[Symptomatology of Idiopathic Normal Pressure Hydrocephalus].

Idiopathic normal pressure hydrocephalus causes a triad of gait disturbance, dementia, and urinary incontinence in the elderly. All these symptoms may manifest as age-related functional decline or from neurological and non-neurological diseases common in the elderly. In idiopathic normal pressure hydrocephalus, characterized by ataxic-ataxic gait, subcortical dementia, and urge urinary incontinence, it is clinically important to consider these characteristic features. This overview describes the symptomatology of idiopathic normal pressure hydrocephalus.

Lumboperitoneal Shunt Surgery under Spinal Anesthesia on Idiopathic Normal Pressure Hydrocephalus Patients.

Since the publication of guidelines for managing idiopathic normal pressure hydrocephalus (iNPH) in 2004, an increasing number of patients with iNPH have been undergoing shunt surgery in Japan. However, shunt surgeries for iNPH can be challenging because the procedures are performed on elderly patients. General anesthesia-related risks, such as postoperative pneumonia or delirium, are higher in the elderly. To decrease these risks, we applied spinal anesthesia on a lumboperitoneal shunt (LPS). Herein, we analyzed our methods focusing on the postoperative outcomes. We retrospectively analyzed 79 patients who underwent LPS at our institution with more than one year of follow-up. The patients were divided into two groups based on the anesthetic approach, that is, 1) general anesthesia and 2) spinal anesthesia, and were examined in terms of postoperative complications, delirium, and postoperative hospital stay. In the general anesthesia group, two patients had respiratory complications after the surgery. The postoperative delirium score using the intensive care delirium screening checklist (ICDSC) was 0 (2) (median [interquartile range]), and the length of postoperative hospital stay was 11 (4) days. In the spinal anesthesia group, no patients had respiratory complications. The postoperative mean ICDSC was 0 (1), and the length of postoperative hospital stay was 10 (3) days. Although there was no significant difference regarding postoperative delirium existed, LPS under spinal anesthesia decreased respiratory complications and significantly shortened the postoperative hospital stay. LPS under spinal anesthesia could be an alternative to general anesthesia in elderly patients with iNPH and possibly lessen the general anesthesia-related risks.

The impact of cerebrospinal fluid shunting on quality of life in idiopathic normal pressure hydrocephalus: a long-term analysis.

OBJECTIVE: Idiopathic normal pressure hydrocephalus (iNPH) represents an insidious type of dementia considered reversible after shunt placement. Although the clinical outcome has been widely studied, few studies have reported on quality of life (QOL) after surgery. This study evaluated the long-term clinical and QOL outcomes of iNPH patients after ventriculoperitoneal shunt (VPS) implantation. Factors influencing QOL in iNPH were also investigated. METHODS: From 2009 to 2020, a single-institution retrospective study was conducted to compare shunted iNPH patients with a homogeneous control group. QOL was analyzed using the SF-36 questionnaire with yearly follow-up for as long as 11 years. Severity of symptoms, comorbidities, and clinical data were also recorded. RESULTS: Among 187 treated patients, 15 had died at the time of the authors' evaluation, and 45 did not match the inclusion criteria. The mean ± SD (range) follow-up was 118.5 ± 4.2 (18-132) months. QOL improved in 103/130 (79%) patients through 5 years after shunt surgery, although it remained lower than that of the control group (p < 0.0001). The SF-36 score reduced progressively, reaching baseline at 5-7 years of follow-up and decreased to below baseline at 7-11 years of follow-up (p < 0.0001). Predictors of improved QOL were younger age (p < 0.001), lower body mass index (BMI) (p < 0.001), and better Mini-Mental State Examination (MMSE) performance (p < 0.001) before surgery. Decreased postoperative QOL was associated with cerebrovascular disease, diabetes, and severity of symptoms (gait and cognition) at presentation (p < 0.001). CONCLUSIONS: VPS implantation, along with a strict and comprehensive follow-up, has been shown to improve QOL in iNPH patients for as long as 5 years after surgery. Younger age, lower BMI, and better MMSE score are positive predictors of improved QOL after shunt placement.

A case of primary central nervous system lymphoma presenting as a shunt complication.

The authors describe an 82-year-old female with a right frontal ventriculoperitoneal (VP) shunt for long-standing normal pressure hydrocephalus (NPH) who presented with worsening incontinence and gait instability. She was found to have right lateral ventricle collapse around the shunt catheter and subsequently underwent shunt revision, which failed to improve her symptoms. Magnetic resonance imaging (MRI) was obtained on postoperative day two, which demonstrated a ventricular lesion. Endoscopic brain biopsy was performed and a diagnosis of primary central nervous system lymphoma (PCNSL) was made. The authors believe this is the first published case of PCNSL presenting as a VP shunt complication in a patient with NPH.

Hospital Frailty Risk Score Predicts Adverse Events and Readmission Following a Ventriculoperitoneal Shunt Surgery for Normal Pressure Hydrocephalus.

OBJECTIVE: The aim of this study was to evaluate the impact of a Hospital Frailty Risk Score (HFRS) on unplanned readmission and health care resource utilization in normal pressure hydrocephalus (NPH) patients undergoing a ventriculoperitoneal (VP) shunt surgery. METHODS: A retrospective cohort study was performed using the 2016-2019 Nationwide Readmission Database. All NPH patients (≥60 years) undergoing a VP shunt surgery were identified using ICD-10-CM diagnostic and procedural codes. Patients were dichotomized into 2 cohorts as follows: Low HFRS (<5) and Intermediate-High HFRS (≥5). A multivariate logistic regression analysis was then used to identify independent predictors of adverse event (AE) and 30- and 90-day readmission. RESULTS: Of 13,262 patients, 4386 (33.1%) had an Intermediate-High HFRS score. A greater proportion of the Intermediate-High HFRS cohort experienced at least one AE (1.9 vs. 22.1, P < 0.001). The Intermediate-High HFRS cohort also had a longer length of stay (2.3 ± 2.4 days vs. 7.0 ± 7.7 days, P < 0.001), higher non-routine discharge rate (19.9% vs. 39.9%, P < 0.001), and greater admission cost ($14,634 ± 5703 vs. $21,749 ± 15,234, P < 0.001). The Intermediate-High HFRS cohort had higher rates of 30- (7.6% vs. 11.0%, P < 0.001) and 90-day (6.8% vs. 8.3%, P < 0.001) readmissions. On a multivariate regression analysis, Intermediate-High HFRS compared to Low HFRS was an independent predictor of any AE (odds ratio, 16.6; 95% confidence interval, [12.9-21.5]; P < 0.001) and 30-day readmission (odds ratio, 1.4; 95% confidence interval, [1.2-1.7]; P < 0.001). CONCLUSIONS: Our study suggests that frailty, as defined by HFRS, is associated with increased resource utilization in NPH patients undergoing VP shunt surgery. Furthermore, HFRS was an independent predictor of adverse events and 30-day hospital readmission.

Impact of Subjective Evaluations in Predicting Response to Ventriculoperitoneal Shunt for Idiopathic Normal Pressure Hydrocephalus.

BACKGROUND: Cerebrospinal fluid tap test is a common procedure to predict the efficacy of ventriculoperitoneal shunt for idiopathic normal pressure hydrocephalus. Objective tests after cerebrospinal fluid tap test are used to establish the surgical indication, but subjective improvements may also be important in selection of surgical candidates. The aim of this study was to evaluate surgical outcomes of patients with ventriculoperitoneal shunt for idiopathic normal pressure hydrocephalus, comparing patients showing objective improvement with patients improving only on subjective assessments. METHODS: In this retrospective analysis, patients were divided into 2 groups: group 1 included patients with improvement on objective evaluation after cerebrospinal fluid tap test; group 2 included patients who showed only subjective improvement. The surgical outcomes of the 2 groups were compared. RESULTS: Of 28 included patients, 17 were objective responders (group 1), and 11 were subjective responders (group 2). Clinical and radiological characteristics were similar. The only significant difference was the baseline Berg Balance Scale, which was lower in objective responders (P = 0.0015). At 3 months after surgery and at last follow-up, there was no difference in surgical outcomes between the 2 groups. However, in the group of subjective responders, a continuous improvement for incontinence and gait was more frequently observed (P = 0.04 and P < 0.001, respectively). CONCLUSIONS: Surgical outcomes after ventriculoperitoneal shunt were similar between the 2 groups, with a more favorable trend in terms of symptom improvement for subjective responders. Subjective assessment seems to be an important factor to consider in preoperative evaluation.

Increased interstitial fluid in periventricular and deep white matter hyperintensities in patients with suspected idiopathic normal pressure hydrocephalus.

Periventricular white matter changes are common in patients with idiopathic normal pressure hydrocephalus (iNPH) and considered to represent focally elevated interstitial fluid. We compared diffusion measures in periventricular hyperintensities in patients with imaging features of iNPH to patients without. The hypothesis is that periventricular hyperintensities in patients with presumed iNPH show higher water content than in patients without imaging features of iNPH. 21 patients with iNPH Radscale 7-12 ("high probability of iNPH") and 10 patients with iNPH Radscale 2-4 ("low probability of iNPH") were examined with a neurodegeneration imaging protocol including a diffusion microstructure imaging sequence. Periventricular hyperintensities and deep white matter hyperintensities were segmented and diffusion measures were compared. In patients with imaging features of iNPH, the free water content in periventricular hyperintensities was significantly higher compared to the control group (p = 0.005). This effect was also detectable in deep white matter hyperintensities (p = 0.024). Total brain volumes and total gray or white matter volumes did not differ between the groups. Periventricular cap free water fraction was highly discriminative regarding patients with presumed iNPH and controls with an ROC AUC of 0.933. Quantitative diffusion microstructure imaging shows elevated water content in periventricular hyperintensities in patients with imaging features of iNPH, which could be the imaging correlate for pathologic fluid accumulation and may be used as an imaging biomarker in the future.

Clinical and radiological features of main dementias / Aspectos clínicos e radiológicos das principais demências

Dementia is a syndrome characterized by a decline of two or more cognitive functions, affecting social or professional life. Alzheimer's Disease is a neurodegenerative disorder that represents 53% of dementia cases; memory loss, inability to recognize faces, impaired judgement, disorientation and confusion are possible common symptoms. Vascular Dementia is responsible for 42% of dementia cases, due to cerebrovascular pathologies, and the clinical aspects are related to the extension and location of the brain injury. Lewy Bodies Dementia is a neurodegenerative disorder that represents 15% of dementia cases, and its symptoms include visual hallucinations, parkinsonism and fluctuating cognitive decline. Frontotemporal dementia is a group of clinical syndromes, divided in Behavioral-variant, characterized by disinhibition, compulsions, apathy, aberrant sexual behavior and executive dysfunction; and Primary Progressive Aphasia, which is subdivided in Nonfluentvariant and Semantic-variant. Vitamin B12 deficiency is a reversible cause of dementia, with a wide clinical feature, that includes psychiatric symptoms such as depression and irritability, hematological symptoms related to anemia (e.g. dyspnea and fatigue), and neurological symptoms including dementia and neuropathy. Normal pressure hydrocephalus is also reversible, presenting forgetfulness, changes in mood, decline of executive functions, reduced attention, and a lack of interest in daily activities as symptoms. The radiological findings vary depending on the etiology of dementia. For that reason, understanding neuroimaging and clinical aspects is important to diagnose effectively.

A demência é uma síndrome que consiste em um declínio de um ou mais domínios cognitivos, que afeta o desempenho social ou profissional do indivíduo. A Doença de Alzheimer é um transtorno neurocognitivo que representa 53% dos casos de demência; seus sintomas podem incluir perda de memória, incapacidade de reconhecer rostos familiares, julgamento comprometido desorientação e confusão mental. A Demência Vascular é responsável por 42% dos casos de demência e é causada por doenças cerebrovasculares, seus achados clínicos são relacionados com o local e com a extensão do dano cerebral. Já a Demência por Corpos de Lewy é uma doença neurocognitiva que representa 15% dos casos de demência, cujos sintomas incluem alucinações visuais, parkinsonismo e flutuação cognitiva. A Demência Frontotemporal, por sua vez, é um grupo de síndromes, que se dividem em variante comportamental ­ caracterizada por desinibição, compulsão, apatia, hipersexualidade e disfunções executivas ­ e Afasia Progressiva Primária, subdividida em variante não-fluente e variante semântica, que cursam com disfunções da linguagem. Há, ainda, a Deficiência de Vitamina B12, uma causa reversível de demência. Ela possui um quadro clínico variado, que inclui sintomas psiquiátricos, como depressão e irritabilidade, sintomas hematológicos relacionados a anemia, como dispneia e fadiga) e sintomas neurológicos, que incluem demência e neuropatias. Uma outra causa reversível é a Hidrocefalia de Pressão Normal, que se apresenta com esquecimentos, alterações de humor, perda de função executiva e redução da atenção e do interesse nas atividades cotidianas. Os achados de neuroimagem variam dependendo da etiologia da demência. Assim, compreender os aspectos clínicos e radiológicos é importante para um diagnóstico efetivo..

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment.

BACKGROUND: After ventriculoperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (iNPH) with adjustable gravitational valves, a certain proportion of patients develop secondary clinical worsening after initial improvement of clinical symptoms. The aim of this study was to analyze this group of patients with secondary deterioration and to evaluate the performed shunt management. METHODS: For this investigation, we retrospectively reviewed our NPH registry for patients included between 1999 and 2013 with a decrease by a minimum of two points in the Kiefer score in the first year of follow up and an increase of two points in the Kiefer score between the second and the fifth year after shunt surgery (secondary deterioration). Then, we analyzed the patient's shunt management (adapting the valve pressure setting, shuntography, valve replacement, catheter replacement, implant an adjustable gravitational unit). Additionally, we searched for risk factors for secondary deterioration. RESULTS: Out of 259 iNPH patients, 53 (20%) patients showed secondary deterioration on an average of 2.7 (2-4 years) years after shunt surgery. Fourteen (26%) patients with secondary deterioration improved after shunt or valve management and 58% remained without clinical benefit after management. We had a drop-out rate of 15% due to incomplete datasets. Our shunt management reduced the rate of secondary deterioration from 20 to 15%. On the basis of our findings, we developed an algorithm for shunt management. Risk factors for secondary deterioration are the age of the patient at the time of shunting, newly diagnosed neurodegenerative diseases, and overdrainage requiring adjusting the valve to higher-pressure levels. CONCLUSION: Twenty percent of patients with iNPH were at risk for secondary clinical worsening about 3 years after shunt surgery. About one-fourth of these patients benefited for additional years from pressure level management and/or shunt valve revision. Our findings underline the need for long-term follow-ups and intensive shunt management to achieve a favorable long-term outcome for patients with iNPH and VPS.

Calcifying Pseudoneoplasm of Neuraxis (CAPNON) in the Posterior Third Ventricle-Challenge for Neuroendoscopy.

BACKGROUND: We report the first case of a purely intraventricular calcifying pseudoneoplasm of neuraxis (CAPNON) in the posterior third ventricle. CASE DESCRIPTION: A 63-year-old male without any previous medical history presented with Hakim triad. Imaging showed a calcified lesion of the posterior third ventricle with hydrocephalus. An endoscopic third ventriculostomy was performed. Endoscopic removal or debulking of the lesion was impossible due to its rock-hard consistency, and thus the procedure was aborted after biopsy. CONCLUSIONS: When encountering such calcified lesions within the ventricular system, especially in proximity to eloquent regions, the decision making process should include the hard consistency and parenchymal adhesions as obstacles to neuroendoscopic removal. Even for biopsy, a higher morbidity rate compared with typical soft tumors should be assumed. Although data on intraventricular CAPNON is limited, biopsy of the lesion and treatment of associated hydrocephalus appear to be the primary neurosurgical goals, followed by imaging surveillance.

Spinal paraganglioma presenting as normal pressure hydrocephalus: a rare occurrence.

Objective: We report a 63 years old female who presented with confusion, urinary retention and gait disturbances.Method: CT head shows communicating hydrocephalus. Spinal MRI demonstrated an L5- S1 intradural lesion.Result: Histopathology proved it to be paraganglioma. Postoperative CT head showed reduction in size of ventricles. Association between spinal tumors and hydrocephalus is known but occurs infrequently.Conclusion: The presence of an intraspinal tumor must be kept in mind as a possible cause of the hydrocephalus.

Quantitative Gait Analysis and Cerebrospinal Fluid Tap Test for Idiopathic Normal-pressure Hydrocephalus.

We investigated gait performance utilizing a quantitative gait analysis for 2 groups: (1) idiopathic normal-pressure hydrocephalus (INPH) patients who had a positive response to the cerebrospinal fluid tap test (CSFTT) and (2) healthy controls. The aims of the study were (1) to analyze the characteristics of gait features, (2) to characterize changes in gait parameters before and after the CSFTT, and (3) to determine whether there was any relationship between stride time and stride length variability and Frontal Assessment Battery (FAB) scores in INPH patients. Twenty-three INPH patients and 17 healthy controls were included in this study. Compared with healthy controls, the gait of INPH patients was characterized by lower velocity, shorter stride length, and more broad-based gait. Patients with INPH had a longer stance phase with increased double-limb support. Variability in stride time and stride length was increased in INPH patients. Stride time and stride length variability were correlated with FAB score. After the CSFTT, gait velocity, stride length, and step width significantly improved. There were significant decreases in stride time and stride length variability. These results suggest that the CSFTT for INPH patients might improve the so-called balance-related gait parameter (ie, step width) as well. Stride time and stride length variability also responded to the CSFTT. Association between FAB scores and both stride time and stride length variability suggests involvement of similar circuits producing gait variability and frontal lobe functions in INPH patients.

The Tight Medial and High Convexity Subarachnoid Spaces Is the First Finding of Idiopathic Normal Pressure Hydrocephalus at the Preclinical Stage.

Disproportionately enlarged subarachnoid-space hydrocephalus (DESH) findings are often reported as characteristic radiological features of idiopathic normal pressure hydrocephalus (iNPH). However, the process of development of DESH remains unclear. The aim of the present study was to determine the dynamic deforming process and pathophysiology of iNPH. All patients >50 years of age who underwent whole body FDG-PET/CT scanning at Kindai University Hospital between May 2017 and April 2018 were included in this retrospective study, and their brain image findings and clinical information were assessed. We defined DESH-like findings, which had one or two equivocal features of the three components of DESH findings, as preclinical morphologic features of DESH (PMD). PMD were classified into six subtypes based on their component of DESH findings: PMD-T, only tight medial and high convexity subarachnoid spaces (TMC); PMD-S, only enlarged Sylvian fissures; PMD-V, only ventriculomegaly; PMD-TV, TMC and ventriculomegaly; PMD-TS, TMC and enlarged Sylvian fissures; PMD-SV, enlarged Sylvian fissures and ventriculomegaly. A total of 2196 cases (70.5 ± 9.3 years) were enrolled, with 54 cases (77.1 ± 5.9 years) with DESH findings, and 42 cases (72.9 ± 7.9 years) with PMD (five PMD-T, two PMD-V, 12 PMD-TV, 18 PMD-TS, and five PMD-SV). In each component of DESH, 35 of 42 (83.3%) cases with PMD had TMC. We suggest that the TMC is the first change on DESH findings in most iNPH cases, and may be an important part of the pathophysiology of iNPH.

Normal Pressure Hydrocephalus.

PURPOSE OF REVIEW: Since it was first described in 1965, normal pressure hydrocephalus (NPH) has been a controversial subject. New studies have shed light on its epidemiology and pathogenesis and provided objective ways to measure outcome in patients with NPH. Neuroimaging has improved and allows better recognition of both NPH and the presence of overlapping diseases RECENT FINDINGS: Several recent epidemiologic studies confirm that NPH is a rare disease, but the presence of large ventricles is a common finding with aging. NPH may be multifactorial, including congenital causes, vascular disease, and impaired CSF absorption. MRI features of NPH include enlarged ventricular size and CSF fluid collection outside the ventricles not due to atrophy. The term disproportionately enlarged subarachnoid space hydrocephalus (DESH) has been used to describe prognostic MRI features in NPH, including a "tight high convexity" and enlargement of CSF spaces in the sylvian fissure. DESH has been included in the Japanese guideline for the diagnosis and treatment of NPH. A new NPH scale has been published that provides an objective framework for evaluating patients with NPH before and after shunt placement. Programmable shunts can noninvasively manage overdrainage complications. Surgical outcome has been improving over time. Recent studies have led to improved recognition of overlapping diseases such as Alzheimer pathology, which co-occurs in about 30% of NPH cases. Fludeoxyglucose positron emission tomography (FDG-PET) is a promising imaging modality for diagnosing NPH and detecting concomitant degenerative disease. SUMMARY: A systematic approach to patients with possible NPH allows recognition of the subset of patients who will respond to shunt surgery and identification of those with alternative diagnoses.

Pathogenesis of idiopathic Normal Pressure Hydrocephalus: A review of knowledge.

Idiopathic Normal Pressure Hydrocephalus (iNPH) is a frequent neuropsychiatric entity. Clinically it is characterised by Hakim's triad: Dementia, gait disturbance and urinary incontinence. While its symptomatology is typical, the etiology and thereby physiopathology of iNPH still remain enigmatic. This review summarizes and synthesizes different etiologic conceptions and physiopathologic aspects of iNPH. A research of literature via the PubMed/MEDLINE and the Cochrane database was conducted. Only English language articles clearly outlining a reasonable concept of physiopathology were included. Most authors advocate that iNPH is a result of chronically altered cerebrospinal fluid (CSF) dynamics, i.e. deranged CSF production, kinetics and reabsorption. In addition, there are vascular, metabolo-neurodegenerative and hereditary factors. Neuroinflammation does not seem to play a significant role in the etiology of iNPH. All in all, iNPH seems to combine several pathogenetic factors leading to a self-reinforcing vicious circle. The majority of studies hint at CSF disturbances on grounds of altered hemodynamics.

Hemangioblastomatosis-associated negative-pressure hydrocephalus managed with improvised shunt.

Low-pressure hydrocephalus (LPH) is a rare clinical diagnosis, characterized by neurologic decline and ventriculomegaly that persists despite normal to low intracranial pressure. LPH is typically managed by negative-pressure drainage via ventriculostomy, followed by low-resistance shunt insertion. We present the case of a middle-aged man with a history of hemangioblastomatosis who had spontaneous subarachnoid hemorrhage. He was treated with a ventriculoperitoneal shunt and then underwent resection of a Meckel's cave hemangioblastoma and whole brain irradiation. One month later, he presented to us with worsening symptoms and hydrocephalus despite shunt interrogations and revisions revealing no malfunction. Ventriculostomy drainage at negative-pressure was required for resolution of symptoms and ventriculomegaly, leading us to a diagnosis of LPH. This was successfully treated using an improvised ultra-low pressure valveless ventriculoperitoneal shunt, with maintained resolution of LPH for over one year. The system was created by ligating the distal slit valve end of a peritoneal catheter to prevent reflux and allow sub-zero pressure drainage by siphoning.

Clinical Experience with Cerebrospinal Fluid Aß42, Total and Phosphorylated Tau in the Evaluation of 1,016 Individuals for Suspected Dementia.

BACKGROUND: Elevated total tau (tTau), 181-phosphorylated phosphorylated tau (pTau), and low amyloid-ß42 (Aß42) in cerebrospinal fluid (CSF) represent a diagnostic biomarker for Alzheimer's disease (AD). OBJECTIVE: The goal was to determine the overall accuracy of CSF Aß42, tTau, pTau, and the Aß42/total tau index (ATI) in a non-research, clinical setting for the diagnosis of AD. METHODS: From medical records in 1,016 patients that had CSF studies for dementia over a 12-year period (2005 to 2017), we calculated the sensitivity and specificity of CSF Aß42, tTau, and pTau and the ATI in relation to the final clinical diagnosis. RESULTS: Compared with non-demented patients and patients with other dementias or mild cognitive impairment (MCI), the sensitivity and specificity of the recommended ATI and pTau cut-offs (ATI < 1.0 and pTau >61 pg/ml) for the diagnosis of AD were 0.88 and 0.72, respectively. Similar results were obtained comparing AD with non-demented patients only (0.88, 0.82) and AD with other types of dementia (0.81, 0.77). A subgroup of patients with presumed normal pressure hydrocephalus (n = 154) were biopsied at the time of shunt placement. Using the pathological manifestations of AD as the standard, the sensitivity was 0.83 while the specificity was 0.72. CONCLUSIONS: In a non-research setting, CSF biomarkers for AD showed a high sensitivity in accordance with previous studies, but modest specificity differentiating AD from other types of dementia or MCI. This study of unselected patients provides a valid and realistic assessment of the diagnostic accuracy of these CSF biomarkers in clinical practice.