gms | German Medical Science

73. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Griechischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

29.05. - 01.06.2022, Köln

Article

Send article

Clinical course in patients with mucopolysaccharidosis and spinal stenosis with spinal cord compression

Klinischer Verlauf bei Patienten mit Mukopolysaccharidose und Spinalkanalstenose mit Rückenmarkskompression

Meeting Abstract

Search Medline for

  • presenting/speaker Tobias Pantel - Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Neurochirurgie, Hamburg, Deutschland
  • Mona Lindschau - Universitätsklinikum Hamburg-Eppendorf, International Center for Lysosomal Disorders (ICLD), Hamburg, Deutschland
  • Andreas Luebke - Universitätsklinikum Hamburg-Eppendorf, Institut für Neuropathologie, Hamburg, Deutschland
  • Philipp Kunkel - Universitätsmedizin Mannheim, Department of Pediatric Neurosurgery, Mannheim, Deutschland
  • Marc Dreimann - Universitätsklinikum Hamburg-Eppendorf, Division of Spine Surgery, Department of Trauma and Orthopedic Surgery, Hamburg, Deutschland
  • Nicole Muschol - Universitätsklinikum Hamburg-Eppendorf, International Center for Lysosomal Disorders (ICLD), Hamburg, Deutschland
  • Sven Oliver Eicker - Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Neurochirurgie, Hamburg, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 73. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Griechischen Gesellschaft für Neurochirurgie. Köln, 29.05.-01.06.2022. Düsseldorf: German Medical Science GMS Publishing House; 2022. DocP145

doi: 10.3205/22dgnc458, urn:nbn:de:0183-22dgnc4583

Published: May 25, 2022

© 2022 Pantel et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Objective: Mucopolysaccharidosis (MPS) types I, II, IV and VI is regularly associated with spinal pathologies. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression (SCC) due to bone variances and accumulation of glycosaminoglycans (GAG) in the surrounding ligaments and dura layers is a serious complication of MPS disease. Data on optimal perioperative therapeutic care of SS is limited.

Methods: A retrospective analysis of patients with MPS and SS for the time period 01/1998 to 03/2021 was performed. Demographics, clinical data, neurological status, diagnostic evaluations (radiography, MRI, electrophysiology), and treatment modalities were extracted. A Cox regression analysis was performed to identify prognostic factors for neurological outcomes.

Results: Out of a total of 209 MPS patients, 15 had SS with SCC and met the inclusion criteria to this study. Our cohort was dominated by MPS I (-H) (n = 7; 46.7%). Preoperative neurological deterioration was predominantly the trigger for further diagnostics (n = 12; 80%). The surgical procedure of choice was dorsal instrumentation with microsurgical decompression (n = 14; 93.3%). A univariate Cox regression analysis unveiled MPS type I (-H) to be associated with favorable neurological outcomes.

Conclusion: Early detection of SS is highly relevant in patients with MPS. Detailed neurological assessment during follow-up is crucial for timeous detection of emerging neurological deterioration to enable optimal treatment. The surgical intervention of choice is a dorsal instrumentation with microsurgical decompression and resection of thickened spinal ligaments and dura layers.