NODDI MRI shows phenotype-specific white matter alterations in Wilson disease patients
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Key Takeaway
Interpret NODDI findings in Wilson disease as preliminary imaging biomarkers requiring validation.
This observational study used neurite orientation dispersion and density imaging (NODDI) to characterize microstructural white matter alterations in 30 Wilson disease patients (19 with predominant neurological involvement, 11 with hepatic manifestation) compared to 30 matched healthy controls. The study examined NODDI-derived metrics including neurite density index (NDI), orientation dispersion index (ODI), and isotropic volume fraction (ISOVF).
Patients with neurological Wilson disease showed widespread reductions in both NDI and ODI compared to healthy controls, suggesting decreased axonal density and organization. In contrast, patients with hepatic Wilson disease showed increases in ISOVF, indicating higher free water content. Decreased NDI in specific white matter fibers correlated with neurological impairment, processing speed deficits, and visual attention problems.
No safety or tolerability data were reported for the imaging procedure. Key limitations include the small sample size, lack of reported statistical measures (p-values, confidence intervals, effect sizes), and the observational design that precludes causal conclusions. The authors suggest NODDI could be useful as an imaging biomarker for forecasting conversion to neurological manifestations and monitoring disease progression, but this potential utility requires validation in larger, longitudinal studies.
View Original Abstract ↓
BackgroundNeurite orientation dispersion and density imaging (NODDI) shows promise in providing specific insights into the neurite morphology underlying white matter (WM) damage in neurodegenerative diseases. This study aimed to advance the currently limited knowledge by characterizing NODDI-derived microstructural WM alterations in Wilson disease (WD) and examining their relationships with clinical symptoms.
Methods30 WD patients, including 19 with predominant neurological involvement (neuro-WD) and 11 with hepatic manifestation (hep-WD), and 30 matched healthy controls underwent multi-shell diffusion-weighted magnetic resonance imaging. NODDI metrics, including neurite density index (NDI), orientation dispersion index (ODI), and isotropic volume fraction (ISOVF), and diffusion tensor imaging-based fractional anisotropy (FA) were estimated. Group differences in diffusion parameters across the WM skeleton were determined using tract-based spatial statistics. Additionally, voxel-wise correlations with neurological and cognitive scores were investigated.
ResultsWe observed widespread NDI and ODI reductions in neuro-WD patients and ISOVF increases in hep-WD patients compared with healthy controls, particularly involving the corpus callosum, corona radiata, superior longitudinal fasciculus, external and internal capsule, and superior fronto-occipital fasciculus. A comparable yet more subtle pattern was found when comparing phenotypes. Distinct NDI and ODI constellations were identified as the microstructural determinants of FA alterations. Decreased NDI in the aforementioned fibers were correlated with neurological impairment, processing speed, and visual attention.
ConclusionsPhenotype-specific microstructural WM alterations were identified, characterized by globally reduced axonal density and fiber organization in neuro-WD and excess free water in hep-WD. NODDI could be useful as an imaging biomarker for forecasting conversion to neurological WD manifestations and monitoring of disease progression.
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