Comorbid alpha synucleinopathies in idiopathic normal pressure hydrocephalus

• Published in: Journal of neurology 2022-04, Vol.269 (4), p.2022-2029
• Main Authors: Sakurai, Anri, Tsunemi, Taiji, Ishiguro, Yuta, Okuzumi, Ayami, Hatano, Taku, Hattori, Nobutaka
• Format: Article
• Language: English
• Subjects: Basal ganglia; Brain diseases; Central nervous system diseases; Cerebrospinal fluid; Comorbidity; Computed tomography; Dementia; Dementia disorders; Humans; Hydrocephalus; Hydrocephalus, Normal Pressure - complications; Hydrocephalus, Normal Pressure - diagnostic imaging; Hydrocephalus, Normal Pressure - epidemiology; Lewy bodies; Lewy Body Disease - complications; Lewy Body Disease - diagnostic imaging; Lewy Body Disease - epidemiology; Medicine; Medicine & Public Health; Movement disorders; Neurodegenerative diseases; Neurology; Neuroradiology; Neurosciences; Original Communication; Parkinson Disease - complications; Parkinson Disease - diagnostic imaging; Parkinson Disease - epidemiology; Parkinson's disease; Patients; Retrospective Studies; Single photon emission computed tomography; Synuclein; Synucleinopathies - diagnostic imaging; Synucleinopathies - epidemiology
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-021-10778-1

Objective This study aimed to determine the prevalence and clinical features of Parkinson’s disease (PD)/PD dementia (PD/PDD) or dementia with Lewy bodies (DLB) in idiopathic normal pressure hydrocephalus (iNPH). Methods Patients with iNPH who were admitted to the Department of Neurology, Juntendo University School of Medicine over the past 10 years have been retrospectively analyzed. The diagnosis of iNPH and concomitant PD/PDD or DLB was established using diagnostic criteria. Motor symptoms were assessed by the Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) III. 123 I-ioflupane single-photon emission computed tomography (DaT-SPECT) and cerebrospinal fluid (CSF) real-time quaking-induced conversion (RT-QuIC)-based assay were performed for alpha synuclein aggregation. Results Overall, 79 patients met the criteria for iNPH, of which 34 developed iNPH without accompanying disorders (iNPHa; 43%), 23 developed iNPH with comorbid PD/PDD (iNPHc + PD/PDD; 29.1%), and 8 developed iNPH with comorbid DLB (iNPHc + DLB; 10.1%). Significant differences in facial expansion and upper-limb parkinsonism were observed with a comorbidity of either PD/PDD or DLB. The specific binding ratio (SBR) of DaTscan was reduced in iNPHa ( p  = 0.02), but it reduced further with comorbid PD/PDD ( p