Progressive Supranuclear Palsy: A Crucial Co-occurrence in Normal Pressure Hydrocephalus or an Etiology of Hydrocephalic Presentation?

Abstract

Co-occurrences of neurodegenerative diseases in normal pressure hydrocephalus (NPH) is a remarkable topic of discussion, which has been mentioned in recent studies and it was also emphasized by the International Society for Hydrocephalus and Cerebrospinal Fluid Disorder. In this report, I illustrate two remarkable cases which were initially diagnosed with idiopathic normal pressure hydrocephalus (iNPH) and ventriculoperitoneal (VP) shunt surgery was performed, which yielded significant improvement in the clinic of both patients in the short-term period. However, in the long-term follow-up, both of them deteriorated, and re-evaluation of the neurological examination and neuroimaging data yielded the full clinical picture of progressive supranuclear palsy (PSP). Via the illustration of these cases and related literature data, I rediscuss some related hypotheses about the co-occurrence of these two conditions of hydrocephalus and PSP. I also suggest some distinct discussions to be clarified in future studies.

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

“…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. Nevertheless, long-term follow-up of patients also showed that the response to shunt therapy does not persist [2,3]. However, because there is no randomized controlled trial investigating the efficiency of shunt therapy in these iNPH patients with neurodegenerative comorbidity, reliable conclusions cannot be drawn about whether CSF diversion therapy has an overall beneficial role in the pathophysiology of NPH and/or underlying neurodegenerative disease.…”

“…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. Nevertheless, long-term follow-up of patients also showed that the response to shunt therapy does not persist [2,3].…”

Higher prevalence of idiopathic normal pressure hydrocephalus‐like MRI features in progressive supranuclear palsy: An imaging reminder of atypical parkinsonism

“…Though differentiating NPH from PSP may provide more precise prediction on therapeutic prognosis, several reports have demonstrated that shunting can achieve transient response in PSP with NPH-like radiologic features. (Morariu, 1979;Magdalinou et al, 2013;Onder, 2020). Most of the additional imaging indexes require specific settings or analysis; thus, wide clinical application is limited.…”