Lateralized ante mortem and post mortem pathology in a case of Lewy body disease with corticobasal syndrome.
Lateralized ante mortem and post mortem pathology in a case of Lewy body disease with corticobasal syndrome.
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在患有皮质综合征的Lewy身体疾病的病例中，侧向验尸和验尸病理学。
DOI：
10.1002/trc2.12294
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发表时间：
2022
影响因子：
4.8
通讯作者：
Irwin, David J
中科院分区：
文献类型：
--
作者：
Coughlin, David G;Coslett, H Branch;Peterson, Claire;Phillips, Jeffrey S;McMillan, Corey;Lee, Edward B;Trojanowski, John Q;Grossman, Murray;Irwin, David J
路易体疾病的病理学特征是α-突触核蛋白病理学。阿尔茨海默病 (AD) 的共同病理学可以影响表型。体内 AD 生物标志物可以表明在异常情况下存在这种共同病理，但病理验证仍然至关重要。该患者最初出现皮质基底节综合征，后来出现幻视和帕金森病，与突触核蛋白病一致。该患者接受了脑脊液采样、18F-flortaucipir PET 扫描和大脑捐赠，双侧区域可用于数字组织学分析。 CSF Aβ42 和 t-tau 位于 AD 范围内。 18F-flortaucipir 扫描显示所有肺叶均存在右侧保留（t = 4.3‐10.0，P < .006）。尸检时发现新皮质阶段路易体病理学和高水平的 AD 神经病理学变化。 α-突触核蛋白和 tau 病理学存在右侧偏化（分别为 T 值 = 3.1、P 值 = .007 和 T 值 = 3.3、P 值 = .004）。该病例具有重叠的 tau 蛋白病和突触核蛋白病临床特征，具有深入的生物标志物特征，罕见的双侧尸检采样显示偏侧 tau 蛋白和 α-突触核蛋白病理学，表明可能存在协同关系。
Lewy body diseases are pathologically characterized by α‐synuclein pathology. Alzheimer's disease (AD) co‐pathology can influence phenotypes. In vivo AD biomarkers can suggest the presence of this co‐pathology in unusual cases, but pathological validation remains essential. This patient originally presented with corticobasal syndrome and later developed visual hallucinations and parkinsonism consistent with a synucleinopathy. The patient underwent CSF sampling, 18F‐flortaucipir PET scanning, and brain donation with bilateral regions available for digital histological analysis. CSF Aβ42 and t‐tau were in the AD range. 18F‐flortaucipir scanning showed right‐lateralized retention in all lobes (t = 4.3‐10.0, P < .006). Neocortical stage Lewy body pathology and high levels of AD neuropathological changes were present at autopsy. There was right lateralization of α‐synuclein and tau pathology (T value = 3.1, P value = .007 and T value = 3.3, P value = .004 respectively). This case with overlapping tauopathy and synucleinopathy clinical features had in‐depth biomarker characterization and rare bilateral post‐mortem sampling showing lateralized tau and α‐synuclein pathology suggesting possible synergistic relationships.