Die idiopathische Rückenmarkshernie und ihre Differenzialdiagnosen^[1]

Zusammenfassung

Ursache einer fokalen Verlagerung des Rückenmarks kann eine idiopathische Rückenmarksherniation (Idiopathic Spinal Cord Herniation) sein, bei der das Rückenmark durch einen Duradefekt in den Epiduralraum gedrängt wird. Dies führt zu einer Verlagerung und Einklemmung des Rückenmarks. Die idiopathische Rückenmarksherniation ist eine seltene Krankheit und wird in vielen Fällen zunächst fehldiagnostiziert – mit entsprechender Verzögerung des Behandlungsbeginns. Das Krankheitsbild der idiopathischen Rückenmarksherniation ist leicht mit liquorisointensen intraspinalen, extramedullären Raumforderungen zu verwechseln, wie z. B. Epidermoidzysten oder Teratomen, intraduralen Arachnoidalzysten, epiduralen Hämatomen oder Abszessen, zystischen Tumoren der Nervenscheiden, Synovial- oder Tarlov-Zysten, Meningozelen und Pseudomeningozelen. Bei den ersten Untersuchungen mittels CT und nativer MRT zeigt sich eine fokale Verlagerung des Rückenmarks mit gleichzeitiger Erweiterung des Liquorraums, doch reicht der Befund häufig nicht aus, um die Ursache zu erkennen. Die hochauflösende Dünnschicht-MRT ermöglicht die genaue Lokalisierung des Duradefekts und des durch diesen Defekt in den Epiduralraum drängenden Rückenmarks. Sind in den Aufnahmen Artefakte erkennbar, die eine unbehinderte Liquorpulsation innerhalb des erweiterten Liquorraums anzeigen, schließt dies eine raumfordernde Läsion aus. Umgekehrt kann ein Füllungsdefekt in einer konventionellen oder einer CT-Myelografie zur Bestätigung einer liquorisointensen Raumforderung beitragen. Der Ausschluss einer extramedullären Zyste mit Randverstärkung wird durch intravenöse Verabreichung eines Kontrastmittels erleichtert. Das klinische Bild ist gewöhnlich unspezifisch, aber der akute Charakter der Symptome, das Auftreten von Fieber und die Kenntnis eines stattgehabten Traumas können die Befundung der bildgebenden Darstellungen leiten und bei der Eingrenzung der Differenzialdiagnose helfen. Ein multimodales Vorgehen bei der Bildgebung ist entscheidend für die Differenzierung einer idiopathischen Rückenmarksherniation von liquorisointensen intraspinalen, extramedullären Raumforderungen.

Abstract

Focal spinal cord displacement can be caused by idiopathic spinal cord herniation (ISCH), in which the cord protrudes through a dural defect into the epidural space, causing cord displacement and tethering. ISCH is uncommon and often is misdiagnosed initially, which results in delayed management. ISCH can be mimicked by space-occupying cerebrospinal fluid (CSF)-isointense intraspinal extramedullary lesions, such as epidermoid cysts or teratomas, intradural arachnoid cysts, epidural hematomas or abscesses, cystic nerve sheath tumors, synovial or Tarlov cysts, meningoceles, and pseudomeningoceles. Initial computed tomography (CT) and unenhanced magnetic resonance (MR) imaging studies may depict focal cord displacement and a widened CSF space but often are not sufficient to identify the underlying cause. High-resolution thin-section MR imaging can delineate the exact location of the dural defect and the protrusion of the herniated cord through this defect into the epidural space. At imaging, unimpeded CSF pulsation artifacts seen within a widened CSF space exclude a space-occupying lesion. A filling defect seen at conventional or CT myelography can help confirm a CSF-isointense space-occupying lesion; intravenous contrast agent administration can help exclude a rim-enhancing cystic extramedullary lesion. The clinical presentation usually is nonspecific, but symptom acuity, fever, and trauma can guide the imaging evaluation and help narrow the differential diagnosis. A multimodality imaging approach is essential to differentiate ISCH from space-occupying CSF-isointense intraspinal extramedullary lesions.

^{1 ©}2014 The Radiological Society of North America. All rights reserved. Originally puplished in English in RadioGraphics 2014; 34: 313 – 329. Online published in 10.1148 /rg.342125136. Translated and reprinted with permission of RSNA. RSNA is not responsible for any inaccuracy or error arising from the translation from English to German.

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