Rofo 2021; 193(11): 1269-1276
DOI: 10.1055/a-1447-0264
Review

MRT-Befunde bei der idiopathischen intrakraniellen Hypertension (Pseudotumor cerebri)

Article in several languages: English | deutsch
Julia Juhász
Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein – Campus Kiel, Germany
,
Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein – Campus Kiel, Germany
,
Olav Jansen
Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein – Campus Kiel, Germany
› Author Affiliations

Zusammenfassung

Hintergrund Der Pseudotumor cerebri, auch idiopathische intrakranielle Hypertension genannt, ist eine Erkrankung mit erhöhtem Liquordruck unklarer Genese. Leitsymptom sind Kopfschmerzen, dessen Charakteristiken anderen Kopfschmerzerkrankungen ähneln, weshalb die idiopathische intrakranielle Hypertension oft unerkannt bleibt. Die Inzidenz wird mit 1/100 000 angegeben, wobei die Dunkelziffer wesentlich höher liegt. Dieser Artikel weist auf die essenzielle Rolle der MRT-Bildgebung in der Differenzialdiagnostik hin.

Methode Es wurde eine Literaturrecherche zu dem Syndrom IIH und Pseudotumor cerebri in englischer und deutscher Sprache durchgeführt. Berücksichtigt wurden sowohl Original- und Übersichtsarbeiten als auch Fallbeispiele. Da der Schwerpunkt des Artikels die MRT-Diagnostik ist, wurde eine Bilderauswahl getroffen, um die charakteristischen MR-morphologischen Merkmale zu veranschaulichen.

Ergebnisse und Schlussfolgerung Die Diagnose der idiopathischen intrakraniellen Hypertension beruht auf einem Ausschlussverfahren. Die typischen klinischen Symptome sind Kopfschmerzen, Sehstörung, pulsatiler Tinnitus und Übelkeit. Findet man eine zugrunde liegende Ursache für den erhöhten intrakraniellen Druck, spricht man von einer sekundären intrakraniellen Hypertension. Die Diagnostik beinhaltet eine ausführliche Anamnese, neurologische und ophthalmologische Untersuchungen, Lumbalpunktion und neuroradiologische bildgebende Verfahren. Insbesondere die MRT hat in den letzten Jahren an Bedeutung gewonnen, da mittlerweile gut erkennbare Zeichen einer Liquordruckänderung definiert sind. Die Therapieansätze sind symptomorientiert und haben das Ziel der Drucksenkung. Bei genauer Diagnostik und rechtzeitigem Therapiebeginn hat die idiopathische intrakranielle Hypertension eine gute Prognose, besonders was die Erhaltung der Sehfähigkeit betrifft.

Kernaussagen:

  • Die idiopathische intrakranielle Hypertension ist eine wichtige Differenzialdiagnose bei unspezifischen Kopfschmerzen und Sehstörungen.

  • Betroffen sind v. a. übergewichtige Frauen im gebärfähigen Alter.

  • Wichtigster Baustein in der Diagnostik ist die MRT.

Zitierweise

  • Juhász J, Hensler J, Jansen O. MRI-findings in idiopathic intracranial hypertension (Pseudotumor cerebri). Fortschr Röntgenstr 2021; 193: 1269 – 1276



Publication History

Received: 31 October 2020

Accepted: 03 March 2021

Article published online:
12 May 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Bono F, Cristiano D, Mastrandrea C. et al. The upper limit of normal CSF opening pressure is related to bilateral transverse sinus stenosis in headache sufferers. Cephalalgia: an international journal of headache 2010; 30: 145-151
  • 2 Thurtell MJ, Bruce BB, Newman NJ. et al. An update on idiopathic intracranial hypertension. Reviews in neurological diseases 2010; 7: e56-e68
  • 3 Rohr A. Pseudotumor cerebri. Neuroradiol Scan 2013; 3: 267-284
  • 4 Ball AK, Clarke CE. Idiopathic intracranial hypertension. The Lancet Neurology 2006; 5: 433-442
  • 5 Kilgore KP, Lee MS, Leavitt JA. et al. Re-evaluating the Incidence of Idiopathic Intracranial Hypertension in an Era of Increasing Obesity. Ophthalmology 2017; 124: 697-700
  • 6 Bruce BB, Preechawat P, Newman NJ. et al. Racial differences in idiopathic intracranial hypertension. Neurology 2008; 70: 861-867
  • 7 Alperin N, Ranganathan S, Bagci AM. et al. MRI evidence of impaired CSF homeostasis in obesity-associated idiopathic intracranial hypertension. AJNR. American journal of neuroradiology 2013; 34: 29-34
  • 8 Kosmorsky GS. Idiopathic intracranial hypertension: pseudotumor cerebri. Headache 2014; 54: 389-393
  • 9 Skau M, Brennum J, Gjerris F. et al. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia: an international journal of headache 2006; 26: 384-399
  • 10 Tibussek D, Distelmaier F, von Kries R. et al. Pseudotumor cerebri in childhood and adolescence -- results of a Germany-wide ESPED-survey. Klinische Padiatrie 2013; 225: 81-85
  • 11 Bono F, Messina D, Giliberto C. et al. Bilateral transverse sinus stenosis predicts IIH without papilledema in patients with migraine. Neurology 2006; 67: 419-423
  • 12 Wall MGD. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991; 114: 155-180
  • 13 Messina D, Bono F, Fera F. et al. Empty sella and bilateral transverse sinus stenosis predict raised intracranial pressure in the absence of papilloedema: a preliminary study. Journal of neurology 2006; 253: 674-676
  • 14 Digre KB, Nakamoto BK, Warner JEA. et al. A comparison of idiopathic intracranial hypertension with and without papilledema. Headache 2009; 49: 185-193
  • 15 Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59: 1492-1495
  • 16 Khoo KF, Kunte H. Olfactory dysfunction in patients with idiopathic intracranial hypertension. Neurology 2014; 82: 189
  • 17 Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology 2001; 56: 1746-1748
  • 18 McGeeney BE, Friedman DI. Pseudotumor cerebri pathophysiology. Headache 2014; 54: 445-458
  • 19 Mollan SP, Ali F, Hassan-Smith G. et al. Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management. Journal of neurology, neurosurgery, and psychiatry 2016; 87: 982-992
  • 20 Karahalios DG, Rekate HL, Khayata MH. et al. Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies. Neurology 1996; 46: 198-202
  • 21 Lenck S, Radovanovic I, Nicholson P. et al. Idiopathic intracranial hypertension: The veno glymphatic connections. Neurology 2018; 91: 515-522
  • 22 King JO, Mitchell PJ, Thomson KR. et al. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology 2002; 58: 26-30
  • 23 Farb RI, Vanek I, Scott JN. et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003; 60: 1418-1424
  • 24 Higgins JNP, Pickard JD. Lateral sinus stenoses in idiopathic intracranial hypertension resolving after CSF diversion. Neurology 2004; 62: 1907-1908
  • 25 Buell TJ, Raper DMS, Pomeraniec IJ. et al. Transient resolution of venous sinus stenosis after high-volume lumbar puncture in a patient with idiopathic intracranial hypertension. Journal of neurosurgery 2018; 129: 153-156
  • 26 Rohr A, Dörner L, Stingele R. et al. Reversibility of venous sinus obstruction in idiopathic intracranial hypertension. AJNR. American journal of neuroradiology 2007; 28: 656-659
  • 27 de Simone R, Marano E, Fiorillo C. et al. Sudden re-opening of collapsed transverse sinuses and longstanding clinical remission after a single lumbar puncture in a case of idiopathic intracranial hypertension. Pathogenetic implications. Neurological sciences: official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2005; 25: 342-344
  • 28 Bono F, Giliberto C, Mastrandrea C. et al. Transverse sinus stenoses persist after normalization of the CSF pressure in IIH. Neurology 2005; 65: 1090-1093
  • 29 Juhász J, Lindner T, Jansen O. et al. Changes in intracranial venous hemodynamics in a patient with idiopathic intracranial hypertension after lumbar puncture precedes therapeutic success. Journal of magnetic resonance imaging: JMRI 2018; 47: 286-288
  • 30 de Simone R, Ranieri A, Montella S. et al. The role of dural sinus stenosis in idiopathic intracranial hypertension pathogenesis: the self-limiting venous collapse feedback-loop model. Panminerva medica 2014; 56: 201-209
  • 31 Bateman GA. Association between arterial inflow and venous outflow in idiopathic and secondary intracranial hypertension. Journal of clinical neuroscience: official journal of the Neurosurgical Society of Australasia 2006; 13: 550-556 ; discussion 557
  • 32 Juhász J, Lindner T, Riedel C. et al. Quantitative Phase-Contrast MR Angiography to Measure Hemodynamic Changes in Idiopathic Intracranial Hypertension. AJNR. American journal of neuroradiology 2018; 39: 682-686
  • 33 Bastin ME, Sinha S, Farrall AJ. et al. Diffuse brain oedema in idiopathic intracranial hypertension: a quantitative magnetic resonance imaging study. Journal of neurology, neurosurgery, and psychiatry 2003; 74: 1693-1696
  • 34 Ireland B, Corbett JJ, Wallace RB. The search for causes of idiopathic intracranial hypertension. A preliminary case-control study. Archives of neurology 1990; 47: 315-320
  • 35 Hoffmann J, May A. Neues beim Pseudotumor cerebri (Idiopathische intrakranielle Hypertension). Akt Neurol 2017; 44: 466-475
  • 36 Giuseffi V, Wall M, Siegel PZ. et al. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991; 41: 239-244
  • 37 Kesler A, Goldhammer Y, Hadayer A. et al. The outcome of pseudotumor cerebri induced by tetracycline therapy. Acta neurologica Scandinavica 2004; 110: 408-411
  • 38 Rohr A. Pseudotumor cerebri. Radiologie up2date 2008; 8: 183-199
  • 39 Rohr AC, Riedel C, Fruehauf M-C. et al. MR imaging findings in patients with secondary intracranial hypertension. AJNR. American journal of neuroradiology 2011; 32: 1021-1029
  • 40 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81: 1159-1165
  • 41 Salehi Ravesh M, Jensen-Kondering U, Juhasz J. et al. Optimization of 3D phase contrast venography for the assessment of the cranio-cervical venous system at 1.5 T. Neuroradiology 2019; 61: 293-304
  • 42 Wall M. Update on idiopathic intracranial hypertension. Neurologic clinics 2017; 35: 45-57
  • 43 Rohr A, Bindeballe J, Riedel C. et al. The entire dural sinus tree is compressed in patients with idiopathic intracranial hypertension: a longitudinal, volumetric magnetic resonance imaging study. Neuroradiology 2012; 54: 25-33
  • 44 Baryshnik DB, Farb RI. Changes in the appearance of venous sinuses after treatment of disordered intracranial pressure. Neurology 2004; 62: 1445-1446
  • 45 Rohr A, Reimann G, Alfke K. et al. Pseudotumor cerebri – was ist normal? Normwerte für indirekte marker bei intrakranieller Hypertension. Fortschr Röntgenstr 2008; 180: 884-890
  • 46 Piper RJ, Kalyvas AV, Young AMH. et al. Interventions for idiopathic intracranial hypertension. The Cochrane database of systematic reviews 2015; 8: CD003434
  • 47 Thenuwara K, Todd MM, Brian JE. Effect of mannitol and furosemide on plasma osmolality and brain water. Anesthesiology 2002; 96: 416-421
  • 48 Celebisoy N, Gökçay F, Sirin H. et al. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta neurologica Scandinavica 2007; 116: 322-327
  • 49 Mollan SP, Davies B, Silver NC. et al. Idiopathic intracranial hypertension: consensus guidelines on management. Journal of neurology, neurosurgery, and psychiatry 2018; 89: 1088-1100
  • 50 Berdahl JP, Fleischman D, Zaydlarova J. et al. Body mass index has a linear relationship with cerebrospinal fluid pressure. Investigative ophthalmology & visual science 2012; 53: 1422-1427
  • 51 Kupersmith MJ, Gamell L, Turbin R. et al. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology 1998; 50: 1094-1098
  • 52 Bateman GA. Stenoses in idiopathic intracranial hypertension: to stent or not to stent?. AJNR. American journal of neuroradiology 2008; 29: 215 ; author reply 215-216
  • 53 Teleb MS, Cziep ME, Issa M. et al. Stenting and angioplasty for idiopathic intracranial hypertension: a case series with clinical, angiographic, ophthalmological, complication, and pressure reporting. Journal of neuroimaging: official journal of the American Society of Neuroimaging 2015; 25: 72-80
  • 54 Higgins JNP, Cousins C, Owler BK. et al. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. Journal of neurology, neurosurgery, and psychiatry 2003; 74: 1662-1666
  • 55 Abubaker K, Ali Z, Raza K. et al. Idiopathic intracranial hypertension: lumboperitoneal shunts versus ventriculoperitoneal shunts--case series and literature review. British journal of neurosurgery 2011; 25: 94-99
  • 56 Corbett JJ, Nerad JA, Tse DT. et al Results of optic nerve sheath fenestration for pseudotumor cerebri. The lateral orbitotomy approach. Archives of ophthalmology (Chicago, Ill.: 1960) 1988; 106: 1391-1397
  • 57 Handley JD, Baruah BP, Williams DM. et al. Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review. Surgery for obesity and related diseases: official journal of the American Society for Bariatric Surgery 2015; 11: 1396-1403