Session Information
Date: Wednesday, September 25, 2019
Session Title: Neuroimaging
Session Time: 1:15pm-2:45pm
Location: Les Muses Terrace, Level 3
Objective: To determine the inter- and intra-rater reliability of brain imaging in X-linked dystonia-parkinsonism (XDP).
Background: XDP is a neurodegenerative movement disorder presenting with adult-onset dystonia followed by parkinsonism over the course of the disease. Characteristic MRI alterations such as a hyperintense putaminal rim and atrophy of the caudate and putamen have been described [1,2]. We aimed to evaluate the accuracy of routine MRI to identify basal ganglia alterations in XDP.
Method: T1- and multiecho T2-weighted MRI images were acquired on a 3T MRI scanner in 19 XDP patients (mean age: 40.1 ± 7.2 years, mean disease duration 3.1 ± 1.5 years, male n=19) and 26 controls (mean age: 35.8 ± 8.0 years, male n= 18). Images were rated twice within four months’ time by two experienced neuroradiologists blinded to the clinical status.
Results: Across raters and time points, a hyperintense putaminal rim was detected in 7 to 14 of 19 patients and 0 to 15 of 26 controls. Putaminal atrophy was noticed in 3 to 13 of 19 patients and 0 to 1 of 26 controls, and caudate atrophy was detected in 4 to 10 of 19 patients and was not detected in controls. Intrarater reliability was weak/moderate for hyperintense putaminal rim (rater I: κ = 0.48; rater II: κ = 0.67; all p < 0.001), weak/perfect for putaminal atrophy (rater I: κ = 0.45; rater II: κ = 1.0; all p ≤ 0.001) and weak/strong for caudate atrophy (rater I: κ = 0.54; rater II: κ = 0.86; all p < 0.001). Putaminal atrophy was associated with a weak interrater reliability (κ = 0.26, p = 0.033), whereas no significant interrater reliability was found for hyperintense putaminal rim (κ = 0.09, p = 0.198) and caudate atrophy (κ = 0.01, p = 0.892).
Conclusion: Moderate intrarater and the absence of interrater reliability of MRI-associated basal ganglia changes implicate the necessity of a reliable automated voxel-based analysis regimen in XDP.
References: 1. Pasco PM, Ison CV, Munoz EL, et al. Understanding XDP through imaging, pathology, and genetics. Int J Neurosci. 2011;121 Suppl 1:12-17. 2. Hanssen H, Heldmann M, Prasuhn J, et al. Basal ganglia and cerebellar pathology in X-linked dystonia-parkinsonism. Brain. 2018;141(10):2995-3008.
To cite this abstract in AMA style:
J. Junker, A. Neumann, R. Rosales, D. Jamora, C. Diesta, J. Prasuhn, T. Muente, C. Klein, P. Schramm, N. Brueggemann. Role of clinical neuroimaging in X-linked dystonia-parkinsonism [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/role-of-clinical-neuroimaging-in-x-linked-dystonia-parkinsonism/. Accessed May 11, 2025.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/role-of-clinical-neuroimaging-in-x-linked-dystonia-parkinsonism/