Progressive supranuclear palsy often develops idiopathic normal pressure hydrocephalus‐like magnetic resonance imaging features

Abstract

Background and purpose 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. Methods 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. Results 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. Conclusions 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.

Magnetic resonance parkinsonism indices and interpeduncular angle in idiopathic normal pressure hydrocephalus and progressive supranuclear palsy

“…These indices proved to be useful in distinguishing PSP and iNPH from healthy controls and from other neurodegenerative diseases, but less useful to distinguish PSP and iNPH between each other [9].…”

Clinical Aspects of the Differential Diagnosis of Parkinson’s Disease and Parkinsonism

“… 102 In addition, there is increasing evidence for the importance of comorbidities in patients with iNPH such as Alzheimer’s disease and Parkinson-plus syndrome. 105 106 107 It is therefore important to find biomarkers for diagnosing iNPH as well as for selecting good candidates for surgery.…”

Co‐occurrence of idiopathic normal‐pressure hydrocephalus‐like magnetic resonance imaging features in progressive supranuclear palsy

“…Co-occurrence of idiopathic normal-pressure hydrocephalus-like magnetic resonance imaging features in progressive supranuclear palsy I read with great interest the article by Ohara et al [1], who found that progressive supranuclear palsy (PSP) patients more often develop idiopathic normal-pressure hydrocephalus (iNPH)-like magnetic resonance imaging (MRI) features than those with other neurodegenerative diseases. I congratulate the authors for conducting such an original study; however, I would like to make some comments to help clarify their report and present some crucial perspectives.…”

“…Remarkably, Ohara et al report that eight patients with PSP and two with Parkinson's disease (PD) received a CSF tap test based on iNPH-like MRI features [1]. Three out of the eight PSP patients and one PD patient were reported to have responded to the CSF tap test [1].…”

“…Remarkably, Ohara et al report that eight patients with PSP and two with Parkinson's disease (PD) received a CSF tap test based on iNPH-like MRI features [1]. Three out of the eight PSP patients and one PD patient were reported to have responded to the CSF tap test [1]. In our previous report [2] and in studies by several others [3], short-term recovery after shunt therapy in these patients with co-occurrent iNPH and PSP was demonstrated.…”