Abstract

The diagnosis of idiopathic normal pressure hydrocephalus (iNPH) and the outcome of lumboperitoneal shunt treatment remains to be systematically explored. Here, we aim to evaluate whether the severity of dopaminergic degeneration and white matter small vessel disease could be predictors of outcome for iNPH patients subjected to lumboperitoneal shunt treatment. This is a single center retrospective study with 39 patients with probable iNPH undergoing programmable surgical lumboperitoneal shunt from June 2016 to March 2018 at Hualien Tzu Chi Hospital. In all patients, dopaminergic degeneration was determined with 99mTc- TRODAT-1 SPECT scan, while white matter small vessel disease (Fazekas scale) was assessed with Brain MRI. The iNPH grading scale (iNPHGS) score and Karnofsky Performance Score (KPS) pre- and post-operation (6-month follow-up) were available for all patients. Linear regression was used to correlate the severities of dopaminergic degeneration and small vessel disease with lumboperitoneal shunt treatment outcomes. Their iNPHGS score improved significantly after surgery (pre-operatively, 7.8 ± 2.6; post-operatively, 5.7 ± 2.6 (26.9% improvement) (p < 0.05)). Moreover, the KPS was also improved significantly after surgery, by a mean of 24.6% from the baseline score (p < 0.05). A significant correlation was observed between the severity of dopaminergic degeneration and a poorer improvement of iNPHGS score (p = 0.03). However, improvement of the iNPHGS score was not correlated with white matter small vessel disease. Dopaminergic degeneration comorbidity neutralized the degree of improvement after surgery. Although white matter small vessel disease was correlated with iNPH incidence, it may not be a prognostic factor for shunt operation. These findings have implications for the use of dopaminergic imaging, as they might help predict the surgical outcome of patients with iNPH, while vascular mechanisms seem to be involved in iNPH pathophysiology.

Highlights

  • Idiopathic normal pressure hydrocephalus is an increasingly recognized disease among the elderly population that is associated with the clinical triad of gait disturbance, rapid cognitive impairment, and urinary incontinence [1,2]. iNPH involves the abnormal accumulation of cerebrospinal fluid (CSF) in the ventricular system of the brain

  • In accordance with previous reports, we found that a history of hypertension and DM was much more common among patients with iNPH, and detected a strong association between the incidence of small vessel disease and iNPH, which suggested that a better control of vascular risk factors might lead to a lower incidence of iNPH, or a better outcome of CSF drainage intervention [11,21]

  • We demonstrated that dopaminergic neurodegeneration, as assessed using 99 mTc-TRODAT-1 SPECT, might decrease the benefit of symptomatic improvement observed after lumboperitoneal shunt surgery for iNPH

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Summary

Introduction

Idiopathic normal pressure hydrocephalus (iNPH) is an increasingly recognized disease among the elderly population that is associated with the clinical triad of gait disturbance, rapid cognitive impairment, and urinary incontinence [1,2]. iNPH involves the abnormal accumulation of cerebrospinal fluid (CSF) in the ventricular system of the brain. INPH involves the abnormal accumulation of cerebrospinal fluid (CSF) in the ventricular system of the brain. It remains a diagnosis without definitive and objective clinical signs, radiological findings, and pathological evidence, further research is warranted to identify better predictors of the outcomes of this disease [3]. Many of the clinical manifestations of small vessel disease are similar to the symptoms of iNPH, such as cognitive decline, gait disability, and extrapyramidal symptoms; this depends on the underlying pathophysiology [5]. The manner in which the presence of small vessel disease might affect the surgical outcomes in patients with probable iNPH has not been addressed

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