Dale Russell C, Brilot Fabienne
Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Sydney, Australia.
J Child Neurol. 2012 Nov;27(11):1470-81. doi: 10.1177/0883073812451327. Epub 2012 Jul 25.
The basal ganglia are deep nuclei in the brain that include the caudate, putamen, globus pallidus, and substantia nigra. Pathological processes involving the basal ganglia often result in disorders of movement and behavior. A number of different autoimmune disorders predominantly involve the basal ganglia and can result in movement and psychiatric disorders. The classic basal ganglia autoimmune disorder is Sydenham chorea, a poststreptococcal neuropsychiatric disorder. Resurgence in the interest in Sydenham chorea is the result of the descriptions of other poststreptococcal neuropsychiatric disorders including tics and obsessive-compulsive disorder, broadly termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection. Encephalitic processes affecting the basal ganglia are also described including the syndromes basal ganglia encephalitis, encephalitis lethargica, and bilateral striatal necrosis. Last, systemic autoimmune disorders such as systemic lupus erythematosus and antiphospholipid syndrome can result in chorea or parkinsonism. Using paradigms learned from other autoantibody associated disorders, the authors discuss the autoantibody hypothesis and the role of systemic inflammation in autoimmune basal ganglia disorders. Identification of these entities is important as the clinician has an increasing therapeutic repertoire to modulate or suppress the aberrant immune system.
基底神经节是大脑中的深部核团，包括尾状核、壳核、苍白球和黑质。涉及基底神经节的病理过程常导致运动和行为障碍。许多不同的自身免疫性疾病主要累及基底神经节，并可导致运动和精神障碍。经典的基底神经节自身免疫性疾病是 Sydenham 舞蹈病，一种链球菌感染后的神经精神疾病。对 Sydenham 舞蹈病兴趣的复苏是由于对其他链球菌感染后神经精神疾病的描述，包括抽动症和强迫症，统称为与链球菌感染相关的儿童自身免疫性神经精神疾病。还描述了影响基底神经节的脑炎过程，包括基底神经节脑炎、昏睡性脑炎和双侧纹状体坏死综合征。最后，系统性自身免疫性疾病，如系统性红斑狼疮和抗磷脂综合征，可导致舞蹈病或帕金森综合征。作者利用从其他自身抗体相关疾病中学到的范例，讨论了自身抗体假说以及全身炎症在自身免疫性基底神经节疾病中的作用。识别这些疾病很重要，因为临床医生调节或抑制异常免疫系统的治疗方法越来越多。
