Chakraborty Arka Prava, Mukherjee Adreesh, Sinharoy Uma, Chakrabarty Madhushree, Sengupta Mainak, Chowdhury Jasodhara, Biswas Atanu
Department of Neurology, Institute of Postgraduate Medical Education and Research and Bangur Institute of Neurosciences, Kolkata, West Bengal, India.
Department of Genetics, University of Calcutta, West Bengal, India.
Ann Indian Acad Neurol. 2025 Jan 1;28(1):66-71. doi: 10.4103/aian.aian_503_24. Epub 2025 Feb 6.
Although the motor symptoms of Wilson's disease have received particular attention from researchers and medical professionals, non-motor symptoms might become increasingly prevalent with the advancement of the disease and can even appear before the onset of motor symptoms. However, clinicopathological correlations for most of these non-motor features are still poorly understood. The correlations between non-motor and motor symptoms have been examined in this study.
Fifty patients with Wilson's disease participated in this study. Each subject was administered the Global Assessment Scale and the Non-Motor Symptom Questionnaire (NMS Quest) for the assessment of motor and non-motor symptoms, respectively. Cognitive functions were evaluated with Addenbrooke's Cognitive Examination III (Bengali version) and the Digit Span Test. Sleep-related problems were assessed with Pittsburgh Sleep Quality Index.
Of the patients who participated in this study, 82%, 56%, 90%, 18%, 82%, 8%, 60%, 56%, and 66% had digestive, urinary, apathy-attention-memory, hallucinations/delusions, depression/anxiety, sexual function, cardiovascular, sleep disorder, and miscellaneous (pain, weight, swelling, sweating, and diplopia) symptoms, respectively. NMS-Digestion ( P ≤ 0.001), NMS-Urinary ( P = 0.007), NMS-Miscellany ( P = 0.001), NMS-Memory (0.011), and NMS-Sleep Disorder ( P = 0.031) significantly predicted parkinsonism. NMS-Digestion was a significant predictor of dystonia ( P < 0.001).
Awareness regarding non-motor symptoms and their associations with motor symptoms might help physicians develop more efficient treatment regimens that can alleviate non-motor symptoms which can be equally troublesome and disabling for these patients. Management of non-motor symptoms is crucial for the overall well-being of these patients.
尽管威尔逊病的运动症状受到了研究人员和医学专业人员的特别关注，但随着疾病进展，非运动症状可能会越来越普遍，甚至可能在运动症状出现之前就已出现。然而，对于这些非运动特征中的大多数，临床病理相关性仍知之甚少。本研究对非运动症状与运动症状之间的相关性进行了研究。
50例威尔逊病患者参与了本研究。分别采用整体评估量表和非运动症状问卷（NMS Quest）对每位受试者的运动和非运动症状进行评估。使用Addenbrooke认知检查III（孟加拉语版）和数字广度测试评估认知功能。使用匹兹堡睡眠质量指数评估与睡眠相关的问题。
参与本研究的患者中，分别有82%、56%、90%、18%、82%、8%、60%、56%和66%出现消化、泌尿、淡漠-注意力-记忆、幻觉/妄想、抑郁/焦虑、性功能、心血管、睡眠障碍以及其他（疼痛、体重、肿胀、出汗和复视）症状。非运动症状-消化（P≤0.001）、非运动症状-泌尿（P = 0.007）、非运动症状-其他（P = 0.001）、非运动症状-记忆（0.011）和非运动症状-睡眠障碍（P = 0.031）显著预测帕金森综合征。非运动症状-消化是肌张力障碍的显著预测因素（P < 0.001）。
了解非运动症状及其与运动症状的关联可能有助于医生制定更有效的治疗方案，以缓解对这些患者同样造成困扰和致残的非运动症状。管理非运动症状对这些患者的整体健康至关重要。
