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儿科神经精神障碍伴运动和非运动现象。

Pediatric neuropsychiatric disorders with motor and nonmotor phenomena.

机构信息

Department of Clinical Medicine and Neuroscience, CUNY School of Medicine, New York, NY, United States; Department of Medicine, Section of Internal Medicine and Neurology, White Plains Hospital, White Plains, NY, United States.

出版信息

Handb Clin Neurol. 2023;196:367-387. doi: 10.1016/B978-0-323-98817-9.00028-4.

Abstract

The concept of pediatric autoimmune neuropsychiatric disorders associated with group A beta-hemolytic streptococcus (PANDAS) has become seminal since first introduced more than two decades ago. At the time of this writing, most neurologists, pediatricians, psychiatrists, and general pediatricians will probably have heard of this association or treated an affected child with PANDAS. The concept of an acute-onset, and typically self-limited, postinfectious autoimmune neuropsychiatric disorder resembling PANDAS manifesting vocal and motor tics and obsessive-compulsive disorder has broadened to other putative microbes and related endogenous and exogenous disease triggers. These disorders with common features of hypometabolism in the medial temporal lobe and hippocampus in brain fluorodeoxyglucose positron emission tomography fused to magnetic resonance imaging (FDG PET-MRI), form a spectrum: with the neuropsychiatric disorder Tourette syndrome and PANDAS with its well-defined etiopathogenesis at one end, and pediatric abrupt-onset neuropsychiatric syndrome (PANS), alone or associated with specific bacterial and viral pathogens, at the other end. The designation of PANS in the absence of a specific trigger, as an exclusionary diagnosis, reflects the current problem in nosology.

摘要

儿科自身免疫性神经精神障碍与 A 组β溶血性链球菌相关(PANDAS)的概念自二十多年前首次提出以来,一直具有重要意义。在撰写本文时,大多数神经科医生、儿科医生、精神科医生和普通儿科医生可能已经听说过这种关联,或者治疗过患有 PANDAS 的患儿。这种急性发作且通常自限性的感染后自身免疫性神经精神障碍的概念,类似于 PANDAS,表现为发声和运动抽动以及强迫症,已扩展到其他潜在的微生物以及相关的内源性和外源性疾病触发因素。这些具有内侧颞叶和海马体低代谢特征的疾病,在脑氟代脱氧葡萄糖正电子发射断层扫描融合磁共振成像(FDG PET-MRI)中得到了体现,形成了一个谱:一端是神经精神障碍图雷特综合征和具有明确病因发病机制的 PANDAS,另一端是儿科急性发作性神经精神综合征(PANS),单独存在或与特定细菌和病毒病原体相关。在没有特定触发因素的情况下,将 PANS 指定为排除性诊断,反映了目前分类学中的问题。

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