Summary
Background
Cerebrospinal fluid (CSF) shunting is a highly effective treatment for idiopathic normal pressure hydrocephalus (iNPH). However, secondary deterioration can occur at a later time. Thus, the current study aimed to evaluate the incidence rate and causes of secondary deterioration.
Methods
A retrospective analysis was conducted on all patients with iNPH who were treated with implantation of a CSF shunt since 1993. A meticulous shunt workup was recommended to all patients who presented to our department with secondary deterioration during their follow-up visits. Data about the proportion of patients with such deterioration and its causes, subsequent treatment, and clinical outcome were obtained.
Results
A total of 169 patients were included, and the mean follow-up time was 69.2 months. In total, 119 (70.4%) patients presented with a total of 153 secondary deteriorations. In 9 cases (5.9%), the deterioration was caused by delayed subdural hematoma and in 27 (22.1%) cases, by shunt dysfunction. Invasive shunt testing was commonly required to validate shunt failure. Moreover, 19 of 27 patients experienced a satisfactory improvement after revision surgery. In total, 86 deteriorations were attributed to nonsurgical causes, and the valve pressure was decreased in 79 patients, with only 16.5% presenting with a satisfactory improvement after lowering of valve pressure.
Conclusions
Most patients with shunted iNPH presented with deterioration in the later course of the disease. Shunt dysfunction was considered a cause of secondary deterioration. Moreover, shunt revision surgery was a highly effective treatment, and patients with deterioration should undergo screening procedures for shunt dysfunction, including invasive shunt testing.
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All authors contributed to the study concept and design. Material preparation, data collection, and analysis were performed by Stefanie Kaestner, Rhea Behrend, and Christian Roth. The first draft of the manuscript was written by Stefanie Kaestner. Review and editing were performed by Katharina Graf and Wolfgang Deinsberger. All authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (name of institute/committee) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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The authors present a retrospective study of secondary deterioration in a large cohort of patients with idiopathic normal pressure hydrocephalus.169 patients are followed up for a mean of 69.2 months. Patients were selected for shunting by a tap test. Secondary deterioration occurred in 70%, at a mean of 37.8 months. At the time of secondary deterioration, patients underwent a CT scan and shunt radiography, and, if normal, had the opening pressure of their valve reduced. If there was no improvement, as was often the case, shunt function was investigated with injection of contrast medium, fluoroscopy and test aspiration of 30 mLs of CSF. 27 from 153 episodes of secondary deterioration were related to shunt malfunction; clinical improvement was evident after shunt revision in 19 episodes. The authors conclude that on long-term follow-up secondary deterioration is very common. More importantly, as about a fourth of these episodes were associated with shunt malfunction, investigation of the shunt, rather than assuming a nonsurgical cause, often results in improvement with shunt revision.This is an important study, as secondary deterioration in NPH is often attributed to progressive vascular or neurodegenerative causes. The study underlines the value of formal shunt investigation within a large cohort followed up for a reasonably long time.
Kristian Aquilina,
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Kaestner, S., Behrends, R., Roth, C. et al. Treatment for secondary deterioration in idiopathic normal pressure hydrocephalus in the later course of the disease: a retrospective analysis. Acta Neurochir 162, 2431–2439 (2020). https://doi.org/10.1007/s00701-020-04475-7
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DOI: https://doi.org/10.1007/s00701-020-04475-7