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OBJECTIVE Tailored surgery to extensively resect epileptogenic lesions using intraoperative electrocorticography (ioECoG) may improve seizure outcomes. However, resection of large areas is associated with decreased memory function postoperatively. The authors assessed whether ioECoG could provide useful information on how to minimize the focus resection and obtain better seizure outcomes without memory deterioration. They examined the postoperative seizure-free period and memory alteration in a retrospective cohort of patients with mesial temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) in whom the extent of removal was determined using ioECoG findings. METHODS The authors enrolled 82 patients with TLE associated with HS who were treated surgically. Transsylvian amygdalohippocampectomy was indicated as the first step. When visual inspection identified interictal epileptic discharges from the lateral temporal lobe on ioECoG, anterior temporal lobectomy (ATL) was eventually performed. The patients were divided into the selective amygdalohippocampectomy (SA, n = 40) and ATL (n = 42) groups. Postoperative seizure outcomes were assessed at 1, 2, 3, 5, and 7 years postoperatively using the International League Against Epilepsy classification. The Kaplan-Meier survival analysis was applied to evaluate the period of seizure recurrence between the SA and ATL groups. Factors attributed to seizure recurrence were analyzed using the Cox proportional hazards model, and they were as follows: epileptic focal laterality; age at seizure onset (< 10 or ≥ 10 years old); seizure frequency (more than weekly or less than weekly seizures); history of focal to bilateral tonic-clonic seizure; infectious etiology; and surgical procedure. The Wechsler Memory Scale–Revised was used to evaluate memory function pre- and postoperatively. RESULTS Seizure outcomes were significantly worse in the SA group than in the ATL group at 2 years postoperatively (p = 0.045). The International League Against Epilepsy class 1 outcomes at 7 years postoperatively in the SA and ATL groups were 63% and 81%, respectively. Kaplan-Meier analysis showed that seizure recurred significantly earlier in the SA group than in the ATL group (p = 0.031). The 2-way ANOVA analysis was used to compare the SA and ATL groups in each memory category, and revealed that there was no significant difference regardless of the side of surgery. CONCLUSIONS Visual assessment of ioECoG cannot be used as an indicator to minimize epileptic focus resection in patients with TLE associated with HS. ATL is more effective in obtaining seizure-free outcomes; however, both ATL and SA can preserve memory function.

Background: The amyloid-β oligomers, consisting of 10–20 monomers (AβO10–20), have strong neurotoxicity and are associated with cognitive impairment in Alzheimer’s disease (AD). However, their role in patients with idiopathic normal pressure hydrocephalus (iNPH) is poorly understood. Objective: We hypothesized that cerebrospinal fluid (CSF) AβO10–20 accumulates in patients with iNPH, and its clearance after CSF shunting contributes to neurological improvement. We measured CSF AβO10–20 levels before and after CSF shunting in iNPH patients evaluating their diagnostic and prognostic role. Methods: We evaluated two iNPH cohorts: “evaluation” (cohort-1) with 32 patients and “validation” (cohort-2) with 13 patients. Comparison cohorts included: 27 neurologically healthy controls (HCs), and 16 AD, 15 Parkinson’s disease (PD), and 14 progressive supranuclear palsy (PSP) patients. We assessed for all cohorts CSF AβO10–20 levels and their comprehensive clinical data. iNPH cohort-1 pre-shunting data were compared with those of comparison cohorts, using cohort-2 for validation. Next, we compared cohort-1’s clinical and CSF data: 1) before and after CSF shunting, and 2) increased versus decreased AβO10–20 levels at baseline, 1 and 3 years after shunting. Results: Cohort-1 had higher CSF AβO10–20 levels than the HCs, PD, and PSP cohorts. This result was validated with data from cohort-2. CSF AβO10–20 levels differentiated cohort-1 from the PD and PSP groups, with an area under receiver operating characteristic curve of 0.94. AβO10–20 levels in cohort-1 decreased after CSF shunting. Patients with AβO10–20 decrease showed better cognitive outcome than those without. Conclusion: AβO10–20 accumulates in patients with iNPH and is eliminated by CSF shunting. AβO10–20 can be an applicable diagnostic and prognostic biomarker.

Purpose Since a case of hydrocephalus in humans considered to be caused by ciliary dysfunction was first reported by Greenstone et al. in 1984, numerous papers on the correlation between ciliary function and hydrocephalus have been published. Methods We reviewed the published literature on primary ciliary dyskinesia in humans causing hydrocephalus, focusing on articles specifically examining the relation between ciliary function and hydrocephalus and its treatment. In addition, the authors’ experience is briefly discussed. Results Full texts of 16 articles reporting cases of human hydrocephalus (including ventriculomegaly) due to defects in ependymal ciliary function or primary ciliary dyskinesia observed in clinical practice were extracted. In recent years, studies on animal models, especially employing knockout mice, have revealed genetic mutations that cause hydrocephalus via ciliary dysfunction. However, a few reports on the onset of hydrocephalus in human patients with primary ciliary dyskinesia have confirmed that the incidence of this condition was extremely low compared to that in animal models. Conclusion In humans, it is rare for hydrocephalus to develop solely because of abnormalities in the cilia, and it is highly likely that other factors are also involved along with ciliary dysfunction.

Background Individuals with Sturge-Weber syndrome (SWS) often expereince intractable epilepsy and cognitive decline. We hypothesized that the extent of the leptomeningeal capillary malformation (LCM) may correlate with the severity of neurological impairment due to SWS. We tested the hypothesis in a cross-sectional study of seizure severity and electroencephalographic (EEG) findings and a retrospective cohort study for surgical indications related to the extent of the LCM. Methods We enrolled 112 patients and classified them according to LCM distribution: (1) bilateral, (2) hemispheric, (3) multilobar, and (4) single lobe. Age at seizure onset, seizure semiology and frequency, and EEG findings were compared. Surgical indications were evaluated for each group by Fisher exact test, and predictors for surgery were evaluated by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-Neurological Score (SWS-NS). Results The bilateral and hemispheric groups had early seizure onset (4.0 months old and 3.0 months old), frequent seizures (88.9% and 80.6% had more than one per month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). The groups’ EEG findings did not differ substantially. Surgical indications were present in 77.8% of the bilateral, 88.1% of the hemispheric, and 46.8% of the multilobar groups. Seizure more than once per month was a predictor of surgical treatment. Seizure subscore improved postoperatively in the hemispheric and multilobar groups. Even after surgical treatment, the bilateral and hemispheric groups exhibited higher SWS-NSs than members of the other groups. Conclusion Our study demonstrated a strong association between extensive LCM and epilepsy severity. Surgical intervention improved seizure outcome in patients with SWS with large LCMs.

Idiopathic normal pressure hydrocephalus (iNPH) is a condition resulting from impaired cerebrospinal fluid (CSF) absorption and excretion characterized by a triad of symptoms comprising dementia, gait disturbance (impaired trunk balance), and urinary incontinence. CSF biomarkers not only assist in diagnosis but are also important for analyzing the pathology and understanding appropriate treatment indications. As the neuropathological findings characteristic of iNPH have yet to be defined, there remains no method to diagnose iNPH with 100% sensitivity and specificity. Neurotoxic proteins are assumed to be involved in the neurological symptoms of iNPH, particularly the appearance of cognitive impairment. The symptoms of iNPH can be reversed by improving CSF turnover through shunting. However, early diagnosis is essential as once neurodegeneration has progressed, pathological changes become irreversible and symptom improvement is minimal, even after shunting. Combining a variety of diagnostic methods may lead to a more definitive diagnosis and accurate prediction of the prognosis following shunt treatment. Identifying comorbidities in iNPH using CSF biomarkers does not contraindicate shunting-based intervention, but does limit the improvement in symptoms it yields, and provides vital information for predicting post-treatment prognosis.

Spontaneous intracranial hypotension (SIH) is caused by cerebrospinal fluid (CSF) leakage. Patients with SIH experience postural headaches, nausea, etc., due to CSF hypovolemia. Imaging studies and clinical examinations, such as radioisotope (RI) scintigraphy, are useful for diagnosing SIH. However, 20-30% of patients do not show typical morphology and clinical test results. We previously reported that CSF contains transferrin (Tf) isoforms: “brain-type” Tf derived from the choroid plexus and “serum-type” Tf derived from blood. We showed that both isoforms increased in the CSF of patients with SIH by Western blotting. In the present study, we demonstrate that conventional ELISA for quantifying total Tf is useful for diagnosing SIH more accurately than Western blotting. In addition, SIH with chronic subdural hematoma (CSDH) was also accurately diagnosed. Total Tf in the CSF can serve as a useful biomarker for diagnosing SIH with or without CSDH.

ObjectiveBecause of the lack of highly skilled experts, automated technologies that support electroencephalogram (EEG)-based in epilepsy diagnosis are advancing. Deep convolutional neural network-based models have been used successfully for detecting epileptic spikes, one of the biomarkers, from EEG. However, a sizeable number of supervised EEG records are required for training.ApproachThis study introduces the Satelight model, which uses the self-attention (SA) mechanism. The model was trained using a clinical EEG dataset labeled by five specialists, including 16,008 epileptic spikes and 15,478 artifacts from 50 children. The SA mechanism is expected to reduce the number of parameters and efficiently extract features from a small amount of EEG data. To validate the effectiveness, we compared various spike detection approaches with the clinical EEG data.Main resultsThe experimental results showed that the proposed method detected epileptic spikes more effectively than other models (accuracy = 0.876 and false positive rate = 0.133).SignificanceThe proposed model had only one-tenth the number of parameters as the other effective model, despite having such a high detection performance. Further exploration of the hidden parameters revealed that the model automatically attended to the EEG’s characteristic waveform locations of interest.

Objectives Comorbidities of idiopathic normal pressure hydrocephalus (iNPH), such as Alzheimer's disease (AD) and Parkinson's spectrum (PS) disorder, can affect the long-term prognosis of cerebrospinal fluid (CSF) shunting. Therefore, it is important to be able to predict comorbidities in the early stage of the disease. This study aimed to predict the comorbidities of iNPH using neuropsychological tests and cognitive performance evaluation. Materials & methods Forty-nine patients with possible iNPH were divided into three groups: iNPH without AD or PS comorbidity (group-1), iNPH with AD comorbidity (group-2), and iNPH with PS comorbidity (group-3), according to CSF biomarkers such as phosphorylated tau and dopamine transporter imaging. Scores on the new EU-iNPH-scale, which is based on 4 neuropsychological tests (Rey Auditory Verbal Learning Test, Grooved Pegboard test, Stroop colour-naming test and interference test), were compared for each group. In addition, the scores before and 12 months after CSF shunting for each group were compared. Results EU-iNPH-scale using 4 neuropsychological tests could distinguish group-1 from group-2 or group-3 by area under the curve values of 0.787 and 0.851, respectively. Patients in group-1 showed a remarkable increase in memory and learning ability after surgery. Group-2 performed significantly poorer than group-1 patients on memory testing, but otherwise showed improvements in most of the neuropsychological tests. Group-3 performed significantly worse than group-1 patients-especially on Stroop tests-but showed post-surgery improvement on only the Stroop colour-naming test. Conclusions The 4 neuropsychological tests of the EU-iNPH-scale can help predict iNPH comorbidities and evaluate the prognosis of CSF shunting.

Background and purpose The pathophysiology of idiopathic normal pressure hydrocephalus (iNPH) has not been completely clarified. We investigated the brain structure in iNPH using automatic ventricular volumetry, single-tensor diffusion tensor imaging (DTI) and bi-tensor free-water (FW) imaging analyses while focusing on cognitive impairments before and after lumboperitoneal shunt surgery. Materials and methods This retrospective study included 12 iNPH patients with structural magnetic resonance imaging (MRI) and diffusion MRI (dMRI) on a 3T-MRI scanner who underwent neuropsychological assessments before and after shunting and 8 healthy controls. Ventricular volumetry was conducted on structural MRI datasets using FreeSurfer. Ventricular volume was compared pre- and postoperatively. Correlation analyses were performed between ventricular volume or volume change and neuropsychological scores or score change. Tract-based spatial statistics were performed using dMRI datasets for group analyses between iNPH and controls and between pre- and post-surgery iNPH patients and for correlation analyses using neuropsychological scores. Tract-specific analyses were performed in the anterior thalamic radiation (ATR), followed by comparison and correlation analyses. Results The third ventricular volume was significantly decreased after shunting; its volume reduction negatively correlated with a neuropsychological improvement. Compared with controls, iNPH patients had lower fractional anisotropy and higher axial, radial, and mean diffusivities, and FW in the periventricular white matter including ATR, resulting in no difference in FW-corrected indices. Single-tensor DTI indices partially correlated with neuropsychological improvements, while FW-corrected indices had no correlations. Conclusion Third ventricle enlargement is possibly linked to cognitive impairment and FW imaging possibly provides better white matter characterization in iNPH.

Background Intracranial hypotension is a disorder characterized by low cerebrospinal fluid (CSF) pressure typically caused by loss of CSF. Although some mechanisms account for the CSF leakage have been elucidated, spinal canal stenosis has never been reported as a pathological cause of intracranial hypotension. C1-C2 sign is a characteristic imaging feature, which indicates CSF collection between the spinous processes of C1 and C2, occasionally observed on magnetic resonance imaging (MRI) in patients with intracranial hypotension. Case presentation A 58-year-old man was presented to our institute with complaints of posterior cervical pain persisting for 3 months, along with numbness and muscle weakness of extremities. A fat suppression T2-weighted image of MRI illustrated fluid collection in the retrospinal region at C1-C2 level, and an 111In-DTPA cisternoscintigram clearly revealed the presence of CSF leakage into the same region. The MRI also showed stenosis in spinal canal at C3/4 level, and a computed tomography (CT) myelogram suggested a blockage at the same level. We gave a diagnosis as intracranial hypotension due to the CSF leakage, which might be caused by the spinal canal stenosis at C3/4 level. Despite 72 h of conservative therapy, a brain CT showed the development of bilateral subdural hematomas. We, therefore, performed burr-hole drainage of the subdural hematoma, blood-patch therapy at C1/2 level, and laminoplasty at C3–4 at the same time. Improvement of symptoms and imaging features which suggested the CSF leak and subdural hematoma were obtained post-operatively. Conclusion The present case suggested the mechanism where the CSF leakage was revealed as fluid collection in the retrospinal region at C1-C2 level. Increased intradural pressure due to the spinal canal stenosis resulted in dural tear. CSF leaked into the epidural space and subsequently to the retrospinal region at C1-C2 level, due to the presence of spinal canal stenosis caudally as well as the vulnerability of the tissue structure in the retrospinal region at C1-C2 level. Thus, our theory supports the mechanisms of previously reported CSF dynamics associated to C1-C2 sign, and also, we suggest spinal canal stenosis as a novel etiology of intracranial hypotension.

Background: We analyzed the predictive value of the tap test (TT) on the outcome of cerebrospinal fluid (CSF) shunting in patients with idiopathic normal pressure hydrocephalus (iNPH) and cognitive impairment up to 12 months postoperatively.Methods: We analyzed the data of two prospective multicenter studies on ventriculoperitoneal shunt (VPS) and lumboperitoneal shunt (LPS) use in iNPH patients. We selected patients with Mini-Mental State Examination (MMSE) scores ≤ 26 points as study subjects. We used a multivariate logistic regression model to obtain the optimal threshold of MMSE scores after TT to predict the score improvement at 12 months following shunting and that helped to control for confounding factors such as age and MMSE scores before TT. We used logistic regression models to identify variables with age-adjusted odds ratio (A-OR) and multivariate-adjusted OR (M-OR).Results: For an improvement of ≥3 points in the MMSE score cutoff 7 days following TT in VPS and LPS cohort studies, the MMSE scores improved by 6 points after 12 months. The VPS cohort had sensitivity, specificity, and area under the curve (AUC) of 69.2, 73.7, and 0.771%, respectively; however, for the LPS cohort, they were 86.2, 90.9, and 0.906%, respectively. For MMSE scores that improved by ≥3 points in patients after the TT, the possibility of an improvement by 6 points at 12 months following CSF shunt had A-OR 7.77 and M-OR 6.3 times for the VPS, and A-OR 62.3 and M-OR 59.6 times for the LPS cohort.Conclusion: CSF shunting contributes to improved cognitive function in iNPH patients. Furthermore, MMSE score evaluation at the TT can sensitively predict improvement in postoperative MMSE scores following LPS intervention.Clinical Trial Registration: SINPHONI-1 (ClinicalTrials.gov, no. NCT00221091), first posted: September 22, 2005.SINPHONI-2 [University Hospital Medical Information Network (UMIN) Clinical Trials no. UMIN000002730], the posted: February 1, 2010.

Background In many brain volumetry reports, data from both controls and patients have been used to create the diffeomorphic anatomical registration through exponentiated lie algebra (DARTEL) templates for spatial normalization, whereas some studies used data from only the control group. Purpose To investigate the association between lesion detection and the processes used in creating DARTEL template in group comparisons with voxel-based morphometry. Material and Methods Three-dimensional T1-weighted MRI data of 31 healthy controls, 31 patients with Alzheimer’s disease, and 31 patients with idiopathic normal pressure hydrocephalus were obtained using a 3-T magnetic resonance scanner. Segmented gray matter images were then deformed into three types of DARTEL templates using the Statistical Parametric Mapping 12 software. Results Processes 1 (DARTEL template with all individuals) and 2 (DARTEL template with healthy controls) showed significant lesions, as observed in previous reports, whereas process 3 (DARTEL template with individuals divided into two groups) showed significantly larger lesions than those noted with processes 1 and 2. Conclusion The present study demonstrated that lesion detection depends on the processes used in the creation of the DARTEL template.

Background Patients with idiopathic normal-pressure hydrocephalus (iNPH) are typically older adults with multiple comorbidities that are associated with a reduction in the efficacy of iNPH treatment via cerebrospinal fluid (CSF) shunt placement. Objective The present study aimed to investigate the effectiveness of CSF shunt for iNPH using data from a nationwide epidemiological survey in Japan. Methods We examined 1,423 patients (581 women) aged ≥60 years (median age [25%-75%]: 77 [73-80] years) who were diagnosed with iNPH following a hospital visit in 2012. Patients who experienced an improvement of at least one modified Rankin Scale (mRS) grade after the CSF shunt were classified as "improvement" while the remaining patients were classified as "non-improvement." The efficacy of the shunt intervention (n = 842) was analyzed using a binomial logistic regression analysis. Results An analysis of risk factors associated with shunt placement in patients with mRS grade 2 revealed an association between comorbid chronic ischemic lesions (odds ratio [OR], 2.28; 95% confidence interval [CI], 1.11-4.67; p = 0.025) and cervical spondylosis (OR, 3.62; 95% CI, 1.15-11.34; p = 0.027). Patients with mRS grade 3 at study entry had an association with comorbid Alzheimer's disease (OR, 3.02; 95% CI, 1.44-6.31; p = 0.003). Conclusions The results presented here showed that any age-related risk is minimal and should not be cause for rejection of surgical treatment options. Clinical decisions regarding CSF shunt should be individualized to each patient, with adequate consideration of the relative risks and benefits, including maximizing a healthy life expectancy.

Numerous studies have been conducted on indoor and outdoor seamless positioning and indoor&ndash, outdoor detection methods. However, the classification of real space into two types, outdoor space and indoor space, is difficult. One type of space that is difficult to classify is top-bounded space, which can be observed in commercial facilities, logistics facilities, and street-facing sidewalks. In this study, we designed a method for detecting stays in three spatial contexts: Outdoor, top-bounded space, and indoor. This method considers elongated top-bounded spaces covered with a roof and open on one of the sides. Specifically, we selected Global Positioning System (GPS) satellites for stay detection based on the simple extraction of the spatial characteristics of a top-bounded space and designed a decision flow using fuzzy inference based on the signal-to-noise ratio (SNR) of the selected GPS satellites. Moreover, we conducted an evaluation experiment to verify the effectiveness of the proposed method and confirmed that it could correctly detect the stay in three spatial contexts, outdoor, top-bounded space, and indoor, with a high probability of 93.1%.

With the widespread use of indoor positioning technology, various services based on this technology are beginning to be offered to consumers and industrial applications. In the case of logistics facilities, in addition to indoor and outdoor spaces, there are top-bounded spaces (TBSs): elongated areas that are covered with roofs or eaves on the upper parts of buildings. The sides of such spaces are open, and workers and forklifts work in these areas. Only a few studies have been conducted on positioning methods for this unusual environment, and the way by which Signal-to-Noise Ratio (SNR) of Global Positioning System (GPS) changes with the stay in TBSs is unclear. Therefore, we conducted preliminary experiments and confirmed that TBS dwellings are difficult to stably detect with existing methods due to the combination of satellites with variable and unchanged SNRs. In this study, we designed a simple processing flow for selecting satellites with high probabilities of changing SNRs by using the spatial characteristics of TBSs as parameters (height, depth, and side opening orientation). We propose a method to detect the stay in TBSs using the SNR change rates of the selected satellites. As a result of evaluation experiments with three TBSs, we successfully detected the stay in TBSs with about 30% higher probability than those of an existing method.

To cope with the lack of highly skilled professionals, machine learning with proper signal processing is key for establishing automated diagnostic-aid technologies with which to conduct epileptic electroencephalogram (EEG) testing. In particular, frequency filtering with the appropriate passbands is essential for enhancing the biomarkers—such as epileptic spike waves—that are noted in the EEG. This paper introduces a novel class of neural networks (NNs) that have a bank of linear-phase finite impulse response filters at the first layer as a preprocessor that can behave as bandpass filters that extract biomarkers without destroying waveforms because of a linear-phase condition. Besides, the parameters of the filters are also data-driven. The proposed NNs were trained with a large amount of clinical EEG data, including 15,833 epileptic spike waveforms recorded from 50 patients, and their labels were annotated by specialists. In the experiments, we compared three scenarios for the first layer: no preprocessing, discrete wavelet transform, and the proposed data-driven filters. The experimental results show that the trained data-driven filter bank with supervised learning behaves like multiple bandpass filters. In particular, the trained filter passed a frequency band of approximately 10–30 Hz. Moreover, the proposed method detected epileptic spikes, with the area under the receiver operating characteristic curve of 0.967 in the mean of 50 intersubject validations.

Introduction Our previous community-based study demonstrated that some individuals with AVIM [asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus (iNPH) on magnetic resonance imaging (MRI)] progressed to iNPH in several years. In this hospital-based study, we investigated the progression rate from AVIM to iNPH and its possible predictors. Methods We conducted a prospective study of participants with AVIM from several medical institutions/hospitals in Japan. AVIM is defined as “asymptomatic ventriculomegaly with features of iNPH on MRI”; in the present study, asymptomatic was defined as “0 (no symptoms) or 1 (presence of only subjective, but not objective, symptoms) on the iNPH Grading Scale (iNPH-GS).” We also measured possible predicting factors for AVIM-to-iNPH progression, including age, sex, body weight, blood pressure, diabetes mellitus, dyslipidemia, history of mental disease/head injury/sinusitis/smoking/alcohol-intake, Evans index, and the presence of DESH (disproportionately enlarged subarachnoid-space hydrocephalus) findings on brain MRI, and analyzed these potential predictive values. Results In 2012, 93 participants with AVIM were registered and enrolled in the study. Of these, 52 participants were able to be tracked for three years (until 2015). Of the 52 participants, 27 (52%) developed iNPH during the follow-up period (11 definite, 6 probable, and 10 possible iNPH), whereas 25 participants remained asymptomatic in 2015. Among the possible predictive factors examined, the baseline scores of iNPH-GS predicted the AVIM-to-iNPH progression. Conclusions The multicenter prospective study demonstrated that the progression rate from AVIM to iNPH was ~17% per year, and the baseline scores of iNPH-GS predicted the AVIM-to-iNPH progression.

Background Alzheimer's disease (AD) pathology in idiopathic normal pressure hydrocephalus (iNPH) contributes to poor shunt responses. Amyloid-β 1- 42 (Aβ42) toxic conformer was recently identified with features of rapid oligomerization, strong neurotoxicity and synaptotoxicity. Objective This observational study points to Aβ42 toxic conformer as a biomarker for AD pathology and for poor postoperative prognosis in patients with iNPH. Methods The first cohort consisted of patients with AD (n = 17) and iNPH (n = 17), and cognitively normal individuals (CN, n = 12). The second cohort, consisted of 51 patients with iNPH, was divided into two groups according to phosphorylated Tau (pTau) level (low- and high-pTau groups); the low-pTau group was further subdivided according to one-year postoperative change in Aβ42 toxic conformer ratio (%) [Aβ42 toxic conformer/Aβ42×100] (decreased- and increased-conformer subgroups). Enzyme-linked immunosorbent assay was used to measure pTau, Aβ42, and Aβ42 toxic conformer in cerebrospinal fluid. Outcomes were evaluated using neuropsychological tests one- and two-years postoperatively. Results In the first cohort, Aβ42 toxic conformer ratio in the iNPH group (10.8%) was significantly higher than that in the CN group (6.3%) and significantly lower than that in the AD group (17.2%). In the second cohort, the high-pTau group showed cognitive decline two-years postoperatively compared to baseline. However, the low-pTau group showed favorable outcomes one-year postoperatively; furthermore, the increased-conformer subgroup showed cognitive decline two-years postoperatively while the decreased-conformer subgroup maintained the improvement. Conclusions Change in Aβ42 toxic conformer ratio predicts long-term cognitive outcome in iNPH, even in the low-pTau group.

Idiopathic normal pressure hydrocephalus (iNPH) is a dementia-inducing disorder. Primary cause of iNPH is speculated to be a reduction of cerebrospinal fluid (CSF) absorption, which secondarily induces hydrocephalus, compression of brain, and reduction of CSF production. Patients are treated by surgically inserting a shunt to deliver excess CSF to the abdominal cavity. The prognosis for cognitive improvement after shunt surgery has been difficult to predict. We therefore investigated various CSF proteins, hoping to find a biomarker predictive of cognitive performance one to two years after shunt surgery. CSF proteins of 34 iNPH and 15 non-iNPH patients were analysed by Western blotting, revealing two glycan isoforms of transferrin (Tf); 'brain-type' Tf with N-acetylglucosaminylated glycans and 'serum-type' Tf with α2, 6-sialylated glycans. Brain-type Tf levels decreased in iNPH but rapidly returned to normal levels within 1-3 months after shunt surgery. This change was positively correlated with recovery from dementia, per Mini-Mental State Examination and Frontal Assessment Battery scores at 11.8 ± 7.7 months post-operation, suggesting that brain-type Tf is a prognostic marker for recovery from dementia after shunt surgery for iNPH. Histochemical staining with anti-Tf antibody and an N-acetylglucosamine-binding lectin suggests that brain-type Tf is secreted from choroid plexus, CSF-producing tissue.

Background Treating idiopathic normal pressure hydrocephalus (iNPH) with lumboperitoneal shunts (LPSs) may cause cerebrospinal fluid (CSF) overdrainage. Objective To investigate whether LPSs, including gravitational "add-on" and programmable pressure valves (PPVs/+GVs), reduce complications and improve outcomes. Methods We compared PPVs/+small lumen abdominal catheters (SLs) to PPVs/+GVs using different opening pressures for supine and standing positions. We analyzed 115 patients with iNPH in 2 consequent cohorts: 48 patients receiving LPSs with PPVs/+SLs and 67 patients receiving LPSs with PPVs/+GVs. The modified Rankin Scale (mRS), Japan iNPH grading scale, Mini Mental State Examination, Frontal Assessment Battery, and CSF biomarkers were evaluated. Results Comparisons of postoperative clinical factors in 64 patients in the PPV/+SL and PPV/+GV groups using 1:1 propensity score matching revealed differences in the mean (±standard deviation) postoperative mRS (2.65 ± 1.07 vs 2.16 ± 1.02, P = .049) and gait disturbance scores (1.97 ± 1.03 vs 1.39 ± 0.92, P = .011). Thus, outcomes improved in the LPS group with the GV. Serious and nonserious adverse event rates for the PPV/+SL and PPV/+GV groups were 22.9% and 19.4% (P = .647) and 38% and 17.9% (P = .018), respectively, indicating higher rates of subdural collections for the PPV/+SL group. Conclusion This is the first study to examine LPS treatment for iNPH using a GV in tandem with a PPV. Our results suggest that the CSF shunt flow volume is restricted in the standing position and maintained in the supine position, thus improving iNPH symptoms. This may reduce intracranial CSF hypotension-related complications.

We report the case of a young woman who received intrathecal baclofen therapy (ITB) and subsequently became pregnant and had a normal delivery. A 28-year-old woman with flexion myelopathy had anterior decompression with fusion at C4/5 and C5/6 levels. Clinical symptoms improved after surgery. However, when she was 29 years old, her symptoms steadily advanced to Modified Ashworth Scale 3 spasticity level in the lower legs, with pain in both of them and urinary retention tendency. Since a 25 μg intrathecal baclofen injection improved her symptoms, an ITB pump system was implanted. After surgery, lower limb spasticity and urinary retention improved. Two years after ITB pump implantation, the patient married and became pregnant. The patient intended to have normal delivery but the induction of labor was ineffective and childbirth was completed by Cesarean section with lumbar anesthesia. The infant’s Apgar score was 8 at 1 min and 9 at 5 min, and birth-weight was 2,704 g. We measured the baclofen concentration in the patient’s breast milk using high-performance liquid chromatography/tandem mass spectrometry. The level of baclofen in the breast milk was very low (0.617 ng/ml) and the predicted pharmacological effect on the infant was judged to be negligible. No withdrawal symptoms or muscle tone abnormalities were found after birth. Our findings indicate that ITB therapy could be considered for young women with severe spasticity, even if they plan to have children.

This study used location and physical-activity data to test hypothesis that Tokyo’s residents exercise more because they walk to and from public transit stations for daily trips. An activity tracker and a mobile app were used by ten subjects over 48 days to collect data. Analysis of the dataset showed that on an average, all subjects walked over 10,000 steps per day and ∼40% of trips were taken in outdoor urban environments. Results also revealed that the subjects did not always take the shortest path between the origin and destination of a walking trip. These results suggest that urban residents choose routes of travel based on both internal and external factors, including the subjective effects of street design and personal preference.

The symptoms of idiopathic normal pressure hydrocephalus (iNPH) can be improved by shunt surgery, but prediction of treatment outcome is not established. We investigated changes of the corticospinal tract (CST) in iNPH before and after shunt surgery by using diffusion microstructural imaging, which infers more specific tissue properties than conventional diffusion tensor imaging. Two biophysical models were used: neurite orientation dispersion and density imaging (NODDI) and white matter tract integrity (WMTI). In both methods, the orientational coherence within the CSTs was higher in patients than in controls, and some normalization occurred after the surgery in patients, indicating axon stretching and recovery. The estimated axon density was lower in patients than in controls but remained unchanged after the surgery, suggesting its potential as a marker for irreversible neuronal damage. In a Monte-Carlo simulation that represented model axons as undulating cylinders, both NODDI and WMTI separated the effects of axon density and undulation. Thus, diffusion MRI may distinguish between reversible and irreversible microstructural changes in iNPH. Our findings constitute a step towards a quantitative image biomarker that reflects pathological process and treatment outcomes of iNPH., Highlights • NODDI and WMTI provide markers of reversible and irreversible changes in iNPH. • Measures of axon orientation indicated recovery from stretching after surgery. • Axon density remained low after surgery, suggesting chronic neuronal damage. • Axon stretching in simulations differentially affected diffusion metrics.

This paper presents a new immune inspired algorithm, the Clonal Selection Algorithm for Multi-agent Itinerary Planning (CSA-MIP), to solve the MIP problem in wireless sensor networks. CSA-MIP has two computational stages called Stage I and Stage II. When only Stage I is activated in CSA-MIP, in the obtained solutions, the difference between the maximum and minimum numbers of sensor nodes, visited by each mobile agent, is relatively large. When only Stage II is activated in CSA-MIP, in the obtained solutions, the number of mobile agents is relatively small. When both stages are activated in CSA-MIP, in the obtained solutions, the diversity in the number of mobile agents can be achieved and the unbalance in the number of sensor nodes visited by each mobile agent can be reduced, which indicate a higher possibility to obtain solutions of better quality. Moreover, according to the simulation results and analysis of computational complexity, CSA-MIP is shown to perform better than GA-MIP in terms of solution quality and computational efficiency.

Introduction Disproportionately Enlarged Subarachnoid space Hydrocephalus (DESH) is considered as an important imaging feature of idiopathic normal pressure hydrocephalus (iNPH). Method Subjects aged 60 and over in a memory clinic and a community-based cohort were assessed for the presence of ventriculomegaly, Sylvian dilatation, and high convexity tightness by neuroimaging, and a clinical triad of iNPH symptoms, i.e. cognitive, gait and urinary symptoms. Results In the memory clinic-based study (548 subjects), the prevalence of DESH was 1.1% and increased with age. The clinical triad was significantly more frequent in subjects with DESH (50%) compared to those with normal images (none), Sylvian dilatation (7%), and ventriculomegaly (12%). Gait disturbance was also significantly more frequent in DESH (83%) compared to those with normal images (2%), Sylvian dilatation (14%), and ventriculomegaly (26%). In the community-based cohort (946 subjects), the prevalence of DESH was 1.0% and increased with age. The clinical triad (11%) was significantly more common in subjects with DESH compared to those with normal images (none), Sylvian dilatation (2%), and ventriculomegaly (7%). Gait disturbance was also significantly more common in DESH (33%) compared to those with normal images (1%), Sylvian dilatation (4%), and ventriculomegaly (10%). Conclusion The reported prevalence of DESH was approximately 1%, and increased with age. DESH and high convexity tightness were specifically associated with the clinical triad of iNPH. Of the triad, gait disturbance was associated to DESH and high convexity tightness.

Leucine-rich-alpha-2-glykoprotein (LRG) is suggested as a potential biomarker for idiopathic normal pressure hydrocephalus (iNPH). Our goal was to compare the cerebrospinal fluid (CSF) LRG levels between 119 iNPH patients and 33 age-matched controls and with the shunt responses and the brain biopsy Alzheimer’s disease (AD) pathology among the iNPH patients. CSF LRG, Aβ1-42, P-tau181, and T-tau were measured by using commercial ELISAs. The LRG levels in the CSF were significantly increased in the iNPH patients (p &lt, 0.001) as compared to the controls, regardless of the AD pathology. However, CSF LRG did not correlate with the shunt response in contrast to the previous findings. The CSF AD biomarkers, i.e., Aβ1-42, T-tau, and P-tau correlated with the brain biopsy AD pathology as expected but were systematically lower in the iNPH patients when compared to the controls (&lt, 0.001). Our findings support that the LRG levels in the CSF are potentially useful for the diagnostics of iNPH, independent of the brain AD pathology, but contrary to previous findings, not for predicting the shunt response. Our findings also suggest a need for specific reference values of the CSF AD biomarkers for the diagnostics of comorbid AD pathology in the iNPH patients.

Purpose The purpose of this paper is to present the use of artificial immune systems (AISs) to solve constrained design optimization problems for active magnetic bearings (AMBs). Design/methodology/approach This research applies the AIS approach, more specifically, a representative clonal selection-based AIS called CLONALG, to the single-objective structural design optimization of AMBs. In addition, when compared with a genetic algorithm (GA) developed in the previous work, the CLONALG fails to produce best solutions when a nearly zero feasible ratio occurs in an AMB design problem. Therefore, an AIS called ARISCO (AIS for constrained optimization) is proposed to address the above issue. Findings A total of six AMB design cases are solved by the GA, CLONALG, and ARISCO. Based on the simulation results, in terms of solution quality, the ARISCO is shown to have better overall performance than the CLONALG and GA. In particular, when solving a problem with a nearly zero feasible ratio, the ARISCO and GA perform equally and both outperform the CLONALG. Originality/value In summary, the contributions of this paper include: this research applies the AIS approach, more precisely, the CLONALG, to the single-objective structural design optimization of AMBs; the ARISCO overall produces better AMB designs than the CLONALG and a GA developed in the previous work; in situations where a nearly zero feasible ratio occurs, the ARISCO and GA perform equally, and they both outperform the CLONALG.

To evaluate diffusional changes of the corticospinal tract (CST) in patients with idiopathic normal pressure hydrocephalus (iNPH) by neurite orientation dispersion and density imaging (NODDI). Nineteen patients with iNPH and 12 healthy controls were included. Diffusion MRI data for NODDI were acquired with a 3-T system, using 32 motion-probing gradient directions with six b-values (from 0 to 2500 s/mm2). The orientation dispersion index (ODI), intra-cellular volume fraction (Vic), and isotropic volume fraction (Viso) of the CST were calculated by tract-specific analysis in patients and controls. We also measured the fractional anisotropy (FA) and apparent diffusion coefficient (ADC). The ODI of the CST (0.087 ± 0.024 vs. 0.183 ± 0.051, P

This paper presents an effective and simple target tracking approach called PSIPT (Particle filter Single Image based Planar Target tracking). Compared with other works, the uniqueness of PSIPT includes: (1) only a single color camera provides the images to be processed; (2) the particle filter does not perform data fusion calculations; (3) the distance evaluation carried out in the particle filter does not need the camera’s intrinsic and extrinsic parameters. Meanwhile, this paper also reveals that, under different target shapes and cameras, a high degree of negative linear dependence remains between: (1) a target’s pixel height and vertical distance; (2) a target’s vertical distance and PWHD (Pixel-Width-to-Horizontal Distance) ratio. According to the experimental results, PSIPT performs better than its Kalman filter variant in both the L-shape and S-shape tracking experiments. In addition, PSIPT has moderate performance in the target missing surveillance experiment. Moreover, a hybrid and enhan...

Background Chronic cerebrospinal fluid (CSF) over-drainage can induce compressive myelopathy without typical over-drainage manifestations. We present two cases of cervical myelopathy due to chronic CSF over-drainage that were successfully treated by gravitational add-on valve placement. Case description Patient 1 was a 38-year-old man who presented with pain and numbness in his left arm and hand, as well as weakness of the upper extremities. He had undergone cystoperitoneal shunting for a retro-cerebellar arachnoid cyst treatment. Patient 2 was an 83-year-old woman who presented with weakness and numbness of both upper and lower limbs. At the age of 72, ventriculoperitoneal shunting was performed for the treatment of hydrocephalus following subarachnoid hemorrhage. Magnetic resonance imaging (MRI) and three-dimensional computed tomography (3DCT) angiography revealed marked compression of the spinal cord and dural sac by enlarged epidural venous plexus in both patients. A gravitational valve was additionally inserted and patient’s symptoms improved. Post-operative MRI revealed decrease of the epidural venous plexus engorgement and resolution of the spinal cord compression. Conclusions Some rare pathophysiological relationships between intracranial hypotension and cervical myelopathy, can be difficult for diagnosis. Our findings indicate that proper diagnosis of such relationships after CSF shunt placement can provide relief of myelopathic symptoms.

Painful legs and moving toes (PLMT) syndrome is a clinical entity characterized by persistent pain in the feet or legs and involuntary movements of one or more toes. The precise patho-mechanisms of PLMT still remain unknown. Herein, we examined ten patients clinically identified with PLMT syndrome. All patients first presented persistent pain prior to the onset of motor symptoms. Each patient was examined by neurological investigation, neuro-imaging methods including brain magnetic resonance imaging (MRI) and electrophysiological methods. The brain single photon emission computed tomography (SPECT) images of eight patients indicated hypoperfusion of frontal lobes and cerebellum. The conjunction analysis of brain SPECT imaging data of all eight patients, using the 3D-SSP program, compared to 34 controls indicated significant hypoperfusion in the prefrontal cortical, occipital cortical, and cerebellar surfaces, and thalamus, and hyperperfusion in the surface of the anterior cingulate gyrus and parietal cortices including primary and secondary somatosensory cortices, bilaterally. These areas reflected on a part of the pain matrix. Other electrophysiological examinations did not indicate specific abnormalities to explain the patients’ symptoms. On treatment with clonazepam, four out of nine patients could resolve their foot-related motor symptoms, but not the sensory symptoms. Overall, their pain was an intractable and persistent symptom throughout their clinical course. Our study infers that PLMT syndrome is fundamentally a chronic pain disorder, possibly relating to the central sensitization, involving the region of a part of pain matrix. Further studies need to confirm our results by adding more patients.

Background Experiencing a ventriculoperitoneal (VP) shunt complication is a major obstacle in the management of hydrocephalus. Degradation of a shunt catheter associated with surrounding tissue calcification could be 1 reason for a difference in facture rates. Furthermore, tissue reactions around cerebrospinal fluid shunts may be a sign of bacterial shunt infection, which is not uncommon. Case Description A 31-year-old man was living with a ventriculoperitoneal shunt since childhood. Consequently, his cerebrospinal fluid absorption was supposed to be modified by the shunt. Shunt malfunction later occurred concomitant with symptoms of headache and repeated vomiting. He had undergone shunt revision a year before presentation, but examination revealed that a new, extremely rare calcified lesion had formed in the aponeurosis of the abdomen, compressing the shunt tube. We removed it and replaced the shunt tube, thus relieving his symptoms. Conclusions We treated a rare case of shunt dysfunction caused by calcification of the aponeurosis coinciding with significant weight gain over the course of a year. To the best of our knowledge, this is the first report to describe a case of shunt malfunction caused by calcification of the aponeurosis.

The two pillars of descriptive and analytic epidemiological studies on intractable diseases are clarification of the number of patients showing symptoms and basic clinical features. This report introduces the importance of descriptive and analytic epidemiology of intractable diseases with regard to the significance, methodology, and points of attention through a nationwide survey of idiopathic normal pressure hydrocephalus (iNPH) that we experienced including specific survey methods, with introduction of reports from Japan and other countries.

Background and Purpose: This study aimed to investigate the efficacy of cerebrospinal fluid shunt intervention for idiopathic normal pressure hydrocephalus (iNPH) using data from a nationwide epidemiological survey in Japan.Methods: We conducted a cross-sectional study using data from a nationwide epidemiological survey performed in Japan. Propensity score matching was used to select 874 patients from 1,423 patients aged ≥60 years, who were diagnosed with iNPH based on clinical guidelines following a hospital visit in 2012. Patients who experienced an improvement of at least 1 modified Rankin Scale (mRS) grade after the intervention were classified as “improved,” while the remaining patients were classified as “non-improved.” In the shunt intervention (n = 437) and non-shunt intervention (n = 437) groups, the differences in mRS grade improvement were analyzed using the Mann-Whitney U-test. Finally, we examined subjects in the shunt intervention group (n = 974) to compare the outcomes and complications of ventriculoperitoneal (VP) shunt (n = 417) with lumboperitoneal (LP) shunt (n = 540).Results: We examined subjects with iNPH to compare the non-shunt intervention group to the shunt intervention group following adjustment for age and mRS grade at baseline by propensity score matching (0.31–0.901). The mRS grade (mean [SD]) was found to improve with non-shunt intervention (2.46 [0.88]) and shunt intervention (1.93 [0.93]) (p < 0.001) in iNPH patients. The mRS outcome score and complications comparison between the VP and LP shunt groups did not show significant difference.Conclusions: In this study, analysis of the efficacy of shunts for possible iNPH conducted in Japan indicated a significant improvement in the mRS grade between baseline and outcome within 1 year, regardless of the surgical technique, and shunt intervention was found to be effective.

The purpose of this study was to elucidate the specific regional cerebral blood flow (rCBF) alterations for idiopathic normal pressure hydrocephalus (iNPH) by comparing the proportional rCBF and gray matter change from those of a normal database at the same point of SPECT and MRI examinations. Thirty subjects with iNPH underwent both CBF SPECT and MRI. After normalization, voxel-wise two-sample t tests between patients and 11 normal controls were conducted to compare the regional alteration in the gray matter density and rCBF. The rCBF reduction and the gray matter decrease were seen in almost similar regions surrounding Sylvian fissure, the left parietotemporal region and frontal lobes, whereas we did not find rCBF increase at the top of the high convexity, where the increase of the gray matter density was the highest (p

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This paper presents a new method that applies laser line scanning to hydrodynamic coefficient identification for a self-developed autonomous underwater vehicle (AUV). The AUV positions/angles are calculated using laser images captured by an onboard camera, and a heuristic method is used to obtain an optimal combination of the hydrodynamic coefficients to be identified. In this paper, the AUV surge motion is considered, and a simulated experiment is conducted through OpenGL to obtain the surge-related hydrodynamic coefficients. Through the comparisons between the “actual”, “laser image based”, and “identified coefficient based” position datasets for the AUV surge motion, the feasibility of the proposed method is preliminarily validated.

Background:The 3-meter Timed Up and Go test (TUG) is a reliable quantitative test for assessment of gait and balance. We aimed to establish an optimal threshold of TUG at the tap test for predicting outcomes 12 months after shunt surgery in patients with idiopathic normal pressure hydrocephalus (iNPH).Methods:The TUG was measured in a total of 151 patients with possible iNPH before and after a tap test and 12 months after shunt surgery. Among them, 81 patients underwent ventriculoperitoneal shunt implantation (SINPHONI) and 70 underwent lumboperitoneal shunt implantation (SINPHONI-2). The areas under the curve (AUCs), sensitivities, and specificities for predicting shunt effectiveness were assessed.Results:The simple differences of time on TUG at the tap test were significantly more accurate for predicting shunt effectiveness than percent improvement of time. The highest AUC for the synchronized moving cutoff point of TUG time was 0.81 (sensitivity 81.0%; specificity 81.6%) at the threshold of 5 seconds in the SINPHONI-2. For predicting improvements of ≥10 seconds 12 months after lumboperitoneal shunt implantation, the AUC was 0.90, and the sensitivity and specificity at the threshold of 5.6 seconds were 83.3% and 81.0%. Only for patients with a Conclusions:An improvement of 5 seconds was a useful threshold of TUG time at the tap test for predicting a ≥10-second improvement 12 months after shunt surgery, rather than the percent improvement of TUG time.

Objectives There have been no nationwide epidemiological studies of idiopathic normal pressure hydrocephalus (iNPH) in Japan. Therefore, a nationwide epidemiologic survey of iNPH was performed to determine the number of cases and clinical characteristics by sex and diagnostic level. Methods The first survey examined the numbers of cases that met the diagnostic criteria of iNPH and those who underwent shunt operations in 2012. The second survey gathered patients' details to clarify their clinical background characteristics. Results The estimated number of cases meeting the diagnostic criteria in 2012 was 12,900, with 6,700 undergoing shunt operations. The estimated crude prevalence was 10.2/100,000 persons. The age of onset was in the 70s in more than 50% of both men and women. Significantly higher (p

Background: Patients presenting with the classical idiopathic normal pressure hydrocephalus (iNPH) triad often show additional parkinsonian spectrum signs. Accurate differential diagnosis strongly influences the long-term outcome of cerebrospinal fluid (CSF) shunting. Objective: The aim of this study was to find potential CSF microRNA (miRNA) biomarkers for NPH mimics with parkinsonian syndromes that can reliably distinguish them from iNPH patients. Methods: Two cohorts of 81 patients (cohort 1, n = 55; cohort 2, n = 26) with possible iNPH who were treated in two centers between January 2011 and May 2014 were studied. In both cohorts, CSF samples were obtained from patients clinically diagnosed with iNPH (n = 21 and n = 10, respectively), possible iNPH with parkinsonian spectrum (PS) (n = 18, n = 10, respectively), possible iNPH with Alzheimer’s disease (AD) (n = 16), and non-affected elderly individuals (NC) (n = 6). A three-step qRT-PCR analysis of the CSF samples was performed to detect miRNAs that were differentially expressed in the groups. Results: The expression of hsa-miR-4274 in CSF was decreased in both cohorts of PS group patients (cohort 1: p

Background Deep brain stimulation is a treatment option for patients with Parkinson's disease who have motor complications, such as wearing off and dyskinesia. However, in some cases, the benefits of deep brain stimulation seem to diminish over time. Aim We aimed to investigate the reasons for hospitalization of Parkinson's disease patients who were previously implanted with subthalamic nucleus deep brain stimulation devices. We also aimed to investigate the efficacy of our management of problems related to deep brain stimulation and/or Parkinson's disease-related neurological symptoms. Methods A retrospective chart review was carried out on all hospitalized cases of patients who were previously implanted subthalamic nucleus deep brain stimulation at our hospital or other institutions. Results A total of 78 hospitalizations (47 patients) were identified. Of these, 34 hospitalizations (24 patients) were as a result of worsening of Parkinson's disease-related or stimulation-related problems. The reasons for 34 hospitalizations included wearing-off/on-off (n = 17), dyskinesia (n = 12), gait disturbance (n = 5), dysarthria (n = 4), camptocormia (n = 5), hallucination (n = 6) and other psychiatric problems (n = 10). Most of these problems were successfully managed by adjusting both medications and stimulation parameters (31 cases, 91.2%). No case was improved by only adjusting stimulation. The Unified Parkinson's Disease Rating Scale part III score improved by 24.5% (P = 0.02) by hospitalized management. Conclusion Appropriate management of medications and stimulation are most important for patients who have already undergone deep brain stimulation in order to maximize the benefits.

A girl aged 2 years 10 months suddenly went into a deep coma and demonstrated left hemiplegia. At birth, she had exhibited a left-sided facial port-wine stain typical of Sturge-Weber syndrome (SWS) and involving the V1 and V2 distributions of the trigeminal nerve. Computed tomography showed a right thalamic hemorrhage with acute hydrocephalus. Magnetic resonance imaging with Gd enhancement 8 months before the hemorrhage had shown a patent superior sagittal sinus (SSS) and deep venous system. Magnetic resonance imaging and MR angiography studies 2 months before the hemorrhage had revealed obstruction of the SSS and right internal cerebral vein (ICV). Given that a digital subtraction angiography study obtained after the hemorrhage did not show the SSS or right ICV, the authors assumed that impaired drainage was present in the deep venous system at that stage. The authors speculated that the patient's venous drainage pattern underwent compensatory changes because of the occluded SSS and deep venous collectors, shifting outflow through other cortical venous channels to nonoccluded dural sinuses. Sudden congestion (nearly total to total obstruction) of the ICV may have caused the thalamic hemorrhage in this case, which is the first reported instance of pediatric SWS with intracerebral hemorrhage and no other vascular lesion. Findings suggested that the appearance of major venous sinus occlusion in a child with SWS could be a warning sign of hemorrhage.

Background Highly integrated anatomic and functional interactions between the cerebrum and the cerebellum during development have been reported. In our previous study, we conducted a proteome analysis to identify the proteins present in the congenital noncommunicating hydrocephalus in the cerebellum. We found higher expression of high-mobility group box-1 protein (HMGB-1) in hydrocephalic H-Tx rats. Objective We studied the expression pattern of HMGB-1 in the cerebellum. Methods We studied congenital hydrocephalic H-Tx rats aged 1 day and 7 days along with age-matched nonhydrocephalic H-Tx and Sprague-Dawley rats as controls. Gene and protein expressions of HMGB-1 in the cerebellum were assayed by real-time polymerase chain reaction and Western blotting, respectively; furthermore, immunohistochemical analyses were performed by using HMGB-1 (indicator of apoptosis), single-stranded DNA; adhesion factor related to cell migration, HNK-1; and the Purkinje cell-specific antibody, calbindin. Results Cytoplasmic HMGB-1 expression observed in Purkinje cells in the 1-day-old hydrocephalic group was stronger than that in the nonhydrocephalic and Sprague-Dawley groups. Double fluorescent staining with single-stranded DNA confirmed that Purkinje cells were undergoing apoptosis. HNK-1 expression was lower in the Purkinje cell layer in the 7-day-old rats in the hydrocephalic group, and Purkinje cells were disrupted in comparison with the control groups. Morphological changes in the cerebellum were observed in the 7-day-old rats in the hydrocephalic group in comparison with the control groups. Conclusion Our results suggest that cerebellar neuronal cell damage in the early postnatal period may be related to the higher expression of HMGB-1 in the Purkinje cells.