University of Oulu

Korhonen et al. Fluids Barriers CNS (2017) 14:10, DOI 10.1186/s12987-017-0060-7

Frontotemporal dementia as a comorbidity to idiopathic normal pressure hydrocephalus (iNPH) : a short review of literature and an unusual case

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Author: Korhonen, V. E.1,2; Solje, E.3; Suhonen, N. M.4,5;
Organizations: 1Department of Neurosurgery, Kuopio University Hospital, P.O. Box 100, 70029 KYS Kuopio, Finland
2University of Eastern Finland, P.O. Box 100, 70029 KYS Kuopio, Finland
3Institute of Clinical Medicine-Neurology, University of Eastern Finland, P.O. Box 1627, 70211 Kuopio, Finland
4Medical Research Center, Oulu University Hospital, P.O. Box 20, 90029 Oulu, Finland
5Unit of Clinical Neuroscience, Neurology, University of Oulu, P.O. Box 5000, 90014 Oulu, Finland
6Institute of Clinical Medicine-Pathology, School of Medicine, University of Eastern, Kuopio, Finland
7Department of Pathology, Kuopio University Hospital, P.O. Box 162, 70211 Kuopio, Finland
8Department of Radiology, Kuopio University Hospital, P.O. Box 100, 70029 KYS Kuopio, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 1 MB)
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Language: English
Published: Springer Nature, 2017
Publish Date: 2017-06-27


Behavioural variant frontotemporal dementia (bvFTD) and idiopathic normal pressure hydrocephalus (iNPH) are neurodegenerative diseases that can present with similar symptoms. These include decline in executive functions, psychomotor slowness, and behavioural and personality changes. Ventricular enlargement is a key radiological finding in iNPH that may also be present in bvFTD caused by the C9ORF72 expansion mutation. Due to this, bvFTD has been hypothesized as a potential comorbidity to iNPH but bvFTD patients have never been identified in studies focusing in clinical comorbidities with iNPH. Here we describe a patient with the C9ORF72 expansion-associated bvFTD who also showed enlarged ventricles on brain imaging. The main clinical symptoms were severe gait disturbances and psychiatric problems with mild cognitive decline. Cerebrospinal fluid removal increased the patient’s walking speed, so a ventriculoperitoneal shunt was placed. After insertion of the shunt, there was a significant improvement in walking speed as well as mild improvement in cognitive function but not in neuropsychiatric symptoms relating to bvFTD. Comorbid iNPH should be considered in bvFTD patients who have enlarged ventricles and severely impaired gait.

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Series: Fluids and barriers of the CNS
ISSN: 2045-8118
ISSN-E: 2045-8118
ISSN-L: 2045-8118
Volume: 14
Article number: 10
DOI: 10.1186/s12987-017-0060-7
Type of Publication: A2 Review article in a scientific journal
Field of Science: 3124 Neurology and psychiatry
Funding: This study was funded by Kuopio University Hospital EVO-fund
Copyright information: © The Author(s) 2017. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.