Skip to main content

Advertisement

SpringerLink
Log in

Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus

  • Original Article
  • Published:
Neurological Sciences Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Data availability

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

Code availability

Not applicable.

References

  1. 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

    Article  Google Scholar 

  2. Kato N, Arai K, Hattori T (2003) Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 210:57–60

    Article  Google Scholar 

  3. 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.28364

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Ohara M, Hattori T, Yokota T (2020) Progressive supranuclear palsy often develops idiopathic normal pressure hydrocephalus-like MRI features. Eur J Neurol 27:1930–1936

    Article  CAS  Google Scholar 

  5. 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 35:1406–1415

    Article  Google Scholar 

  6. 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

    Google Scholar 

  7. Lee JY, Im K, Kwon KY (2018) Parkinsonian patient with comorbid normal pressure hydrocephalus : clinical usefulness of diagnosis supported by neurological findings. Clin Neuroradiol 28:617–618

    Article  Google Scholar 

  8. Schott JM, Williams DR, Butterworth RJ, Janssen JC, Larner AJ, Holton JL, Rossor MN (2007) Shunt responsive progressive supranuclear palsy? Mov Disord 30:902–903

    Article  Google Scholar 

  9. Starr BW, Hagen MC, Espay AJ (2014) Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics. J Clin Mov Disord 29:1–2

    Article  Google Scholar 

  10. 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 260:1009–1013

    Article  Google Scholar 

  11. 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 82:503–513

    Article  Google Scholar 

  12. 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) 52:775–809

    Article  Google Scholar 

  13. 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 47:1–9

    Article  CAS  Google Scholar 

  14. 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 128:1247–1258

    Article  Google Scholar 

  15. 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 285:197–205

    Article  Google Scholar 

  16. 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

    Google Scholar 

  17. 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 12:189–198

    Article  CAS  Google Scholar 

  18. 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 25:37–45

    Article  Google Scholar 

  19. Appollonio I, Leone M, Isella V, Piamarta F, Consoli T et al (2005) The frontal assessment battery (FAB): normative values in an Italian population sample. Neurol Sci 26:108–116. https://doi.org/10.1007/s10072-005-0443-4

    Article  CAS  PubMed  Google Scholar 

  20. Wechsler D (1981) Manual for the Wechsler Adult Intelligence Scale Revised. Psychological Corporation, New York

    Google Scholar 

  21. 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/10.1159/000117297

    Article  CAS  PubMed  Google Scholar 

  22. Benton AL, Varney NR, Hamsher KD (1978) Visuospatial judgment. A clinical test. Arch Neurol 35:364–367. https://doi.org/10.1001/archneur.1978.00500300038006

    Article  CAS  PubMed  Google Scholar 

  23. (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. 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 18:2678–2683

    Article  Google Scholar 

  25. Dale AM, Fischl B, Sereno MI (1999) Cortical surface-based analysis. I. Segmentation and surface reconstruction. Neuroimage 9:179–194

    Article  CAS  Google Scholar 

  26. 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 3:489–496

    Article  Google Scholar 

  27. Marmarou A, Bergsneider M, Klinge P, Relkin N, Black PM (2005) The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurosurgery 57:S17-28. https://doi.org/10.1227/01.neu.0000168184.01002.60 (discussion ii-v)

    Article  PubMed  Google Scholar 

  28. Mihalj M, Dolić K, Kolić K, Ledenko V (2016) CSF tap test - Obsolete or appropriate test for predicting shunt responsiveness? A systemic review. J Neurol Sci 362:78–84. https://doi.org/10.1016/j.jns.2016.01.028

    Article  PubMed  Google Scholar 

  29. 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 34:1144–1153

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Institute of Neurology, University “Magna Graecia”, Catanzaro, Italy

    Andrea Quattrone, Maurizio Morelli & Alessandro Mechelli

  2. Department of Medical and Surgical Sciences, Neuroscience Centre, University “Magna Graecia”, Catanzaro, Italy

    Alessia Sarica

  3. Institute of Neurosurgery, University “Magna Graecia”, Catanzaro, Italy

    Domenico La Torre

  4. Department of Radiology, Pugliese-Ciaccio Hospital, Catanzaro, Italy

    Pier Paolo Arcuri

  5. Neuroscience Research Center, University “Magna Graecia”, Catanzaro, Italy

    Aldo Quattrone

  6. Institute of Molecular Bioimaging and Physiology, National Research Council (IBFM-CNR), Catanzaro, Italy

    Aldo Quattrone

  7. Neuroscience Centre and Neuroimaging Research Unit, Institute of Molecular Bioimaging and Physiology, National Research Council, Magna Graecia University, 88100, Catanzaro, Italy

    Aldo Quattrone

Authors
  1. Andrea Quattrone
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Alessia Sarica
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Domenico La Torre
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Maurizio Morelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Alessandro Mechelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Pier Paolo Arcuri
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Aldo Quattrone
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Andrea Quattrone and Aldo Quattrone contributed to the study conception and design. All authors contributed to the data collection and analysis. The first draft of the manuscript was written by Andrea Quattrone. All authors revised the article critically for intellectual content and approved the final version of the manuscript to be submitted.

Corresponding author

Correspondence to Aldo Quattrone.

Ethics declarations

Ethics approval

This study was performed in line with the principles of the Declaration of Helsinki. All study procedures and ethical aspects were approved by the institutional review board of Magna Graecia University, Catanzaro, Italy.

Consent to participate

Written informed consent was obtained from each participant included in the study.

Consent for publication

All authors have approved the manuscript before submission, including the names and order of authors; all gave explicit consent to submit and they obtained consent from the responsible authorities at the institute/organization where the work has been carried out.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Quattrone, A., Sarica, A., La Torre, D. et al. Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus. Neurol Sci 43, 1783–1790 (2022). https://doi.org/10.1007/s10072-021-05594-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10072-021-05594-4

Keywords

Search

Navigation