Frontotemporal dementia as a comorbidity to idiopathic normal pressure hydrocephalus iNPH: a short review of literature and an unusual caseReport as inadecuate

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Fluids and Barriers of the CNS

, 14:10

First Online: 19 April 2017Received: 07 February 2017Accepted: 05 April 2017DOI: 10.1186-s12987-017-0060-7

Cite this article as: Korhonen, V.E., Solje, E., Suhonen, N.M. et al. Fluids Barriers CNS 2017 14: 10. doi:10.1186-s12987-017-0060-7


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.

KeywordsC9ORF72 repeat expansion CSF shunt Dementia Idiopathic normal pressure hydrocephalus Neurodegeneration AbbreviationsADAlzheimer’s disease

ALSamyotrophic lateral sclerosis

bvFTDbehavioral variant frontotemporal dementia

CERAD-NBConsortium to Establish a Registry for Alzheimer’s disease Neuropsychological Battery

CHMP2Bchromatin-modifying protein 2B

CSFCerebrospinal fluid

FBIfrontal behavioral inventory

FTLDfrontotemporal lobar degeneration


iNPHidiopathic normal pressure hydrocephalus

MAPTmicrotubule-associated protein tau

p62sequestosome-1-ubiquitin-binding protein

TDP-43TAR DNA binding protein 43

Author: V. E. Korhonen - E. Solje - N. M. Suhonen - T. Rauramaa - R. Vanninen - A. M. Remes - V. Leinonen



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