# Assessment of cervical spinal pathology in patients with MPS I, II and VI

> **NIH NIH R03** · UNIVERSITY OF MINNESOTA · 2024 · $155,000

## Abstract

ABSTRACT
Cervical spine abnormalities in mucopolysaccharidosis (MPS) may result in cervical spinal cord (CSC)
compression and irreversible neurological disability. MRI signal intensity changes in CSC images occur in the
presence of advanced stenosis, myelopathy, and developed clinical symptoms. The proposal presumes that
deficits in CSC morphology and microstructure precede MRI signal changes and are predictive of clinical
myelopathy. We will proceed with systematic validation comparing CSC morphology and microstructure in MPS
patients and case-matched healthy controls. The overall objective is to analyze existing and acquired CSC MRI
data and assess longitudinal CSC morphology and cross-sectional CSC microstructure in the MPS. The central
hypothesis is that quantitative MRI is sensitive to CSC morphological and microstructural degeneration,
myelopathy risk, and disease severity in MPS. To test the central hypothesis and, thereby, attain the overall
objective, we will pursue two specific aims: Aim 1: Assess longitudinal morphological damage of CSC in MPS
types I, II, and VI and Aim 2: Assess cross-sectional microstructural damage of CSC in MPS types I and VI. Aim
1 will test a working hypothesis that CSC morphology is altered due to cervical spinal compression and is
predictive of myelopathy in MPS. Aim 2 will test a working hypothesis that CSC inner integrity is altered due to
MPS I and VI diseases. We will demonstrate that quantitative CSC MRI measurements are proportional to
myelopathy risk and disease severity in MPS. The effects of MPS treatment on CSC measurements will be
investigated where possible. The proposal will identify candidates for biomarkers of myelopathy in MPS.
Regarding the biomarker research, the proposal is a pilot preclinical exploratory phase 1. The project is
innovative because it will: (i) discover the biomarker candidates of myelopathy risk and disease severity in MPS;
(ii) diffusion tensor imaging (DTI) will be assessed in the CSC of MPS patients; (iii) demonstrate that DTI-based
CSC microstructure can assess ongoing degenerative processes before visual and qualitative MRI signal
intensity changes. Overall, the proposal aims to advance objective early myelopathy diagnosis, which can guide
the timing of a prophylactic myelopathy treatment and improve clinical outcomes in MPS.

## Key facts

- **NIH application ID:** 10949962
- **Project number:** 1R03NS139000-01
- **Recipient organization:** UNIVERSITY OF MINNESOTA
- **Principal Investigator:** Rene Labounek
- **Activity code:** R03 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $155,000
- **Award type:** 1
- **Project period:** 2024-07-01 → 2026-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10949962

## Citation

> US National Institutes of Health, RePORTER application 10949962, Assessment of cervical spinal pathology in patients with MPS I, II and VI (1R03NS139000-01). Retrieved via AI Analytics 2026-05-27 from https://api.ai-analytics.org/grant/nih/10949962. Licensed CC0.

---

*[NIH grants dataset](/datasets/nih-grants) · CC0 1.0*