ArticleAnnals of Indian Academy of Neurology2025
Diffusion Restriction in Wilson's Disease: A Radiological Pointer Toward Clinically Severe Disease.
Article in Annals of Indian Academy of Neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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1 citing paper in PubMed.
- Authors' Reply: Progressive Supranuclear Palsy or Something More? Unveiling Late-Onset Wilson's Disease.Annals of Indian Academy of Neurology · 2026Article
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7 authors.
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Abstract
BACKGROUND AND
objectivesWilson's disease (WD) is a rare autosomal recessive disorder due to abnormal hepatic copper transport, leading to copper accumulation in the liver, brain, and other tissues. Although conventional magnetic resonance imaging (MRI) features are valuable for diagnosis, the role of diffusion-weighted imaging (DWI) remains underexplored in WD. The study aims to assess the prevalence and clinical correlates of diffusion restriction on MRI in WD.
methodsA single-center, retrospective study was conducted. Clinicodemographic and MRI findings of patients diagnosed with WD based on a modified Leipzig score cut-off of 4 were analyzed. Characteristics of patients with diffusion restriction (WD DR+ ) and without diffusion restriction (WD DR- ) were compared using statistical tests.
resultsOf 91 patients with WD, 17 (18.7%) demonstrated MRI diffusion restriction. WD DR+ were noted to have a lower median age at symptom onset (14 [9-17] versus 15 [14-23] years; P = 0.020) and presentation (15 [13-20] years versus 19 [15-25.5] years; P = 0.022) compared to WD DR- . WD DR+ had a higher modified Leipzig score compared to WD DR- (5 [4-5] versus 7 [5-8]; P ≤ 0.001). They also had significantly higher Global Assessment Scale (GAS) for WD (32.5 [30-37]) compared to WD DR- (19 [13-22]) ( P < 0.001). WD DR+ had significantly higher proportions of patients with dysphagia (10/17, 58.8% versus 6/74, 8.1%; P < 0.001) and portal hypertension (8/17, 47.1% versus 6/74, 8.1%; P < 0.001). WD DR+ also experienced significantly higher rates of early neurological deterioration (END) (23.5% versus 6.6%, P = 0.04).
conclusionsDiffusion restriction may hence serve as a pointer toward more severe neurological and hepatic involvement in WD, indicating the need for close supervision in this group of patients.
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