Mulroy Eoin, Baschieri Francesca, Magrinelli Francesca, Latorre Anna, Cortelli Pietro, Bhatia Kailash P
Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.
IRCCS Istituto delle Scienze Neurologiche di Bologna Bologna Italy.
Mov Disord Clin Pract. 2021 May 31;8(6):828-842. doi: 10.1002/mdc3.13238. eCollection 2021 Aug.
The association of movement disorders with structural or functional hepatic disease occurs in three principal scenarios: (1) combined involvement of both organ systems from a single disease entity, (2) nervous system dysfunction resulting from exposure to toxic compounds in the setting of defective hepatic clearance, or (3) hepatic and/or neurological injury secondary to exposure to exogenous drugs or toxins. An important early step in the workup of any patient with combined movement disorders and liver disease is the exclusion of Wilson's disease. Diagnostic delay remains common for this treatable disorder, and this has major implications for patient outcomes. Thereafter, a structured approach integrating variables such as age of onset, tempo of progression, nature and severity of liver involvement, movement disorder phenomenology, exposure to drugs/toxins and laboratory/neuroimaging findings is key to ensuring timely diagnosis and disease-specific therapy. Herein, we provide an overview of disorders which may manifest with a combination of movement disorders and liver disease, structured under the three headings as detailed above. In each section, the most common disorders are discussed, along with important clinical pearls, suggested diagnostic workup, differential diagnoses and where appropriate, treatment considerations.
(1)单一疾病实体同时累及两个器官系统;(2)在肝脏清除功能缺陷的情况下,因接触有毒化合物导致神经系统功能障碍;或(3)接触外源性药物或毒素继发肝脏和/或神经损伤。对于任何患有运动障碍和肝脏疾病的患者,检查的一个重要早期步骤是排除威尔逊病。对于这种可治疗的疾病,诊断延迟仍然很常见,这对患者的预后有重大影响。此后,采用结构化方法整合发病年龄、进展速度、肝脏受累的性质和严重程度、运动障碍表现、药物/毒素接触情况以及实验室/神经影像学检查结果等变量,是确保及时诊断和进行针对性疾病治疗的关键。在此,我们概述了可能同时出现运动障碍和肝脏疾病的疾病,按照上述三个标题进行分类阐述。在每个部分中,将讨论最常见的疾病,以及重要的临床要点、建议的诊断检查、鉴别诊断,并在适当情况下讨论治疗注意事项。