Comorbid alpha synucleinopathies in idiopathic normal pressure hydrocephalus
Comorbid alpha synucleinopathies in idiopathic normal pressure hydrocephalus
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DOI：
10.1007/s00415-021-10778-1
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发表时间：
2021-09-01
影响因子：
6
通讯作者：
Hattori, Nobutaka
中科院分区：
文献类型：
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作者：
Sakurai, Anri;Tsunemi, Taiji;Hattori, Nobutaka
目的探讨特发性常压脑积水（iNPH）患者帕金森病(PD)/帕金森痴呆（PD/PDD）或路易体痴呆（DLB）的患病率及临床特征。方法回顾性分析近10年来俊天大学医学院神经内科收治的iNPH患者资料。根据诊断标准确定iNPH和合并PD/PDD或DLB的诊断。运动症状由运动障碍协会发起的统一帕金森病评定量表（MDS-UPDRS） III修订版进行评估。采用i -123-碘氟烷单光子发射计算机断层扫描（pat - spect）和脑脊液（CSF）实时震动诱导转换（RT-QuIC）检测α -突触核蛋白聚集。结果79例患者符合iNPH标准，其中34例患者无伴发疾病（iNPHa, 43%）， 23例患者伴发PD/PDD (iNPHc + PD/PDD, 29.1%)， 8例患者伴发DLB （iNPHc + DLB, 10.1%）。面部扩张和上肢帕金森病在PD/PDD或DLB合并症中观察到显著差异。DaTscan的特异性结合比（SBR）在iNPHa中降低（p = 0.02），但在PD/PDD合共（p < 0.01）或DLB中进一步降低（p < 0.01）。RT-QuIC对所有13例共病PD/PDD均为阳性，对所有19例iNPHa均为阴性。结论突触核蛋白病与iNPH共存。这些可以通过进行DaTscan和RT-QuIC来区分，这可能会影响其临床特征。
Objective This study aimed to determine the prevalence and clinical features of Parkinson's disease (PD)/PD dementia (PD/PDD) or dementia with Lewy bodies (DLB) in idiopathic normal pressure hydrocephalus (iNPH). Methods Patients with iNPH who were admitted to the Department of Neurology, Juntendo University School of Medicine over the past 10 years have been retrospectively analyzed. The diagnosis of iNPH and concomitant PD/PDD or DLB was established using diagnostic criteria. Motor symptoms were assessed by the Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) III. I-123-ioflupane single-photon emission computed tomography (DaT-SPECT) and cerebrospinal fluid (CSF) real-time quaking-induced conversion (RT-QuIC)-based assay were performed for alpha synuclein aggregation. Results Overall, 79 patients met the criteria for iNPH, of which 34 developed iNPH without accompanying disorders (iNPHa; 43%), 23 developed iNPH with comorbid PD/PDD (iNPHc + PD/PDD; 29.1%), and 8 developed iNPH with comorbid DLB (iNPHc + DLB; 10.1%). Significant differences in facial expansion and upper-limb parkinsonism were observed with a comorbidity of either PD/PDD or DLB. The specific binding ratio (SBR) of DaTscan was reduced in iNPHa (p = 0.02), but it reduced further with comorbid PD/PDD (p < 0.01) or DLB (p < 0.01). RT-QuIC was positive for all 13 comorbid PD/PDD and negative for all 19 iNPHa. Conclusion These results highlight that synucleinopathies coexist with iNPH. These can be differentiated by performing DaTscan and RT-QuIC, which can affect its clinical features.