Miki Kawazoe, Shunsuke Koga, Hiroaki Sekiya, Keith Anthony Josephs, Neill R Graff-Radford, Dennis W Dickson
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The patients had been followed at Mayo Clinic and had autopsy diagnosis of either PSP or LBD. Patients were excluded if they had Alzheimer disease or a history of a disorder that could cause hydrocephalus, such as chronic meningitis or neoplasia. The study included 101 patients with PSP and 89 with LBD. The frequency of DESH and a high EI on brain MRI were analyzed in PSP and LBD with logistic regression analyses, adjusting for age, sex, and brain weight. The NPH triad of symptoms was assessed relative to imaging findings.</p><p><strong>Results: </strong>We found that DESH and high EI were similar between PSP and LBD. The mean age at death (PSP: 74.0 [8.2]; LBD: 80.0 [9.2]) and brain weight (PSP: 1,190 [123]; LBD: 1,300 [150]) were greater in LBD compared with PSP (<i>p</i> < 0.001 for each). The frequency of DESH was greater in LBD than PSP (13% vs 3%, <i>p</i> = 0.004), while a high EI was similar in PSP and LBD (36% vs 32%, <i>p</i> = 0.500). The adjusted odds ratios for DESH and high EI were similar between the 2 groups (DESH: adjusted ORs 0.3, 95% CI 0.06-1.25, <i>p</i> = 0.119; high EI: adjusted ORs 1.8, 95% CI 0.86-4.06, <i>p</i> = 0.120).</p><p><strong>Discussion: </strong>These findings suggest that DESH and high EI, often considered biomarkers for iNPH, may lack specificity and may be found in a subset of patients with PSP or LBD leading to unnecessary neurosurgery for iNPH.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. 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In this study, we investigated iNPH clinical and neuroimaging features in patients with autopsy-confirmed PSP or Lewy body disease (LBD) by assessing the normal pressure hydrocephalus (NPH) triad of symptoms and imaging features of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) and Evans index (EI) on antemortem MRI scans.</p><p><strong>Methods: </strong>Among our study participants (N = 190), the mean (SD) age was 76.8 (9.2) years and 134 (70.5%) were male. The patients had been followed at Mayo Clinic and had autopsy diagnosis of either PSP or LBD. Patients were excluded if they had Alzheimer disease or a history of a disorder that could cause hydrocephalus, such as chronic meningitis or neoplasia. The study included 101 patients with PSP and 89 with LBD. The frequency of DESH and a high EI on brain MRI were analyzed in PSP and LBD with logistic regression analyses, adjusting for age, sex, and brain weight. The NPH triad of symptoms was assessed relative to imaging findings.</p><p><strong>Results: </strong>We found that DESH and high EI were similar between PSP and LBD. The mean age at death (PSP: 74.0 [8.2]; LBD: 80.0 [9.2]) and brain weight (PSP: 1,190 [123]; LBD: 1,300 [150]) were greater in LBD compared with PSP (<i>p</i> < 0.001 for each). The frequency of DESH was greater in LBD than PSP (13% vs 3%, <i>p</i> = 0.004), while a high EI was similar in PSP and LBD (36% vs 32%, <i>p</i> = 0.500). 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引用次数: 0
摘要
背景和目的：一些研究表明特发性常压脑积水（iNPH）可以模仿其他神经退行性疾病，特别是进行性核上性麻痹（PSP）。在本研究中，我们通过评估死前MRI扫描中不成比例增大的蛛网膜下腔脑积水（DESH）和Evans指数（EI）的正常压力脑积水（NPH）三联征和影像学特征，探讨尸检证实的PSP或路易体病（LBD）患者的iNPH临床和神经影像学特征。方法：研究对象190例，平均（SD）年龄76.8（9.2）岁，男性134例（70.5%）。这些患者在梅奥诊所进行了随访，尸检诊断为PSP或LBD。如果患者患有阿尔茨海默病或有可能导致脑积水的疾病史，如慢性脑膜炎或肿瘤，则排除在外。该研究包括101例PSP患者和89例LBD患者。采用logistic回归分析分析PSP和LBD患者脑MRI上DESH和高EI的频率，并对年龄、性别和脑重量进行调整。相对于影像学表现评估NPH三联征。结果：我们发现PSP和LBD的DESH和高EI相似。平均死亡年龄(PSP: 74.0 [8.2]；LBD: 80.0[9.2])和脑重（PSP: 1190 [123]）；LBD: 1,300[150])在LBD中高于PSP （p < 0.001）。LBD患者DESH发生率高于PSP患者（13% vs 3%, p = 0.004），而高EI发生率在PSP和LBD患者中相似（36% vs 32%, p = 0.500）。两组间DESH和高EI的校正比值比相似(DESH：校正比值比0.3,95% CI 0.06-1.25, p = 0.119；高EI：调整后or为1.8,95% CI 0.86-4.06, p = 0.120)。讨论：这些发现表明，通常被认为是iNPH的生物标志物DESH和高EI可能缺乏特异性，并且可能在PSP或LBD患者中发现，导致不必要的iNPH神经外科手术。
Disproportionately Enlarged Subarachnoid-Space Hydrocephalus on MRI in Pathologically Confirmed Progressive Supranuclear Palsy.
Background and objective: Several studies have shown that idiopathic normal-pressure hydrocephalus (iNPH) can mimic other neurodegenerative disorders, particularly progressive supranuclear palsy (PSP). In this study, we investigated iNPH clinical and neuroimaging features in patients with autopsy-confirmed PSP or Lewy body disease (LBD) by assessing the normal pressure hydrocephalus (NPH) triad of symptoms and imaging features of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) and Evans index (EI) on antemortem MRI scans.
Methods: Among our study participants (N = 190), the mean (SD) age was 76.8 (9.2) years and 134 (70.5%) were male. The patients had been followed at Mayo Clinic and had autopsy diagnosis of either PSP or LBD. Patients were excluded if they had Alzheimer disease or a history of a disorder that could cause hydrocephalus, such as chronic meningitis or neoplasia. The study included 101 patients with PSP and 89 with LBD. The frequency of DESH and a high EI on brain MRI were analyzed in PSP and LBD with logistic regression analyses, adjusting for age, sex, and brain weight. The NPH triad of symptoms was assessed relative to imaging findings.
Results: We found that DESH and high EI were similar between PSP and LBD. The mean age at death (PSP: 74.0 [8.2]; LBD: 80.0 [9.2]) and brain weight (PSP: 1,190 [123]; LBD: 1,300 [150]) were greater in LBD compared with PSP (p < 0.001 for each). The frequency of DESH was greater in LBD than PSP (13% vs 3%, p = 0.004), while a high EI was similar in PSP and LBD (36% vs 32%, p = 0.500). The adjusted odds ratios for DESH and high EI were similar between the 2 groups (DESH: adjusted ORs 0.3, 95% CI 0.06-1.25, p = 0.119; high EI: adjusted ORs 1.8, 95% CI 0.86-4.06, p = 0.120).
Discussion: These findings suggest that DESH and high EI, often considered biomarkers for iNPH, may lack specificity and may be found in a subset of patients with PSP or LBD leading to unnecessary neurosurgery for iNPH.
期刊介绍：
Neurology® Genetics is an online open access journal publishing peer-reviewed reports in the field of neurogenetics. The journal publishes original articles in all areas of neurogenetics including rare and common genetic variations, genotype-phenotype correlations, outlier phenotypes as a result of mutations in known disease genes, and genetic variations with a putative link to diseases. Articles include studies reporting on genetic disease risk, pharmacogenomics, and results of gene-based clinical trials (viral, ASO, etc.). Genetically engineered model systems are not a primary focus of Neurology® Genetics, but studies using model systems for treatment trials, including well-powered studies reporting negative results, are welcome.
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