Pathologically Verified Corticobasal Degeneration Mimicking Richardson's Syndrome Coexisting with Clinically and Radiologically Shunt-Responsive Normal Pressure Hydrocephalus.
Pathologically Verified Corticobasal Degeneration Mimicking Richardson's Syndrome Coexisting with Clinically and Radiologically Shunt-Responsive Normal Pressure Hydrocephalus.
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经过病理验证的皮质性皮质变性，模仿了理查森综合征与临床和放射学上的反应性正常压力脑化脑化的综合征。
DOI：
10.1002/mdc3.13442
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发表时间：
2022-05
影响因子：
4
通讯作者：
Takahashi, Yuji
中科院分区：
文献类型：
--
作者：
Saitoh, Yuji;Iwasaki, Masaki;Mizutani, Masashi;Kimura, Yukio;Hasegawa, Masato;Sato, Noriko;Takao, Masaki;Takahashi, Yuji
关键词：
正常压力脑积水（NPH）表现为步态不稳定、认知障碍和尿失禁。这种 NPH 临床三联征有时会与神经退行性疾病患者的脑室扩大一起发生。患有经病理证实的神经退行性疾病（例如进行性核上性麻痹（PSP））的患者在生前接受过 NPH 诊断。本研究介绍了一名经病理证实的皮质基底节变性 (CBD) 与临床分流反应性 NPH 共存的患者的临床和病理特征。我们对一名 CBD 患者进行了临床、放射学和病理学评估，该患者生前诊断为 PSP 理查森综合征 (PSP-RS)，并伴有分流反应性 NPH。一名 59 岁女性出现运动迟缓和步态不稳定，随后出现频繁跌倒、尿失禁和核上垂直凝视麻痹。 63岁时，她的步态障碍和尿失禁迅速恶化，并出现认知障碍。 NPH 的典型表现包括脑室扩大、蛛网膜下腔异常扩大、脑积水以及脑血流灌注减少和增加的 2 层外观。分流器放置改善了一年多的步态不稳定性，并改善了 NPH 的放射学指标。然而，中脑在尺寸短暂增加后随着时间的推移而萎缩。尽管生前诊断很可能是 PSP-RS，但病理评估证实了 CBD。侧脑室室管膜内壁严重不连续，室管膜下稀疏，神经胶质增生伴 tau 阳性沉积。分流手术可以改善 4 次重复 tau蛋白病患者的 NPH 症状。有必要对临床结果进行仔细评估，以预测分流术作为神经退行性疾病与 NPH 共存患者的治疗选择的益处。
Normal pressure hydrocephalus (NPH) manifests as gait instability, cognitive impairment, and urinary incontinence. This clinical triad of NPH sometimes occurs with ventriculomegaly in patients with neurodegenerative disease. Patients with pathologically verified neurodegenerative diseases, such as progressive supranuclear palsy (PSP), have received antemortem diagnoses of NPH. This study presents clinical and pathological features of a patient with pathologically verified corticobasal degeneration (CBD) coexisting with clinically shunt‐responsive NPH. We performed clinical, radiological, and pathological evaluations in a patient with CBD whose antemortem diagnosis was PSP Richardson's syndrome (PSP‐RS) coexisting with shunt‐responsive NPH. A 59‐year‐old woman developed bradykinesia and gait instability and then frequent falls, urinary incontinence, and supranuclear vertical gaze palsy followed. At 63 years of age, her gait disturbance and urinary incontinence had deteriorated rapidly, and cognitive impairment was disclosed. There were typical findings of NPH with ventriculomegaly and disproportionately enlarged subarachnoid space hydrocephalus as well as a 2‐layer appearance with decreased and increased cerebral blood perfusion. Shunt placement ameliorated gait instability for more than 1 year and improved radiological indicators of NPH. However, atrophy of the midbrain progressed with time after transient increases in size. Although the antemortem diagnosis was probable PSP‐RS, pathological evaluation verified CBD. There were severe discontinuities of the ependymal lining of the lateral ventricles and subependymal rarefaction and gliosis with tau‐positive deposition. Shunt surgery could ameliorate NPH symptoms in patients with 4‐repeat tauopathies. Careful assessments of clinical findings are necessary to predict the benefits of shunts as a therapeutic option for patients with neurodegenerative diseases coexisting with NPH.
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DOI：
10.1186/2054-7072-1-2
发表时间：
2014
期刊：
Journal of clinical movement disorders
影响因子：
--
作者：
Starr BW;Hagen MC;Espay AJ
通讯作者：
Espay AJ
影响因子：
11.2
作者：
Arai, T;Ikeda, K;Mochizuki, A
通讯作者：
Mochizuki, A
DOI：
10.1002/mds.26987
发表时间：
2017-06
期刊：
Movement disorders : official journal of the Movement Disorder Society
影响因子：
--
作者：
Höglinger GU;Respondek G;Stamelou M;Kurz C;Josephs KA;Lang AE;Mollenhauer B;Müller U;Nilsson C;Whitwell JL;Arzberger T;Englund E;Gelpi E;Giese A;Irwin DJ;Meissner WG;Pantelyat A;Rajput A;van Swieten JC;Troakes C;Antonini A;Bhatia KP;Bordelon Y;Compta Y;Corvol JC;Colosimo C;Dickson DW;Dodel R;Ferguson L;Grossman M;Kassubek J;Krismer F;Levin J;Lorenzl S;Morris HR;Nestor P;Oertel WH;Poewe W;Rabinovici G;Rowe JB;Schellenberg GD;Seppi K;van Eimeren T;Wenning GK;Boxer AL;Golbe LI;Litvan I;Movement Disorder Society-endorsed PSP Study Group
通讯作者：
Movement Disorder Society-endorsed PSP Study Group
影响因子：
8.6
作者：
Quattrone, Andrea;Sarica, Alessia;Quattrone, Aldo
通讯作者：
Quattrone, Aldo
影响因子：
2.8
作者：
Ugga L;Cuocolo R;Cocozza S;Pontillo G;Elefante A;Quarantelli M;Vicidomini C;De Pandis MF;De Michele G;D'Amico A;de Divitiis O;Brunetti A
通讯作者：
Brunetti A