Nave A H, Harmel P, Buchert R, Harms L
Klinik und Hochschulambulanz für Neurologie, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Berlin Institute of Health (BIH), Berlin, Germany.
BMC Neurol. 2018 May 2;18(1):60. doi: 10.1186/s12883-018-1063-y.
Pediatric autoimmune neuropsychiatric disorder after streptococcal infection (PANDAS) is a specific autoimmune response to group-A streptococcal infections in children and adolescents with a sudden onset of obsessive-compulsive disorders or tic-like symptoms. Cerebral metabolic changes of patients have not yet been observed.
We present a case of an 18-year old male with a PANDAS-like condition after developing tic-like symptoms and involuntary movements three weeks after cardiac surgery. The patient had suffered from pharyngotonsillitis before the symptoms started. The anti-streptolysin O (ASO) titer was elevated (805 kU/l). Antibiotic therapy did not improve his condition. Intravenous immunoglobulins and high-dose cortisone therapy had minor beneficial effects on his involuntary movements. 18F-Fluorodeoxyglucose positron emission tomography/ computer tomography (18F-FDG PET/CT) demonstrated pronounced hypermetabolism of the basal ganglia and cortical hypometabolism. The patient was treated with five cycles of plasmapheresis. A marked clinical improvement was observed after four months. Cerebral metabolic alterations had completely normalized.
This is the first report of cerebral metabolic changes observed on FDG-PET/CT in a patient with a PANDAS-like condition with a normalization following immunomodulatory treatment. Cerebral FDG-PET/CT might be a promising tool in the diagnosis of PANDAS.
链球菌感染后儿童自身免疫性神经精神障碍(PANDAS)是儿童和青少年对A组链球菌感染的一种特异性自身免疫反应,可突然出现强迫症或抽动样症状。尚未观察到患者的脑代谢变化。
我们报告一例18岁男性患者,在心脏手术后三周出现抽动样症状和不自主运动,表现为类PANDAS病症。在症状出现前患者曾患咽扁桃体炎。抗链球菌溶血素O(ASO)滴度升高(805 kU/l)。抗生素治疗未能改善其病情。静脉注射免疫球蛋白和大剂量皮质类固醇治疗对其不自主运动有轻微改善作用。18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(18F-FDG PET/CT)显示基底节明显代谢亢进和皮质代谢减低。该患者接受了五个疗程的血浆置换治疗。四个月后观察到明显的临床改善。脑代谢改变已完全恢复正常。
这是首例关于类PANDAS病症患者经FDG-PET/CT观察到脑代谢变化且免疫调节治疗后恢复正常的报告。脑FDG-PET/CT可能是诊断PANDAS的一种有前景的工具。