Kalita Jayantee, Kumar Vijay, Parashar Vasudev, Misra Usha K
Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226014, India.
Department of Biotechnology, Yeungnam University, Gyeongsan, Gyeongbuk, 38541, Republic of Korea.
Mol Neurobiol. 2021 Nov;58(11):6020-6031. doi: 10.1007/s12035-021-02525-4. Epub 2021 Aug 26.
This study aims to identify neuropsychiatric manifestations in neurological Wilson disease (NWD), and their correlation with MRI changes and glutamate excitotoxicity. Forty-three consecutive patients with NWD from a tertiary care teaching hospital were evaluated prospectively who fulfilled the inclusion criteria. The neuropsychiatric evaluation was done using Neuropsychiatric Inventory (NPI) battery that assesses 12 domains including delusion, hallucination, agitation/aggression, dysphoria/depression, anxiety, euphoria, apathy, disinhibition, irritability, aberrant motor activity, appetite change, and abnormal nighttime behavior. Cranial MRI was done using a 3 T machine, and locations of signal changes were noted including the total number of MRI lesions. Serum glutamate level was measured by a fluorescence microplate reader. Abnormal NPI in various domains and total NPI scores were correlated with MRI lesions, serum and urinary copper, and glutamate level. The median age of the patients was 16 years. Forty-one (48.8%) patients had cognitive impairment and 37 (86%) had movement disorder. Neurobehavioral abnormality was detected in all-commonest being agitation (90.7%) followed by appetite change (81.4%), elation (74.4%), irritability (69.8%), anxiety (67.4%), depression (65.1%), apathy (44.2%), night time abnormal behavior (32.6%), aberrant motor behavior (20.9%), delusions (16.3%), and hallucination (18.6%). The thalamic lesion was associated with depression, globus pallidus with depression and anxiety, caudate with anxiety and agitation, brainstem with irritability, and frontal cortex with apathy. Serum glutamate level was higher in NWD. NPI sum score correlated with MRI load and glutamate level. Varying severity of neurobehavioral abnormalities are common in the patients with NWD and correlate with the location of MRI lesion and glutamate level.
本研究旨在确定神经型威尔逊病（NWD）的神经精神表现，及其与MRI变化和谷氨酸兴奋性毒性的相关性。对一家三级护理教学医院连续收治的43例符合纳入标准的NWD患者进行了前瞻性评估。使用神经精神科问卷（NPI）对患者进行神经精神评估，该问卷评估12个领域，包括妄想、幻觉、激越/攻击行为、烦躁/抑郁、焦虑、欣快、冷漠、脱抑制、易怒、异常运动行为、食欲改变和异常夜间行为。使用3T机器进行头颅MRI检查，记录信号变化的位置，包括MRI病变的总数。用荧光酶标仪测量血清谷氨酸水平。将各领域异常的NPI及NPI总分与MRI病变、血清和尿铜以及谷氨酸水平进行相关性分析。患者的中位年龄为16岁。41例（48.8%）患者有认知障碍，37例（86%）有运动障碍。所有患者均检测到神经行为异常，最常见的是激越（90.7%），其次是食欲改变（81.4%）、欣快（74.4%）、易怒（69.8%）、焦虑（67.4%）、抑郁（65.1%）、冷漠（44.2%）夜间异常行为（32.6%）、异常运动行为（20.9%）、妄想（16.3%）和幻觉（18.6%）。丘脑病变与抑郁相关，苍白球与抑郁和焦虑相关，尾状核与焦虑和激越相关，脑干与易怒相关，额叶皮质与冷漠相关。NWD患者的血清谷氨酸水平较高。NPI总分与MRI负荷和谷氨酸水平相关。NWD患者中神经行为异常的严重程度各不相同，且与MRI病变位置和谷氨酸水平相关。
