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Idiopathic Normal Pressure Hydrocephalus: Focus on Imaging and Clinical Symptoms
Linköping University, Department of Biomedical and Clinical Sciences, The Division of Cell and Neurobiology. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Anaesthetics, Operations and Specialty Surgery Center, Neurologiska kliniken i Linköping.
2025 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Idiopathic normal pressure hydrocephalus (iNPH) is a disease that often appears in elderly population, causing gait and/or balance disturbance, cognitive decline and urinary incontinence. The prevalence of iNPH is around 3.7% and there are a lot of patients with the disease without receiving a shunt. The diagnosis of iNPH is based on International Guidelines which are updated twice since 2005. The disease has an insidious onset and progress gradually. The patients describe that the symptoms may were caused by age-related reason. The major difference of iNPH with other neurodegenerative diseases is that it is treatable with a shunt surgery and that could improve patients’ symptoms up to 84% raising the hypothesis of reversibility. Hence, iNPH is a big challenge for the neurologists. Additionally, the pathophysiological mechanism behind this disease is still unclear.

The overall aim of this thesis is to increase the knowledge of the clinical and radiological signs in patients with iNPH and identify predicting factors for better shunt surgery outcomes.

Paper I

To identify any disturbance of circadian rhythm in iNPH-patients. The aim was to study any changes of the diurnal rhythm (mesor and circadian period) as well as any changes of the diurnal amplitude and acrophase of the activity in iNPH-patients before and after a shunt operation.  

Paper II

To identify, with the use of DTI, significant changes in hydrocephalus patients compared to controls. To correlate the radiological DTI results with the clinical features (cognition and gait) before and after a shunt surgery. To investigate early white matters changes in patients with iNPH and possibly reversibility after a shunt surgery. To establish a new technique in investigation of patients with iNPH and early identify this group. A significant correlation between ROIs results in one or more brain areas with the iNPH-results will give us the opportunity to use a specific DTI protocol to easier identify iNPH-patients who will have positive results with a shunt operation.

Paper III

To identify any retinal changes (retinal nerve fiber layer [RNFL] thickness, ganglion cell layer [GCL] thickness, optic disc area, rim area, and optic volume) in patients with iNPH compared with healthy individuals. Additionally, to identify any changes in the clinical symptoms before and after a CSF tap test corresponding to the OCT findings.  

Paper IV

To identify possible urinary and bowel symptoms in patients with iNPH and compare them to healthy individuals. The secondary aim is to evaluate possible improvement after the shunt operation.  

Place, publisher, year, edition, pages
Linköping: Linköping University Electronic Press, 2025. , p. 105
Series
Linköping University Medical Dissertations, ISSN 0345-0082 ; 1980
Keywords [en]
INPH, DTI, OCT, Urinary incontinence, Bowel incontinence, Circadian rhythms
National Category
Neurology
Identifiers
URN: urn:nbn:se:liu:diva-213479DOI: 10.3384/9789181180916ISBN: 9789181180909 (print)ISBN: 9789181180916 (electronic)OAI: oai:DiVA.org:liu-213479DiVA, id: diva2:1956175
Public defence
2025-06-04, Birgittasalen, building 240, Campus US, Linköping, 09:00 (Swedish)
Opponent
Supervisors
Available from: 2025-05-05 Created: 2025-05-05 Last updated: 2025-05-05Bibliographically approved
List of papers
1. Circadian rhythm in idiopathic normal pressure hydrocephalus
Open this publication in new window or tab >>Circadian rhythm in idiopathic normal pressure hydrocephalus
2018 (English)In: Clinical neurology and neurosurgery (Dutch-Flemish ed. Print), ISSN 0303-8467, E-ISSN 1872-6968, Vol. 164, p. 72-74Article in journal (Refereed) Published
Abstract [en]

Objectives: The pathogenesis of idiopathic normal pressure hydrocephalus (iNPH) takes place in structures close to the cerebral ventricular system. Suprachiasmatic nucleus (SCN), situated close to the third ventricle, is involved in circadian rhythm. Diurnal disturbances are well-known in demented patients. The cognitive decline in iNPH is potentially reversible after a shunt operation. Diurnal rhythm has never been studied in iNPH. We hypothesize that there is a disturbance of circadian rhythm in iNPH-patients and the aim was to study any changes of the diurnal rhythm (mesor and circadian period) as well as any changes of the diurnal amplitude and acrophase of the activity in iNPH-patients before and after a shunt operation. Patients and methods: Twenty consecutive iNPH-patients fulfilling the criteria of the American iNPH-guidelines, 9 males and 11 females, mean age 73 (49-81) years were included. The patients underwent a pre-operative clinical work-up including 10 m walk time (w10mt) steps (w10 ms), TUG-time (TUGt) and steps (TUGs) and for cognitive function an MMSE score was measured. In order to receive circadian rhythm data actigraphic recordings were performed using the SenseWear 2 (BodyMedia Inc Pittsburgh, PA, USA) actigraph. Cosinor analyses of accelerometry data were performed in "R" using non-linear regression with Levenburg-Marquardt estimation. Pre- and post-operative data regarding mesor, amplitude and circadian period were compared using Wilcoxon-Mann-Whitney test for paired data. Results: Twenty patients were evaluated before and three month post-operatively. Motor function (w10mt, w10 ms, TUGt, TUGs) was significantly improved while MMSE was not significantly changed. Actigraphic measurements (mesor, amplitude and circadian period) showed no significant changes after shunt operation. Conclusion: This is the first systematic study of circadian rhythm in iNPH-patients. We found no significant changes in circadian rhythm after shunt surgery. The conceptual idea of diurnal rhythm changes in hydrocephalus is still interesting from a theoretical standpoint and warrants further studies that could include a combination of better designed actigraphic studies in combination with neuroendocrine markers and imaging methods

Place, publisher, year, edition, pages
ELSEVIER SCIENCE BV, 2018
Keywords
Idiopathic normal pressure hydrocephalus; Circadian rhythm; Suprachiasmatic nucleus
National Category
Neurology
Identifiers
urn:nbn:se:liu:diva-145480 (URN)10.1016/j.clineuro.2017.11.018 (DOI)000424727200016 ()29202376 (PubMedID)
Available from: 2018-03-05 Created: 2018-03-05 Last updated: 2025-05-05
2. Publisher correction: Indication of Thalamo-Cortical Circuit Dysfunction in Idiopathic Normal Pressure Hydrocephalus: A Tensor Imaging Study
Open this publication in new window or tab >>Publisher correction: Indication of Thalamo-Cortical Circuit Dysfunction in Idiopathic Normal Pressure Hydrocephalus: A Tensor Imaging Study
Show others...
2020 (English)In: Scientific Reports, E-ISSN 2045-2322, Vol. 10, no 1, article id 12014Article in journal (Other academic) Published
Place, publisher, year, edition, pages
Nature Publishing Group, 2020
National Category
Neurology
Identifiers
urn:nbn:se:liu:diva-174338 (URN)10.1038/s41598-020-69149-x (DOI)000550057500002 ()32678296 (PubMedID)2-s2.0-85087937747 (Scopus ID)
Note

Correction to: Scientific Reports https://doi.org/10.1038/s41598-020-63238-7

Available from: 2021-03-20 Created: 2021-03-20 Last updated: 2025-05-05Bibliographically approved
3. Optical Coherence Tomography Revealing Ganglion Cell Loss in Idiopathic Normal Pressure Hydrocephalus
Open this publication in new window or tab >>Optical Coherence Tomography Revealing Ganglion Cell Loss in Idiopathic Normal Pressure Hydrocephalus
2021 (English)In: World Neurosurgery, ISSN 1878-8750, E-ISSN 1878-8769, Vol. 149, p. E1061-E1066Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Although there may theoretically be a disturbance in the eye or the visual pathways due to abnormal cerebrospinal fluid (CSF) dynamics in idiopathic normal pressure hydrocephalus (iNPH), it has not been studied systemically. Optical coherence tomography (OCT) is a noninvasive, reproducible procedure for quantitative and qualitative analysis of retinal morphology. METHODS: OCT was used to study the eye fundus before and after a CSF tap test in patients with iNPH compared with healthy individuals (HIs). Twelve patients with iNPH (6 females and 6 males) with a median age of 76 years (64-84 years) and 21 HIs (11 females and 10 males) with a median age of 73 years (64-79 years) were included. The patients underwent neurological, cognitive, and physiotherapeutic evaluation. Brain magnetic resonance imaging, CSF tap test via lumbar puncture, and subsequently CSF analysis were performed. OCT was performed before and after CSF removal. HIs underwent OCT once. RESULTS: The patients had significantly reduced retinal ganglion cell layer thickness 71 mu m (56-81 mu m) compared with the HIs, 79.5 mu m (72-90 mu m) (P = 0.001), but no sig -ificant changes were observed before or after the CSF tap test. All patients improved in motor function in a 10-m walk test after the CSF tap test. The median CSF pressure was 15 and 1 cm H2O, respectively, before and after lumbar puncture with removal of median 43.5 mL CSF. CONCLUSIONS: This pilot study shows OCT findings that differ from HIs and implies a rational for becoming a valuable tool in the diagnosis of iNPH. Further studies are warranted to elucidate the pathology of the retina in iNPH.

Place, publisher, year, edition, pages
Elsevier Science, 2021
Keywords
CSF tap test; Degeneration; Ganglion cell layer; Idiopathic normal pressure hydrocephalus; Optical coherence tomography
National Category
Neurology
Identifiers
urn:nbn:se:liu:diva-175983 (URN)10.1016/j.wneu.2021.01.003 (DOI)000645617600003 ()33444824 (PubMedID)
Available from: 2021-06-01 Created: 2021-06-01 Last updated: 2025-05-05

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