Evaluation and Comparison of Scales for Neurological Assessment in Indian Children with Neuro-Wilson Disease

To the Editor:Wilson disease (WD) is an autosomal-recessive, inherited disorder of copper metabolism. Neuro-WD (NWD), also known as Westphal-Strumpell pseudosclerosis, is attributed to the damage of the nervous tissue by extrahepatic copper toxicity [1]. The neurotoxicity of copper results in neuronal loss, gliosis, and cavity degeneration due to mitochondrial toxicity, oxidative damage, cell membrane injury, and DNA crosslinking [1]. This cross-sectional study of 18 mo period, included children diagnosed with NWD based on Modified Leipzig criteria between the ages of 3–18 y. We evaluated and compared the neurological assessment in children with NWD by Global assessment scale (GAS) [2], Unified Wilson disease rating scale (UWDRS) [3], and ‘Minimal’ UWDRS scores [4] at diagnosis with that of the follow-up. Eight children were included and majority were males (75%). Most common neurological symptom was progressive dystonia (87.5%). Mean age at onset of neurological symptoms was 10.2 ± 1.3 y. Statistically significant associations were found between GAS tier II score and UWDRS score (p < 0.001), and UWDRS score and follow-up ‘Minimal’ UWDRS score (p = 0.004). …