected to illus- Schwerpunkt des Artikels die MRT-Diagnostik ist, wurde eine trate the characteristic MRI morphological features. Bilderauswahl getroffen, um die charakteristischen MR-mor- Results and Conclusion The diagnosis of idiopathic intracra- phologischen Merkmale zu veranschaulichen. nial hypertension is based on an exclusion procedure. Most common syndromes are headache, transient visual obscura- Juhász J et al. MRI-findings in idiopathic… Fortschr Röntgenstr | © 2021. Thieme. All rights reserved. Review Ergebnisse und Schlussfolgerung Die Diagnose der idiopa- gische bildgebende Verfahren. Insbesondere die MRT hat in thischen intrakraniellen Hypertension beruht auf einem Aus- den letzten Jahren an Bedeutung gewonnen, da mittlerweile schlussverfahren. Die typischen klinischen Symptome sind gut erkennbare Zeichen einer Liquordruckänderung definiert Kopfschmerzen, Sehstörung, pulsatiler Tinnitus und Übelkeit. sind. Die Therapieansätze sind symptomorientiert und haben Findet man eine zugrunde liegende Ursache für den erhöhten das Ziel der Drucksenkung. Bei genauer Diagnostik und re- intrakraniellen Druck, spricht man von einer sekundären in- chtzeitigem Therapiebeginn hat die idiopathische intrakra- trakraniellen Hypertension. Die Diagnostik beinhaltet eine nielle Hypertension eine gute Prognose, besonders was die ausführliche Anamnese, neurologische und ophthalmolo- Erhaltung der Sehfähigkeit betrifft. gische Untersuchungen, Lumbalpunktion und neuroradiolo- Introduction and tension headache [11]. The latter is also the reason why IIH can easily be misdiagnosed. Physiologically, intracranial pressure in healthy adults is 5– The second most common complaint is vision disturbances, 15 cmH2O. Values above 20–25 cmH2O are considered patholo- which are described in 72 % of cases, occurring on one or both gically increased intracranial pressure (intracranial hypertension) sides and ranging from reduced visual acuity to blindness [12]. [1].ElevatedCSFpressurecanleadtosymptomssuchashead- Vision disturbances are mainly caused by papillary edema, which ache, vision disturbance, pulsatile tinnitus and nausea [2]. This can be detected in 95 % of cases during ophthalmological exami- syndrome was grouped under the name pseudotumor cerebri nations, thus vision disturbances are a good indicator of increased (PTC) and described by the German neurologist Max Nonne in CSF pressure. The remaining cases are referred to as idiopathic 1904. His aim was to differentiate this group of patients from intracranial hypertension without papilledema (IIHWOP) [13, 14]. those with increased intracranial brain pressures due to cerebral Pulsatile tinnitus is another common symptom at 60 % [12]. tumors, as they exhibited the same clinical symptoms [3]. Nowa- In addition, there are other, non-specific symptoms such as days, this definition is no longer up-to-date, since several pathol- nausea, vomiting and olfactory disorders [15, 16]. ogies, pathophysiological changes and drugs are now known to lead to an increase in intracranial pressure. If an underlying cause is found, it is referred to as secondary intracranial hypertension Pathophysiology (SIH). If the etiology remains unknown, the disease pattern is called idiopathic intracranial hypertension (IIH), a concept intro- Although aspects of this disease pattern are increasingly becom- duced in 1989 by Corbett and Thomson. ing known, the exact pathomechanism has not yet been clarified. IIH is a complex condition that is not yet fully understood, with The Monro-Kellie doctrine states that in a rigid system such as an incidence of 0.9/100 000 in Western countries [4], although it the cranium, the three components (brain tissue, blood, cere- can be assumed that unreported cases are significantly higher. On brospinal fluid) within it form a constant volume, whereby a the one hand, technical developments in recent years have signif- change in one component necessarily results in a change in the icantly improved diagnostics; on the other hand, the prevalence other two [17]. of obesity has increased worldwide [5]. There are several theories regarding the genesis of IIH. The Large differences are also observed depending on geographical common characteristic is the increased CSF pressure, but it is location; the reason for this is not yet fully understood. It seems questionable whether there is a uniform mechanism at all and that genetic predisposition plays a minor role compared to environ- not rather several pathophysiological events acting on each other, mental factors [6], although BMI provides the strongest correlation which culminate in a vicious circle and result in the syndrome. [7]. This explains the higher incidence of 19/100000 in overweight Cerebral spinal fluid women (20 % overweight). IIH can occur at any age, but the largest This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. risk group is overweight women of childbearing age [8]. Depending CSF is largely produced by the choroid plexus; its production is on the study, the female-to-male incidence ratio varies from 4:1 to pressure-independent and relatively constant, but decreases with 15:1 [9]. In children, an incidence of 0.5/100 000 was found with- age. The theory that the increased intracranial pressure is due to out gender and BMI-related differences [10]. an overproduction of cerebrospinal fluid could not be proven [18]. Dysregulation in water transport may play a role in the patho- mechanism, as the water channel protein aquaporin-4 is known to Symptomology be associated with brain edema and, together with aquaporin-1, forms the therapeutic approach to drug therapy. Thus, downregu- Clinical symptoms are caused by increased intracranial pressure lation of these channels can lead to symptom