Spinal cord compression in patients with mucopolysaccharidosis

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Spinal cord compression in patients with mucopolysaccharidosis. / Pantel, Tobias; Lindschau, Mona; Luebke, Andreas M; Kunkel, Philip; Dreimann, Marc; Muschol, Nicole; Eicker, Sven O.

in: EUR SPINE J, Jahrgang 31, Nr. 7, 07.2022, S. 1693-1699.

Publikationen: SCORING: Beitrag in Fachzeitschrift/ZeitungSCORING: ZeitschriftenaufsatzForschungBegutachtung

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@article{48cebb0e6ae44d8fb3a5625a69be59ae,
title = "Spinal cord compression in patients with mucopolysaccharidosis",
abstract = "PURPOSE: Spinal abnormalities frequently occur in patients with mucopolysaccharidosis (MPS) types I, II, IV, and VI. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression due to bone malformations and an accumulation of storage material in soft tissue are serious complications of MPS disease. Data on optimal perioperative therapeutic care of SS is limited.METHODS: A retrospective chart 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 documented. The extent of the SS and spinal canal diameter were analyzed. A Cox regression analysis was performed to identify prognostic factors for neurological outcomes.RESULTS: Out of 209 MPS patients, 15 were included in this study. The most dominant type of MPS was I (-H) (n = 7; 46.7%). Preoperative neurological deterioration was the most frequent indication 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 showed MPS type I (-H) to be associated with favorable neurological outcomes.CONCLUSION: Early detection of spinal stenosis is highly relevant in patients with MPS. Detailed neurological assessment during follow-up is crucial for timeous detection of patients at risk. The surgical intervention of choice is dorsal instrumentation with microsurgical decompression and resection of thickened intraspinal tissue. Patients with MPS type I (-H) demonstrated the best neurological course.",
author = "Tobias Pantel and Mona Lindschau and Luebke, {Andreas M} and Philip Kunkel and Marc Dreimann and Nicole Muschol and Eicker, {Sven O}",
note = "{\textcopyright} 2022. The Author(s).",
year = "2022",
month = jul,
doi = "10.1007/s00586-022-07168-0",
language = "English",
volume = "31",
pages = "1693--1699",
journal = "EUR SPINE J",
issn = "0940-6719",
publisher = "Springer",
number = "7",

}

RIS

TY - JOUR

T1 - Spinal cord compression in patients with mucopolysaccharidosis

AU - Pantel, Tobias

AU - Lindschau, Mona

AU - Luebke, Andreas M

AU - Kunkel, Philip

AU - Dreimann, Marc

AU - Muschol, Nicole

AU - Eicker, Sven O

N1 - © 2022. The Author(s).

PY - 2022/7

Y1 - 2022/7

N2 - PURPOSE: Spinal abnormalities frequently occur in patients with mucopolysaccharidosis (MPS) types I, II, IV, and VI. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression due to bone malformations and an accumulation of storage material in soft tissue are serious complications of MPS disease. Data on optimal perioperative therapeutic care of SS is limited.METHODS: A retrospective chart 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 documented. The extent of the SS and spinal canal diameter were analyzed. A Cox regression analysis was performed to identify prognostic factors for neurological outcomes.RESULTS: Out of 209 MPS patients, 15 were included in this study. The most dominant type of MPS was I (-H) (n = 7; 46.7%). Preoperative neurological deterioration was the most frequent indication 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 showed MPS type I (-H) to be associated with favorable neurological outcomes.CONCLUSION: Early detection of spinal stenosis is highly relevant in patients with MPS. Detailed neurological assessment during follow-up is crucial for timeous detection of patients at risk. The surgical intervention of choice is dorsal instrumentation with microsurgical decompression and resection of thickened intraspinal tissue. Patients with MPS type I (-H) demonstrated the best neurological course.

AB - PURPOSE: Spinal abnormalities frequently occur in patients with mucopolysaccharidosis (MPS) types I, II, IV, and VI. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression due to bone malformations and an accumulation of storage material in soft tissue are serious complications of MPS disease. Data on optimal perioperative therapeutic care of SS is limited.METHODS: A retrospective chart 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 documented. The extent of the SS and spinal canal diameter were analyzed. A Cox regression analysis was performed to identify prognostic factors for neurological outcomes.RESULTS: Out of 209 MPS patients, 15 were included in this study. The most dominant type of MPS was I (-H) (n = 7; 46.7%). Preoperative neurological deterioration was the most frequent indication 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 showed MPS type I (-H) to be associated with favorable neurological outcomes.CONCLUSION: Early detection of spinal stenosis is highly relevant in patients with MPS. Detailed neurological assessment during follow-up is crucial for timeous detection of patients at risk. The surgical intervention of choice is dorsal instrumentation with microsurgical decompression and resection of thickened intraspinal tissue. Patients with MPS type I (-H) demonstrated the best neurological course.

U2 - 10.1007/s00586-022-07168-0

DO - 10.1007/s00586-022-07168-0

M3 - SCORING: Journal article

C2 - 35267074

VL - 31

SP - 1693

EP - 1699

JO - EUR SPINE J

JF - EUR SPINE J

SN - 0940-6719

IS - 7

ER -