Idiopathic Aqueductal Stenosis: Late Neurocognitive Outcome in ETV Operated Adult Patients

ObjectiveThe aim of the present study is to evaluate a neurocognitive outcome in patients affected by late-onset idiopathic aqueductal stenosis (LIAS) who underwent endoscopic third ventriculostomy (ETV). Materials and MethodsA prospective study was conducted between January 2015 and December 2017 in a series of 10 consecutive adult patients referred to the Neurosurgery Department of IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy. All the adult patients admitted with absence of CSF flow through the aqueduct in phase-contrast (PC)-MRI sequences or a turbulence void signal in T2-weighted images in midsagittal thin-slice MR sequences underwent a specific neuroradiological, neurological, and neurocognitive assessment pre- and postoperatively. ResultsAll patients affected by gait and sphincter disturbances improved after ETV. Attentive and executive functions as well as visuo-spatial memory and verbal executive functions improved in several patients. Similarly, the affective and behavioral scales improved in almost 50% of the patients. No major complications have been recorded, and no patients required a second surgery for shunt placement. ConclusionEndoscopic third ventriculostomy represents a safe and effective surgical procedure for the treatment of LIAS. In addition to neurological improvement, we demonstrated also postoperative neurocognitive improvement mainly in attentive and executive functions, visuo-spatial memory, verbal executive functions, and behavioral and affective domains

Open-aqueduct LOVA, LIAS, iNPH: a comparative clinical-radiological study exploring the "grey zone" between different forms of chronic adulthood hydrocephalus

The definition of chronic adult hydrocephalus encompasses different pathological entities with overlapping characteristics, including long-standing overt ventriculomegaly in adults (LOVA), late-onset idiopathic aqueductal stenosis (LIAS) and idiopathic normal pressure hydrocephalus (iNPH). The aim of our study was to identify preoperative clinical and radiological features peculiar of these diseases providing some pathophysiology inferences on these forms of hydrocephalus

In vivo assessment of Lewy body and beta-amyloid copathologies in idiopathic normal pressure hydrocephalus: prevalence and associations with clinical features and surgery outcome

Background: Idiopathic normal pressure hydrocephalus (iNPH) is a clinico-radiological syndrome of elderly individuals likely sustained by different neurodegenerative changes as copathologies. Since iNPH is a potentially reversible condition, assessing neurodegenerative pathologies in vitam through CSF biomarkers and their influence on clinical features and surgical outcome represents crucial steps.Methods: We measured a-synuclein seeding activity related to Lewy body (LB) pathology by the real-time quaking-induced conversion assay (RT-QuIC) and Alzheimer disease core biomarkers (proteins total-tau, phospho-tau, and amyloid-beta) by immunoassays in the cerebrospinal fluid (CSF) of 293 iNPH patients from two independent cohorts. To compare the prevalence of LB copathology between iNPH participants and a control group representative of the general population, we searched for a-synuclein seeding activity in 89 age-matched individuals who died of Creutzfeldt-Jakob disease (CJD). Finally, in one of the iNPH cohorts, we also measured the CSF levels of neurofilament light chain protein (NfL) and evaluated the association between all CSF biomarkers, baseline clinical features, and surgery outcome at 6 months.Results: Sixty (20.5%) iNPH patients showed alpha-synuclein seeding activity with no significant difference between cohorts. In contrast, the prevalence observed in CJD was only 6.7% (p= 0.002). Overall, 24.0% of iNPH participants showed an amyloid-positive (A+) status, indicating a brain co-pathology related to A beta deposition. At baseline, in the Italian cohort, a-synuclein RT-QuIC positivity was associated with higher scores on axial and upper limb rigidity (p=0.003 and p =0.011, respectively) and lower MMSEc scores (p =0.003). A+ patients showed lower scores on the MMSEc (p =0.037) than A- patients. Higher NfL levels were also associated with lower scores on the MMSEc (rho = -0.213; p= 0.021). There were no significant associations between CSF biomarkers and surgical outcome at 6 months (i.e. responders defined by decrease of 1 point on the mRankin scale).Conclusions: Prevalent LB- and AD-related neurodegenerative pathologies affect a significant proportion of iNPH patients and contribute to cognitive decline (both) and motor impairment (only LB pathology) but do not significantly influence the surgical outcome at 6 months. Their effect on the clinical benefit after surgery over a more extended period remains to be determined

Pattern of care and effectiveness of treatment for glioblastoma patients in the real world: Results from a prospective population-based registry. Could survival differ in a high-volume center?

BACKGROUND: As yet, no population-based prospective studies have been conducted to investigate the incidence and clinical outcome of glioblastoma (GBM) or the diffusion and impact of the current standard therapeutic approach in newly diagnosed patients younger than aged 70 years. METHODS: Data on all new cases of primary brain tumors observed from January 1, 2009, to December 31, 2010, in adults residing within the Emilia-Romagna region were recorded in a prospective registry in the Project of Emilia Romagna on Neuro-Oncology (PERNO). Based on the data from this registry, a prospective evaluation was made of the treatment efficacy and outcome in GBM patients. RESULTS: Two hundred sixty-seven GBM patients (median age, 64 y; range, 29-84 y) were enrolled. The median overall survival (OS) was 10.7 months (95% CI, 9.2-12.4). The 139 patients 64aged 70 years who were given standard temozolomide treatment concomitant with and adjuvant to radiotherapy had a median OS of 16.4 months (95% CI, 14.0-18.5). With multivariate analysis, OS correlated significantly with KPS (HR = 0.458; 95% CI, 0.248-0.847; P = .0127), MGMT methylation status (HR = 0.612; 95% CI, 0.388-0.966; P = .0350), and treatment received in a high versus low-volume center (HR = 0.56; 95% CI, 0.328-0.986; P = .0446). CONCLUSIONS: The median OS following standard temozolomide treatment concurrent with and adjuvant to radiotherapy given to (72.8% of) patients aged 6470 years is consistent with findings reported from randomized phase III trials. The volume and expertise of the treatment center should be further investigated as a prognostic factor

Gait Apraxia and Hakim’s Disease: A Historical Review

In 1965, Prof. Salomón Hakim described, for the first time, a condition characterized by normal pressure hydrocephalus and gait alterations. During the following decades, definitions such as “Frontal Gait”, “Bruns’ Ataxia” and “Gait Apraxia” have been frequently used in pertinent literature in the attempt to best define this peculiar motor disturbance. More recently, gait analysis has further shed light on the typical spatiotemporal gait alterations that characterize this neurological condition, but a clear and shared definition of this motor condition is still lacking. In this historical review, we described the origins of the terms “Gait Apraxia”, “Frontal Gait” and “Bruns’ Ataxia”, starting with the first works of Carl Maria Finkelburg, Fritsch and Hitzig and Steinthal during the second half of the 19th century and ending with Hakim’s studies and his formal definition of idiopathic normal pressure hydrocephalus (iNPH). In the second part of the review, we analyze how and why these definitions of gait have been associated with Hakim’s disease in the literature from 1965 to the present day. The definition of “Gait and Postural Transition Apraxia” is then proposed, but fundamental questions about the nature and mechanisms underlying this condition remain unanswered

Handmade Cranioplasty: An Obsolete Procedure or a Surgery That Is Still Useful?

Custom made cranioplasty (CM CPL) represents the gold standard for cranial defect reconstruction, allowing an adequate protection of the brain with good cosmetic results. Nevertheless, it is an expensive procedure, requiring time for preparation of the prothesis. Aim of this study is to evaluate whether and in what cases handmade cranioplasty (HM CPL) still represents a valid alternative to CM CPL.Therefore, all consecutive cranioplasties in our center from 2013 to 2019 have been analyzed. Size and location of cranial defect have been considered, as the cause of decompression. Morbidity rate and final outcome have been evaluated. Series includes 143 patients (61% males, mean age: 60 years, SD:16). HM CPL was performed in 59 cases (41.2%) and CM in the remaining 84 (58.8%). HM CPL was preferred for smaller cranial defects (p: 0.22), inferior to 100 cm (p: 0.01) located in fronto-parietal-occipital area (p: 0.01). Furthermore, HM CPL results in the first choice after removal of infiltrative tumors (p: 0.02). Surgical complications consisted in 14 (9%) infections, 8 hematomas (5.6%), and one cranioplasty displacement (0.7%). At follow-up, in 85.3% of cases the CPL is still in place with satisfactory cosmetic and functional outcome (86.5% of HM CPL, 84.5% of CM CPL).HM CPL may still represent an effective and economic alternative procedure, when specific patient selection criteria are respected. This technique requires a long learning learning curve and demanding surgical maneuvers. Our handmade reconstruction technique with some hints to improve the esthetic result is presented

From Shunt to Recovery: A Multidisciplinary Approach to Hydrocephalus Treatment in Severe Acquired Brain Injury Rehabilitation

Background: Hydrocephalus among Severe Acquired Brain Injury (SABI) patients remains overlooked during rehabilitation. Methods: A retrospective cohort study was carried out of traumatic and non-traumatic SABI patients with hydrocephalus, consecutively admitted over 9 years in a tertiary referral specialized rehabilitation hospital. Patients were treated with ventriculoperitoneal shunt before or during inpatient rehabilitation and assessed using the Level of Cognitive Functioning Scale and Disability Rating Scale. Logistic regression models were used to identify predictors of post-surgical complications. Linear regression models were used to investigate predictors of hospital length of stay (LOS), disability, and cognitive function. Results: Of the 82 patients, 15 had post-surgical complications and 16 underwent cranioplasty. Shunt placement complication risk was higher when fixed vs. when programmable pressure valves were used. A total of 56.3% achieved functional improvement at discharge and 88.7% improved in cognitive function; of the 82 patients, 56% were discharged home. In multiple regression analyses, higher disability at discharge was related to cranioplasty and longer LOS, while poorer cognitive function was associated with cranioplasty. Increase in LOS was associated with increasing time to shunt and decreasing age. Conclusions: A significant improvement in cognitive and functional outcomes can be achieved. Cranioplasty increased LOS, and fixed pressure valves were related to poorer outcomes

Ventriculoatrial Shunt Under Locoregional Anesthesia: A Technical Note.

International audienceVentriculoatrial shunt is routinely performed under general anesthesia and is used to treat various kinds of hydrocephalus. Idiopathic normal pressure hydrocephalus patients are generally elderly and can have high comorbidities; in such patients, avoiding general anesthesia and limiting opioid administration could be beneficial. We started to perform ventriculoatrial shunt under locoregional anesthesia, in order to make this procedure more truly "minimally invasive"

Choroidal morphology and microvascular structure in eyes of patients with idiopathic normal pressure hydrocephalus before and after ventriculo-peritoneal shunt surgery

Abstract The present study aims to investigate the choroidal morphology and microvascular structure in eyes of patients with idiopathic normal pressure hydrocephalus (iNPH) compared with the eyes of healthy age-matched individuals, and to assess the choroidal structure in eyes of iNPH patients before and after shunt surgery using Optical Coherence Tomography (OCT). The primary objective was to assess the choroidal morphology in eyes of iNPH patients before and after ventriculo-peritoneal (VP) surgery compared to age and sex-matched healthy individuals. The secondary objective was to compare the choroidal morphology of iNPH patients before and after a mean of 56 days from shunt surgery. Eighteen consecutive patients diagnosed with iNPH and 18 healthy controls were prospectively recruited between November 2021 and October 2022. Spectral-domain optical coherence tomography (SD-OCT) with enhanced depth imaging (EDI) was conducted before and within 4 months after shunt surgery. Images were binarized using the ImageJ software, and the choroidal vascular index (CVI) was calculated. Sub-foveal choroidal thickness (SFCT), total choroidal area (TCA), luminal choroidal area (LCA), and stromal choroidal area (SCA) were significantly increased in iNPH patients before surgery compared to the control group (p  0.05). In conclusion, the choroid was thicker in iNPH patients before and after VP shunt compared to age-matched healthy individuals. However, there were no difference in the choroidal microstructure in the eyes of iNPH patients before and after a mean of 3 months from VP shunt surgery

Magnetic Resonance-Based Assessment of Optic Nerve Sheath Diameter: A Prospective Observational Cohort Study on Inter- and Intra-Rater Agreement

Background: The measurement of optic nerve sheath diameter (ONSD) as a non-invasive method of estimating intracranial pressure has been widely reported in the literature. However, few studies have evaluated the accuracy of magnetic resonance imaging (MRI) in assessing ONSD measurements, although it is considered a very reliable method, it is not easily repeatable, expensive and is not readily available bedside. Herein, an assessment of the intra- and inter-rater reliability of ONSD assessment using MRI was conducted. Methods: A consecutive, prospective cohort of patients with suspected idiopathic normal-pressure hydrocephalus was analyzed. ONSD MRI measurements of the transverse and sagittal diameters at a distance of 3 mm behind the papilla were evaluated twice each by two expert neuroradiologists. The correlations between MRI examiners were calculated using the concordance correlation coefficient (CCC). Results: Fifty patients were included in the study. ONSD MRI average measurements were substantially higher than clinically expected (>5 mm). Considering intra-rater concordance, only one of the two neuroradiologists achieved an excellent score at CCC. Only a moderate inter-observer CCC for MRI assessment was found at all diameters. Conclusions: The use of a widespread MRI sequence (3D T1) to measure ONSD is not an accurate method since it may overestimate measurements and is dependent upon an operator