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Published in: Neurological Sciences 3/2022

01-03-2022 | Hydrocephalus | Original Article

Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus

Authors: Andrea Quattrone, Alessia Sarica, Domenico La Torre, Maurizio Morelli, Alessandro Mechelli, Pier Paolo Arcuri, Aldo Quattrone

Published in: Neurological Sciences | Issue 3/2022

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Abstract

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.
Literature
1.
go back to reference Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T, Englund E, Gelpi E, Giese A, Irwin DJ, Meissner WG, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Antonini A, Bhatia KP, Bordelon Y, Compta Y, Corvol JC, Colosimo C, Dickson DW, Dodel R, Ferguson L, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris HR, Nestor P, Oertel WH, Poewe W, Rabinovici G, Rowe JB, Schellenberg GD, Seppi K, van Eimeren T, Wenning GK, Boxer AL, Golbe LI, Litvan I, for the Movement Disorder Society- endorsed PSP study group (2017) Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord 6:853–864 CrossRef Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T, Englund E, Gelpi E, Giese A, Irwin DJ, Meissner WG, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Antonini A, Bhatia KP, Bordelon Y, Compta Y, Corvol JC, Colosimo C, Dickson DW, Dodel R, Ferguson L, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris HR, Nestor P, Oertel WH, Poewe W, Rabinovici G, Rowe JB, Schellenberg GD, Seppi K, van Eimeren T, Wenning GK, Boxer AL, Golbe LI, Litvan I, for the Movement Disorder Society- endorsed PSP study group (2017) Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord 6:853–864 CrossRef
2.
go back to reference Kato N, Arai K, Hattori T (2003) Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 210:57–60CrossRef Kato N, Arai K, Hattori T (2003) Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 210:57–60CrossRef
3.
go back to reference Quattrone A, Antonini A, Vaillancourt DE, Seppi K, Ceravolo R, Strafella AP, Morelli M, Nigro S, Vescio B, Bianco MG, Vasta R, Arcuri PP, Weis L, Fiorenzato E, Biundo R, Burciu RG, Krismer F, McFarland NR, Mueller C, Gizewski ER, Cosottini M, Del Prete E, Mazzucchi S, Quattrone A (2020) A new MRI measure to early differentiate progressive supranuclear palsy from de novo Parkinson’s disease in clinical practice: an international study. Mov Disord 36(3):681–689. https://doi.org/10.1002/mds.28364CrossRefPubMedPubMedCentral Quattrone A, Antonini A, Vaillancourt DE, Seppi K, Ceravolo R, Strafella AP, Morelli M, Nigro S, Vescio B, Bianco MG, Vasta R, Arcuri PP, Weis L, Fiorenzato E, Biundo R, Burciu RG, Krismer F, McFarland NR, Mueller C, Gizewski ER, Cosottini M, Del Prete E, Mazzucchi S, Quattrone A (2020) A new MRI measure to early differentiate progressive supranuclear palsy from de novo Parkinson’s disease in clinical practice: an international study. Mov Disord 36(3):681–689. https://​doi.​org/​10.​1002/​mds.​28364CrossRefPubMedPubMedCentral
4.
go back to reference Ohara M, Hattori T, Yokota T (2020) Progressive supranuclear palsy often develops idiopathic normal pressure hydrocephalus-like MRI features. Eur J Neurol 27:1930–1936CrossRef Ohara M, Hattori T, Yokota T (2020) Progressive supranuclear palsy often develops idiopathic normal pressure hydrocephalus-like MRI features. Eur J Neurol 27:1930–1936CrossRef
5.
go back to reference Quattrone A, Sarica A, La Torre D, Morelli M, Vescio B, Nigro S, Barbagallo G, Nisticò R, Salsone M, Arcuri PP, Novellino F, Bianco MG, Arabia G, Cascini G, Quattrone A (2020) Magnetic resonance imaging biomarkers distinguish normal pressure hydrocephalus from progressive supranuclear palsy. Mov Disord 35:1406–1415CrossRef Quattrone A, Sarica A, La Torre D, Morelli M, Vescio B, Nigro S, Barbagallo G, Nisticò R, Salsone M, Arcuri PP, Novellino F, Bianco MG, Arabia G, Cascini G, Quattrone A (2020) Magnetic resonance imaging biomarkers distinguish normal pressure hydrocephalus from progressive supranuclear palsy. Mov Disord 35:1406–1415CrossRef
6.
go back to reference Cucca A, Biagioni MC, Sharma K, Golomb J, Gilbert RM, Di Rocco A, Fleisher JE (2018) Comorbid normal pressure hydrocephalus with parkinsonism: a clinical challenge and call for awareness. Case Rep Neurol Med 21:2513474 Cucca A, Biagioni MC, Sharma K, Golomb J, Gilbert RM, Di Rocco A, Fleisher JE (2018) Comorbid normal pressure hydrocephalus with parkinsonism: a clinical challenge and call for awareness. Case Rep Neurol Med 21:2513474
7.
go back to reference Lee JY, Im K, Kwon KY (2018) Parkinsonian patient with comorbid normal pressure hydrocephalus : clinical usefulness of diagnosis supported by neurological findings. Clin Neuroradiol 28:617–618CrossRef Lee JY, Im K, Kwon KY (2018) Parkinsonian patient with comorbid normal pressure hydrocephalus : clinical usefulness of diagnosis supported by neurological findings. Clin Neuroradiol 28:617–618CrossRef
8.
go back to reference Schott JM, Williams DR, Butterworth RJ, Janssen JC, Larner AJ, Holton JL, Rossor MN (2007) Shunt responsive progressive supranuclear palsy? Mov Disord 30:902–903CrossRef Schott JM, Williams DR, Butterworth RJ, Janssen JC, Larner AJ, Holton JL, Rossor MN (2007) Shunt responsive progressive supranuclear palsy? Mov Disord 30:902–903CrossRef
9.
go back to reference Starr BW, Hagen MC, Espay AJ (2014) Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics. J Clin Mov Disord 29:1–2CrossRef Starr BW, Hagen MC, Espay AJ (2014) Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics. J Clin Mov Disord 29:1–2CrossRef
10.
go back to reference Magdalinou NK, Ling H, Smith JD, Schott JM, Watkins LD, Lees AJ (2013) Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series. J Neurol 260:1009–1013CrossRef Magdalinou NK, Ling H, Smith JD, Schott JM, Watkins LD, Lees AJ (2013) Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series. J Neurol 260:1009–1013CrossRef
11.
go back to reference Espay AJ, Da Prat GA, Dwivedi AK, Rodriguez-Porcel F, Vaughan JE, Rosso M, Devoto JL, Duker AP, Masellis M, Smith CD, Mandybur GT, Merola A, A.E., Lang, (2017) Deconstructing normal pressure hydrocephalus: Ventriculomegaly as early sign of neurodegeneration. Ann Neurol 82:503–513CrossRef Espay AJ, Da Prat GA, Dwivedi AK, Rodriguez-Porcel F, Vaughan JE, Rosso M, Devoto JL, Duker AP, Masellis M, Smith CD, Mandybur GT, Merola A, A.E., Lang, (2017) Deconstructing normal pressure hydrocephalus: Ventriculomegaly as early sign of neurodegeneration. Ann Neurol 82:503–513CrossRef
12.
go back to reference Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, Nakajima M, Hashimoto M, Kuriyama N, Tokuda T, Ishii K, Kaijima M, Hirata Y, Saito M, Arai H, Japanese Society of Normal Pressure Hydrocephalus (2012) Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Neurol Med Chir (Tokyo) 52:775–809CrossRef Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, Nakajima M, Hashimoto M, Kuriyama N, Tokuda T, Ishii K, Kaijima M, Hirata Y, Saito M, Arai H, Japanese Society of Normal Pressure Hydrocephalus (2012) Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Neurol Med Chir (Tokyo) 52:775–809CrossRef
13.
go back to reference Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1–9CrossRef Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1–9CrossRef
14.
go back to reference Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 128:1247–1258CrossRef Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 128:1247–1258CrossRef
15.
go back to reference Miskin N, Patel H, Franceschi AM, Ades-Aron B, Le A, Damadian BE, Stanton C, Serulle Y, Golomb J, Gonen O, Rusinek H, George AE, Alzheimer’s disease neuroimaging initiative (2017) Diagnosis of normal-pressure hydrocephalus: use of traditional measures in the era of volumetric MR imaging. Radiology 285:197–205CrossRef Miskin N, Patel H, Franceschi AM, Ades-Aron B, Le A, Damadian BE, Stanton C, Serulle Y, Golomb J, Gonen O, Rusinek H, George AE, Alzheimer’s disease neuroimaging initiative (2017) Diagnosis of normal-pressure hydrocephalus: use of traditional measures in the era of volumetric MR imaging. Radiology 285:197–205CrossRef
16.
go back to reference Fahn S, Elton RL (1987) Unified Parkinson’s Disease Rating Scale. In: Fahn S, Marsden CD, Calne D, Goldstein M (eds) Recent developments in Parkinson’s disease. MacMillan Healthcare Information, Florham Park, pp 153–163 Fahn S, Elton RL (1987) Unified Parkinson’s Disease Rating Scale. In: Fahn S, Marsden CD, Calne D, Goldstein M (eds) Recent developments in Parkinson’s disease. MacMillan Healthcare Information, Florham Park, pp 153–163
17.
go back to reference Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state:” A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198CrossRef Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state:” A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198CrossRef
18.
go back to reference Kubo Y, Kazui H, Yoshida T, Kito Y, Kimura N, Tokunaga H, Ogino A, Miyake H, Ishikawa M, Takeda M (2008) Validation of grading scale for evaluating symptoms of idiopathic normal-pressure hydrocephalus. Dement Geriatr Cogn Disord 25:37–45CrossRef Kubo Y, Kazui H, Yoshida T, Kito Y, Kimura N, Tokunaga H, Ogino A, Miyake H, Ishikawa M, Takeda M (2008) Validation of grading scale for evaluating symptoms of idiopathic normal-pressure hydrocephalus. Dement Geriatr Cogn Disord 25:37–45CrossRef
20.
go back to reference Wechsler D (1981) Manual for the Wechsler Adult Intelligence Scale Revised. Psychological Corporation, New York Wechsler D (1981) Manual for the Wechsler Adult Intelligence Scale Revised. Psychological Corporation, New York
21.
go back to reference Carlesimo GA, Caltagirone C, Gainotti G (1996) The mental deterioration battery: normative data, diagnostic reliability and qualitative analyses of cognitive impairment. The Group for the Standardization of the Mental Deterioration Battery. Eur Neurol 36:378–84. https://doi.org/10.1159/000117297CrossRefPubMed Carlesimo GA, Caltagirone C, Gainotti G (1996) The mental deterioration battery: normative data, diagnostic reliability and qualitative analyses of cognitive impairment. The Group for the Standardization of the Mental Deterioration Battery. Eur Neurol 36:378–84. https://​doi.​org/​10.​1159/​000117297CrossRefPubMed
23.
go back to reference (1987) Italian standardization and classification of Neuropsychological tests. The Italian Group on the Neuropsychological Study of Aging. Ital J Neurol Sci Suppl 8:1–120 (1987) Italian standardization and classification of Neuropsychological tests. The Italian Group on the Neuropsychological Study of Aging. Ital J Neurol Sci Suppl 8:1–120
24.
go back to reference Ishii K, Kanda T, Harada A, Miyamoto N, Kawaguchi T, Shimada K, Ohkawa S, Uemura T, Yoshikawa T, Mori E (2008) Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus. Eur Radiol 18:2678–2683CrossRef Ishii K, Kanda T, Harada A, Miyamoto N, Kawaguchi T, Shimada K, Ohkawa S, Uemura T, Yoshikawa T, Mori E (2008) Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus. Eur Radiol 18:2678–2683CrossRef
25.
go back to reference Dale AM, Fischl B, Sereno MI (1999) Cortical surface-based analysis. I. Segmentation and surface reconstruction. Neuroimage 9:179–194CrossRef Dale AM, Fischl B, Sereno MI (1999) Cortical surface-based analysis. I. Segmentation and surface reconstruction. Neuroimage 9:179–194CrossRef
26.
go back to reference Ishii K, Soma T, Shimada K, Oda H, Terashima A, Kawasaki R (2013) Automatic volumetry of the cerebrospinal fluid space in idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Dis Extra 3:489–496CrossRef Ishii K, Soma T, Shimada K, Oda H, Terashima A, Kawasaki R (2013) Automatic volumetry of the cerebrospinal fluid space in idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Dis Extra 3:489–496CrossRef
29.
go back to reference Ali F, Martin PR, Botha H, Ahlskog JE, Bower JH, Masumoto JY, Maraganore D, Hassan A, Eggers S, Boeve BF, Knopman DS, Drubach D, Petersen RC, Dunkley ED, van Gerpen J, Uitti R, Whitwell JL, Dickson DW, Josephs KA (2019) Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Mov Disord 34:1144–1153CrossRef Ali F, Martin PR, Botha H, Ahlskog JE, Bower JH, Masumoto JY, Maraganore D, Hassan A, Eggers S, Boeve BF, Knopman DS, Drubach D, Petersen RC, Dunkley ED, van Gerpen J, Uitti R, Whitwell JL, Dickson DW, Josephs KA (2019) Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Mov Disord 34:1144–1153CrossRef
Metadata
Title
Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus
Authors
Andrea Quattrone
Alessia Sarica
Domenico La Torre
Maurizio Morelli
Alessandro Mechelli
Pier Paolo Arcuri
Aldo Quattrone
Publication date
01-03-2022
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 3/2022
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-021-05594-4

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