Jellinger Kurt A
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
J Neural Transm (Vienna). 2025 Jan;132(1):1-22. doi: 10.1007/s00702-024-02816-9. Epub 2024 Sep 4.
Multiple sclerosis (MS) is a heterogenous autoimmune-mediated disease of the central nervous system (CNS) characterized by inflammation, demyelination and chronic progressive neurodegeneration. Among its broad and unpredictable range of neuropsychiatric symptoms, behavioral changes are common, even from the early stages of the disease, while they are associated with cognitive deficits in advanced MS. According to DSM-5, behavioral disorders include attention deficits, oppositional, defiant and conduct disorders, anxiety, panic, obsessive-compulsive disorders (OCD), disruptive and emotional disorders, while others include also irritability, agitation, aggression and executive dysfunctions. Approximately 30 to 80% of individuals with MS demonstrate behavioral changes associated with disease progression. They are often combined with depression and other neuropsychiatric disorders, but usually not correlated with motor deficits, suggesting different pathomechanisms. These and other alterations contribute to disability in MS. While no specific neuropathological data for behavioral changes in MS are available, those in demyelination animal models share similarities with white matter and neuroinflammatory abnormalities in humans. Neuroimaging revealed prefrontal cortical atrophy, interhemispheric inhibition and disruption of fronto-striato-thalamic and frontoparietal networks. This indicates multi-regional patterns of cerebral disturbances within the MS pathology although their pathogenic mechanisms await further elucidation. Benefits of social, psychological, behavioral interventions and exercise were reported. Based on systematical analysis of PubMed, Google Scholar and Cochrane library, current epidemiological, clinical, neuroimaging and pathogenetic evidence are reviewed that may aid early identification of behavioral symptoms in MS, and promote new therapeutic targets and strategies.
多发性硬化症(MS)是一种由自身免疫介导的中枢神经系统(CNS)异质性疾病,其特征为炎症、脱髓鞘和慢性进行性神经退行性变。在其广泛且不可预测的一系列神经精神症状中,行为改变很常见,甚至在疾病早期就会出现,而在晚期MS中,行为改变与认知缺陷相关。根据《精神疾病诊断与统计手册》第5版(DSM-5),行为障碍包括注意力缺陷、对立违抗和品行障碍、焦虑、惊恐、强迫症(OCD)、破坏性行为和情绪障碍,其他还包括易怒、激动、攻击行为和执行功能障碍。约30%至80%的MS患者表现出与疾病进展相关的行为改变。这些行为改变常与抑郁和其他神经精神障碍合并出现,但通常与运动缺陷无关,提示存在不同的发病机制。这些及其他改变会导致MS患者残疾。虽然目前尚无MS行为改变的具体神经病理学数据,但脱髓鞘动物模型中的行为改变与人类白质和神经炎症异常有相似之处。神经影像学显示前额叶皮质萎缩、半球间抑制以及额-纹状体-丘脑和额顶叶网络的破坏。这表明MS病理过程中存在多区域脑功能紊乱模式,尽管其致病机制有待进一步阐明。已有报道称社会、心理、行为干预和运动具有益处。基于对PubMed、谷歌学术和考克兰图书馆的系统分析,本文综述了当前的流行病学、临床、神经影像学和发病机制证据,这些证据可能有助于早期识别MS中的行为症状,并推动新的治疗靶点和策略的发展。