Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus

• Published in: Neurological sciences Vol. 43; no. 3; pp. 1783 - 1790
• Main Authors: Quattrone, Andrea, Sarica, Alessia, La Torre, Domenico, Morelli, Maurizio, Mechelli, Alessandro, Arcuri, Pier Paolo, Quattrone, Aldo
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.03.2022; Springer Nature B.V
• Subjects: Coexistence; Corpus Callosum - pathology; Dilatation; Enlargement; Humans; Hydrocephalus; Hydrocephalus, Normal Pressure - complications; Hydrocephalus, Normal Pressure - diagnostic imaging; Magnetic Resonance Imaging - methods; Medicine; Medicine & Public Health; Mimicry; Neurology; Neuroradiology; Neurosciences; Neurosurgery; Original Article; Paralysis; Patients; Progressive supranuclear palsy; Psychiatry; Sexually transmitted diseases; STD; Supranuclear Palsy, Progressive - complications; Supranuclear Palsy, Progressive - diagnostic imaging; Ventricle; Ventricular dilatation; Normal pressure hydrocephalus; Automated volumetry; Callosal angle; Progressive supranuclear palsy
• ISSN: 1590-1874; 1590-3478; 1590-3478
• DOI: 10.1007/s10072-021-05594-4

Background Progressive supranuclear palsy (PSP) patients can show ventricular enlargement mimicking normal pressure hydrocephalus (NPH). The aim of this study was to distinguish PSP patients with marked ventricular dilatation (PSP-vd) from those with normal ventricular system and to evaluate the coexistence of NPH in PSP-vd patients. Methods One hundred three probable PSP patients, 18 definite NPH patients, and 41 control subjects were enrolled in the study. Evans index (EI) > 0.32 associated with callosal angle (CA) < 100° was used to identify PSP-vd patients. Automated ventricular volumetry (AVV) and Magnetic Resonance Hydrocephalic Index (MRHI) were performed on T1-weighted MR images to evaluate the presence of NPH in PSP-vd patients. Results Twelve (11.6%) out of 103 PSP patients had both abnormal EI and CA values (PSP-vd). In two of these 12 patients, AVV and MRHI values suggested PSP + NPH. In the remaining 10 PSP-vd patients, AVV and MRHI values were higher than PSP patients with normal ventricular system and controls, but lower than PSP + NPH and NPH patients, suggesting a non-hydrocephalic ventricular enlargement. Discussion Our study provides evidence that the combination of EI and CA biomarkers allowed to identify PSP patients with marked ventricular dilatation mimicking NPH. Only a few of these patients had PSP + NPH. Recognition of these PSP patients with enlarged ventricles can positively impact the care of this disease, helping clinicians to identify patients with PSP + NPH who could benefit from shunt procedure and avoid surgery in those with enlarged ventricles without NPH.