Department of Neurology and Neurological Science, Graduate School of Medical and Dental Science, Tokyo Medical and Dental University, Tokyo, Japan.
Eur J Neurol. 2020 Oct;27(10):1930-1936. doi: 10.1111/ene.14322. Epub 2020 Jun 29.
Idiopathic normal pressure hydrocephalus (iNPH) is a clinical entity without established pathological hallmarks. Previous autopsy studies reported that patients with an antemortem diagnosis of iNPH had a different postmortem diagnosis, commonly progressive supranuclear palsy (PSP). Disproportionately enlarged subarachnoid space hydrocephalus (DESH) has been reported as a characteristic feature of iNPH on magnetic resonance imaging (MRI). In addition, periventricular white matter hyperintensities (PVHs) are noted in most patients with iNPH; these PVHs are supposed to reflect transependymal movement of ventricular cerebrospinal fluid. It is hypothesized that PSP develops more iNPH-like MRI features than other neurodegenerative disorders.
Thirty-eight patients with a clinical diagnosis of PSP, 42 with Parkinson's disease (PD) without dementia, 30 with PD with dementia (PDD) and 29 with Alzheimer's disease (AD) were enrolled. The DESH score and PVH grade were measured using the conventional MRI sequence and were compared amongst the patient groups.
Disproportionately enlarged subarachnoid space hydrocephalus score was significantly higher in patients with PSP than PD without dementia, and there was a trend that the DESH score was higher in patients with PSP than PDD or Alzheimer's disease. PVH grade was significantly larger in patients with PSP than PD without dementia. In the components of the DESH score, callosal angle was significantly smaller in patients with PSP than in PD without dementia or PDD.
This study demonstrated that some PSP patients develop iNPH-like MRI features, suggesting the presence of iNPH-like features in the clinical spectrum of PSP. A clinical phenotype of PSP with hydrocephalus is proposed, which should be further investigated in future studies.
特发性正常压力脑积水（iNPH）是一种缺乏明确病理标志的临床实体。既往的尸检研究报告称，生前诊断为 iNPH 的患者死后的诊断通常为进行性核上性麻痹（PSP）。蛛网膜下腔过度扩张脑积水（DESH）被报道为磁共振成像（MRI）中 iNPH 的一个特征性表现。此外，大多数 iNPH 患者都有脑室周围白质高信号（PVHs）；这些 PVHs 被认为反映了脑室脑脊液的经室管膜转运。据推测，PSP 比其他神经退行性疾病发展出更多类似 iNPH 的 MRI 特征。
纳入 38 例临床诊断为 PSP 的患者、42 例无痴呆的帕金森病（PD）患者、30 例痴呆的 PD 患者（PDD）和 29 例阿尔茨海默病（AD）患者。使用常规 MRI 序列测量 DESH 评分和 PVH 分级，并比较各患者组之间的差异。
与无痴呆的 PD 患者相比，PSP 患者的 DESH 评分显著更高，且 PSP 患者的 DESH 评分较 PDD 或 AD 患者有升高趋势。与无痴呆的 PD 患者相比，PSP 患者的 PVH 分级更大。在 DESH 评分的各组成部分中，PSP 患者的胼胝体角显著小于无痴呆的 PD 或 PDD 患者。
本研究表明，一些 PSP 患者出现了类似 iNPH 的 MRI 特征，提示 PSP 临床谱中存在类似 iNPH 的特征。提出了一个伴有脑积水的 PSP 临床表型，应在未来的研究中进一步探讨。
