Neuropathological findings in possible normal pressure hydrocephalus: A post-mortem study of 29 cases with lifelines

Authors

  • Joni J. Hänninen The Department of Pathology, Kuopio University Hospital and the Institute of Clinical Medicine – Pathology, University of Eastern Finland, Kuopio, Finland; The Department of Neurosurgery, Kuopio University Hospital and the Institute of Clinical Medicine – Neurosurgery, University of Eastern Finland, Kuopio, Finland
  • Madoka Nakajima The Department of Neurosurgery, Juntendo University Faculty of Medicine, Tokyo, Japan
  • Aleksi Vanninen The Department of Pathology, Kuopio University Hospital and the Institute of Clinical Medicine – Pathology, University of Eastern Finland, Kuopio, Finland; The Department of Neurosurgery, Kuopio University Hospital and the Institute of Clinical Medicine – Neurosurgery, University of Eastern Finland, Kuopio, Finland
  • Santtu Hytönen The National Institute for Health and Welfare, Forensic Medicine, Kuopio, Finland
  • Jaana Rummukainen The Department of Pathology, Kuopio University Hospital, Kuopio, Finland
  • Anne Maria Koivisto The Department of Neurology, Kuopio University Hospital and the Institute of Clinical Medicine – Neurology, University of Eastern Finland, Kuopio, Finland; The Department of Neurosciences, Medical Faculty, University of Helsinki, Helsinki, Finland; The Department of Geriatrics/Rehabilitation and Internal Medicine, Helsinki University Hospital, Helsinki, Finland
  • Juha E. Jääskeläinen The Department of Neurosurgery, Kuopio University Hospital and the Institute of Clinical Medicine – Neurosurgery, University of Eastern Finland, Kuopio, Finland
  • Hilkka Soininen The Department of Neurology, Kuopio University Hospital, Kuopio, Finland
  • Anna Sutela The Department of Radiology, Kuopio University Hospital and the Institute of Clinical Medicine – Radiology, University of Eastern Finland, Kuopio, Finland
  • Ritva Vanninen The Department of Radiology, Kuopio University Hospital and the Institute of Clinical Medicine – Radiology, University of Eastern Finland, Kuopio, Finland
  • Mikko Hiltunen The Institute of Biomedicine, University of Eastern Finland, Kuopio, Finland
  • Ville Leinonen The Department of Neurosurgery, Kuopio University Hospital and the Institute of Clinical Medicine – Neurosurgery, University of Eastern Finland, Kuopio, Finland
  • Tuomas Rauramaa The Department of Pathology, Kuopio University Hospital and the Institute of Clinical Medicine – Pathology, University of Eastern Finland, Kuopio, Finland

DOI:

https://doi.org/10.17879/freeneuropathology-2022-3331

Keywords:

Hydrocephalus, Normal pressure hydrocephalus, Post-mortem, Neuropathology, Neurodegeneration

Abstract

Aims: There are very few detailed post-mortem studies on idiopathic normal-pressure hydrocephalus (iNPH) and there is a lack of proper neuropathological criteria for iNPH. This study aims to update the knowledge on the neuropathology of iNPH and to develop the neuropathological diagnostic criteria of iNPH.
Methods: We evaluated the clinical lifelines and post-mortem findings of 29 patients with possible NPH. Pre-mortem cortical brain biopsies were taken from all patients during an intracranial pressure measurement or a cerebrospinal fluid (CSF) shunt surgery.
Results: The mean age at the time of the biopsy was 70±8 SD years and 74±7 SD years at the time of death. At the time of death, 11/29 patients (38%) displayed normal cognition or mild cognitive impairment (MCI), 9/29 (31%) moderate dementia and 9/29 (31%) severe dementia. Two of the demented patients had only scarce neuropathological findings indicating a probable hydrocephalic origin for the dementia. Amyloid-β (Aβ) and hyperphosphorylated τ (HPτ) in the biopsies predicted the neurodegenerative diseases so that there were 4 Aβ positive/low Alzheimer’s disease neuropathological change (ADNC) cases, 4 Aβ positive/intermediate ADNC cases, 1 Aβ positive case with both low ADNC and progressive supranuclear palsy (PSP), 1 HPτ/PSP and primary age-related tauopathy (PART) case, 1 Aβ/HPτ and low ADNC/synucleinopathy case and 1 case with Aβ/HPτ and high ADNC. The most common cause of death was due to cardiovascular diseases (10/29, 34%), followed by cerebrovascular diseases or subdural hematoma (SDH) (8/29, 28%). Three patients died of a postoperative intracerebral hematoma (ICH). Vascular lesions were common (19/29, 65%).
Conclusions: We update the suggested neuropathological diagnostic criteria of iNPH, which emphasize the rigorous exclusion of all other known possible neuropathological causes of dementia. Despite the first 2 probable cases reported here, the issue of “hydrocephalic dementia” as an independent entity still requires further confirmation. Extensive sampling (with fresh frozen tissue including meninges) with age-matched neurologically healthy controls is highly encouraged.

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Published

2022-01-26

How to Cite

Hänninen, J. J., Nakajima, M., Vanninen, A., Hytönen, S., Rummukainen, J., Koivisto, A. M., Jääskeläinen, J. E., Soininen, H., Sutela, A., Vanninen, R., Hiltunen, M., Leinonen, V., & Rauramaa, T. (2022). Neuropathological findings in possible normal pressure hydrocephalus: A post-mortem study of 29 cases with lifelines. Free Neuropathology, 3, 2. https://doi.org/10.17879/freeneuropathology-2022-3331
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Issue

Vol. 3 (2022)

Section

Original Papers

License

Copyright (c) 2022 Joni J. Hänninen, Madoka Nakajima, Aleksi Vanninen, Santtu Hytönen, Jaana Rummukainen, Anne Maria Koivisto, Juha E. Jääskeläinen, Hilkka Soininen, Anna Sutela, Ritva Vanninen, Mikko Hiltunen, Ville Leinonen, Tuomas Rauramaa

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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