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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.

Epileptic seizure is the common symptom associated with lipomas in the Sylvian fissure (Sylvian lipomas). Removal of these lipomas carries risks of hemorrhage and brain damage. We report a surgical strategy of not removing the lipoma in a case of intractable temporal lobe epilepsy associated with Sylvian lipoma. We performed anterior temporal lobectomy with preservation of the pia mater of the Sylvian fissure and achieved seizure freedom. Focal cortical dysplasia type 1 of the epileptic neocortex adjacent to the Sylvian lipoma was pathologically diagnosed. We recommend our surgical procedure in similar cases to avoid complications and achieve adequate seizure control.

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.

We assessed the diagnostic utility of the occurrence rate of high-frequency oscillations and modulation index (MI) from intraoperative electrocorticography (ioECoG) in determining the extent of epileptogenicity in mesial temporal lobe epilepsy (TLE) with hippocampal sclerosis (HS).We enrolled 17 patients who underwent selective amygdalohippocampectomy (SelAH) for TLE due to HS. We analyzed the occurrence rate of ripples (80-200 Hz) and fast ripples (200-300 Hz); and MI between ripples and 3-4 Hz (MIIn the poor seizure outcome group, an increase in the occurrence rate of ripples was seen in the hippocampus and LTL pre-SelAH and the LTL post-SelAH. The MIHigh occurrence rate of ripples and MI

Treatment for idiopathic normal pressure hydrocephalus (iNPH) continues to develop. Although ventriculoperitoneal shunt surgery has a long history and is one of the most established neurosurgeries, in the 1970s, the improvement rate of iNPH triad symptoms was poor and the risks related to shunt implantation were high. This led experts to question the surgical indication for iNPH and, over the next 20 years, cerebrospinal fluid (CSF) shunt surgery for iNPH fell out of favor and was rarely performed. However, the development of programmable-pressure shunt valve devices has reduced the major complications associated with the CSF drainage volume and appears to have increased shunt effectiveness. In addition, the development of support devices for the placement of ventricular catheters including preoperative virtual simulation and navigation systems has increased the certainty of ventriculoperitoneal shunt surgery. Secure shunt implantation is the most important prognostic indicator, but ensuring optimal initial valve pressure is also important. Since over-drainage is most likely to occur in the month after shunting, it is generally believed that a high initial setting of shunt valve pressure is the safest option. However, this does not always result in sufficient improvement of the symptoms in the early period after shunting. In fact, evidence suggests that setting the optimal valve pressure early after shunting may cause symptoms to improve earlier. This leads to improved quality of life and better long-term independent living expectations. However, in iNPH patients, the remaining symptoms may worsen again after several years, even when there is initial improvement due to setting the optimal valve pressure early after shunting. Because of the possibility of insufficient CSF drainage, the valve pressure should be reduced by one step (2–4 cmH2O) after 6 months to a year after shunting to maximize symptom improvement. After the valve pressure is reduced, a head CT scan is advised a month later.

BackgroundThe aim of this study was to evaluate the water diffusivity changes along the perivascular space after lumboperitoneal shunt (LPS) surgery in idiopathic normal pressure hydrocephalus.MethodsNine patients diagnosed with idiopathic normal pressure hydrocephalus (iNPH; three men and six women, mean age ± SD = 75.22 ± 5.12 years) according to the guidelines for iNPH in Japan were included in the study. Post-LPS surgery, six patients with iNPH who exhibited improvement in symptoms were defined as responder subjects, while three patients with iNPH who did not were defined as non-responder subjects. We calculated the mean analysis along the perivascular space (ALPS) index of the left and right hemispheres and compared the differences between pre- and post-LPS surgery mean ALPS indices in iNPH patients. In the responder or non-responder subjects, the mean ALPS indices in the pre- and post-operative iNPH groups were compared using Wilcoxon signed-rank tests. Next, correlation analyses between pre- and post-operation changes in the mean ALPS index and clinical characteristics were conducted.ResultsThe mean ALPS index of the post-operative iNPH group was significantly higher than that of the pre-operative iNPH group (p = 0.021). In responder subjects, the mean ALPS index of the post-operative iNPH group was significantly higher than that of the pre-operative iNPH group (p = 0.046). On the other hand, in the non-responder subjects, the mean ALPS index of the post-operative iNPH group was not significantly different compared to the pre-operative iNPH group (p = 0.285). The mean ALPS index change was not significantly correlated with changes in the Mini-Mental State Examination (MMSE) score (r = −0.218, p = 0.574), Frontal Assessment Battery (FAB) score (r = 0.185, p = 0.634), Trail Making Test A (TMTA) score (r = 0.250, p = 0.516), and Evans' index (r = 0.109, p = 0.780). In responder subjects, the mean ALPS index change was significantly correlated with Evans' index in pre-operative patients with iNPH (r = 0.841, p = 0.036).ConclusionThis study demonstrates the improved water diffusivity along perivascular space in patients with iNPH after LPS surgery. This could be indicative of glymphatic function recovery following LPS surgery.

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.

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 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.

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.

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.

Purpose We retrospectively evaluated the diagnostic value of 18 F-2-fluorodeoxy-d-glucose positron emission tomography (FDG-PET) with statistical analysis for the foci detection and predictive utility for postsurgical seizure outcome of patients with mesial temporal lobe epilepsy (mTLE). Method We evaluated 40 patients who were diagnosed mTLE and underwent selective amygdalohippocampectomy (SAH) or anterior temporal lobectomy (ATL) in our institute. Preoperative interictal FDG-PET with statistical analysis using three-dimensional stereotactic surface projection (3D-SSP) was detected with several clinical data including seizure semiology, MRI, scalp electroencephalography, surgical procedure with SAH or ATL and postsurgical outcome. The region of interest (ROI) was defined on ⿿Hippocampus & Amygdala⿿, ⿿Parahippocampal gyrus & Uncus⿿, ⿿T1 & T2⿿, and ⿿T3 & Fusiform gyrus⿿. We obtained the ratio of hypometabolism difference (RHD) by 3D-SSP, and evaluated the relation among hypometabolic extent, surgical outcome and surgical procedure. Result The RHD in each ROIs ipsilateral to operative side was significantly higher than that of contralateral side in good outcome group. Hypometabolism of ⿿Hippocampus & Amygdala⿿ was most reliable prognostic factor. Patients of discordant with presurgical examinations hardly showed obvious lateralized hypometabolism. Nevertheless, when they have significantly high RHD in mesial temporal lobe, good surgical outcome was expected. There was not significant difference of RHD distribution between SAH and ATL in good outcome group. Conclusion Significant hypometabolism in mesial temporal lobe on FDG-PET with 3D-SSP is useful to predict good surgical outcome for patients with mTLE, particularly in discordant patients with hypometabolism in mesial temporal structure. However, FDG-PET is not indicative of surgical procedure.

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.

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

We previously reported increase in leucine-rich α2-glycoprotein (LRG) concentration in cerebrospinal fluid is associated with cognitive decline in humans. To investigate relationship between LRG expression in the brain and memory impairment, we analyzed transgenic mice overexpressing LRG in the brain (LRG-Tg) focusing on hippocampus. Immunostaining and Western blotting revealed age-related increase in LRG expression in hippocampal neurons in 8-, 24-, and 48-week-old controls and LRG-Tg. Y-maze and Morris water maze tests indicated retained spatial memory in 8- and 24-week-old LRG-Tg, while deteriorated in 48-week-old LRG-Tg compared with age-matched controls. Field excitatory postsynaptic potentials declined with age in LRG-Tg compared with controls at 8, 24, and 48 weeks. Paired-pulse ratio decreased with age in LRG-Tg, while increased in controls. As a result, long-term potentiation was retained in 8- and 24-week-old LRG-Tg, whereas diminished in 48-week-old LRG-Tg compared with age-matched controls. Electron microscopy observations revealed fewer synaptic vesicles and junctions in LRG-Tg compared with age-matched controls, which became significant with age. Hippocampal LRG overexpression contributes to synaptic dysfunction, which leads to memory impairment with advance of age.

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.

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.

Summary Objective Some patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development. Methods Ten patients who were surgically treated using the posterior quadrantectomy (PQT) were enrolled in this study. Surgical outcome was analyzed as seizure-free or not at 2 years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (MDI) and psychomotor developmental index (PDI) in the Bayley Scales of Infant Development II preoperatively, and at 6 and 12 months after the PQT. Results Eight of 10 patients were seizure-free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI scores before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12 months after the PQT in seizure-free patients were 80.5 and 84.5, respectively (p

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.

There is a long history of surgical treatment for Parkinson disease (PD). After pioneering trials and errors, the current primary surgical treatment for PD is deep brain stimulation (DBS). DBS is a promising treatment option for patients with medically refractory PD. However, there are still many problems and controversies associated with DBS. In this review, we discuss current issues in DBS for PD, including patient selection, clinical outcomes, complications, target selection, long-term outcomes, management of axial symptoms, timing of surgery, surgical procedures, cost-effectiveness, and new technology.

Background Cognitive impairment is difficult to improve after shunt operation in patients with idiopathic normal pressure hydrocephalus (iNPH). This study aims to identify cerebrospinal fluid (CSF) biomarkers predictive of improvement in cognitive function. Methods This study was conducted between January 2008 and December 2010 on consecutive, unselected admissions to our program for the treatment of patients with clinically suspected iNPH. Lumbar CSF concentrations of total tau (Tau), tau phosphorylated at threonine 181 (p-tau), soluble amyloid precursor protein (sAPP), sAPPα, sAPPβ, and β-amyloid1-42 (Aβ42) were analyzed by ELISA. Results Concentrations of p-tau, sAPP, sAPPα, and sAPPβ were strong diagnostic biomarkers for distinguishing between iNPH and Alzheimer's disease (AD). sAPPα exhibited the highest accuracy in differentiating iNPH from patients with AD and normal controls, with an area under the curve value of 0.994. We examined the prognostic value of p-tau and sAPPα for cognition function after surgery. With a cutoff value of 198 ng/ml or less for sAPPα, sensitivity and specificity are 66.7% and 82.9%, respectively, whilst the Mini-Mental State Examination score at 6 months after surgery is expected to be 25 or more. Conclusion Our results show that sAPPα is a suitable biomarker for the diagnosis and prognosis of iNPH.

A 49-year-old man with Parkinson's disease developed the wearing-off and levodopa-induced dyskinesia with a dystonic component in the left upper limb. He decided to undergo deep brain stimulation. Asymmetric targets were chosen. For right side, globus pallidus interna was selected to improve the dystonia in his left upper extremity, and for left side, the subthalamic nucleus, which had the expected benefit of reducing the patient's medications, was selected. After the surgery, the levodopa-induced dystonia in his left arm was markedly improved, and the levodopa equivalent dose was reduced by 21.4%. Our case suggests that deep brain stimulation with a different target in the left and right hemisphere may be an effective therapeutic option for patients with asymmetric symptoms. This article is protected by copyright. All rights reserved.

A 64-year-old woman with Parkinson's disease who developed motor fluctuations, and both levodopa-induced and stimulation-induced dyskinesia, after long-term treatment of bilateral subthalamic nucleus (STN) deep brain stimulation (DBS), underwent implantation of additional globus pallidus internus (GPi) DBS leads. Although an appropriate DBS target should be chosen, “rescue” GPi DBS can synergistically work with pre-existing STN DBS for the treatment of dyskinesia, which might provide a “third honeymoon.”

Background Pathological gambling can develop in Parkinson’s disease (PD), and impairment of decision-making may play an important role in the mechanism. To assess acute effects of bilateral subthalamic nucleus deep brain stimulation (STN-DBS) on decision-making, patients with PD who were eligible for STN-DBS were evaluated using the Iowa Gambling Task (IGT). Methods The study participants were 16 patients with PD who underwent bilateral STN-DBS, and 16 age-matched control patients with PD. The participants performed the IGT pre-operatively and 2–4 weeks post-operatively with on- and off-stimulation. Participants’ one hundred card selections were divided into five blocks of 20 cards each. Results The total IGT score was not significantly different before surgery, on-stimulation or off-stimulation, but DBS patients tended to perform worse in the on-DBS session compared to off-DBS session ( P = 0.019) only in the last block of the task. The IGT score did not correlate with levodopa equivalent dose or performance on the measures of executive function, but did correlate with self-reported depression symptoms, and active contact of stimulation. Conclusion Bilateral STN-DBS may affect decision-making in acute post-operative stage.

Object: Cerebrospinal fluid (CSF) shunting can improve symptoms of elderly patients idiopathic normal pressure hydrocephalus (iNPH). However, adjunctive means for confirming the diagnosis remain unavailable. We have previously reported specific increase of leucine-rich alpha-2-glycoprotein (LRG) in iNPH CSF, and present study investigates its potential clinical applications. Methods: We performed CSF tap test (TT) on 90 patients and shunting in 52 patients (mean age 73.5 years), evaluating symptom improvement and higher cerebral functions-MMSE and Frontal Assessment Battery (FAB) before and twelve months after shunting. LRG and tau protein concentrations in TT CSF were simultaneously measured using ELIZA. Then we compared the predictive value of these concentrations with TT results regarding successful shunting outcomes. Findings: Positive combinations of TT and LRG concentrations of 67ng/mL or higher, gave 81.6% sensitivity and 78.6% specificity. Therefore we used LRG (67ng/mL) and tau (200pg/mL) cutoff values, LRG≥67ng/ml and tau

Holmes’ tremor is a rare symptom characterized by a complex combination of resting, postural and intention tremor. A 66-year-old right-handed man post-traumatically developed severe action tremor in his right hand, which had a component of the re-emergent type and predominantly involved proximal muscles. He also presented mild resting tremor in his right hand. As the response of levodopa/carbidopa and arotinolol was limited, he started zonisamide, which resulted in dramatic improvement. The Fahn–Tolosa–Marin Tremor Rating Scale motor score improved from 52 points to 24 points. In conclusion, zonisamide could be a therapeutic option for post-traumatic Holmes’ tremor. Further prospective studies are required.

Objectives Pain is one of the common symptoms in patients with Parkinson's disease (PD), with a prevalence of approximately 40–85%. These symptoms affect the quality of life of PD patients. We evaluated the effect of spinal cord stimulation (SCS) to chronic pain and motor symptoms of PD. Materials and Methods Three PD patients were treated with SCS to relieve their persistent and intractable pain. One patient had failed back surgery syndrome and the other two had lumbar canal stenosis. All patients had a stooped posture and pain that was resistant to analgesics. We evaluated motor symptoms using Hoehn and Yahr scale and the Unified Parkinson's Disease Rating Scale (UPDRS), and evaluated pain using visual analog scale and widespread pain index, before and after SCS. Results After SCS insertion, chronic pain in the patients decreased in both the lower back and limbs. Moreover, SCS ameliorated the symptoms of PD. One-year follow-up after SCS showed that UPDRS part III scores, rigidity, and tremor were improved without large alterations in levodopa dosage. Dementia and activities of daily living did not improve after SCS. Discussion and Conclusion Our results indicate that SCS may be a treatment option for both motor symptoms and chronic pain in PD, especially in cases complicated with lumbar canal stenosis or disc herniation. Further studies are needed to evaluate the efficacy of SCS in PD patients.

Background Accidental opening of the frontal sinuses during craniotomy can lead to various postoperative complications. We report a simple and reliable reconstruction method using no exogenous or autogenous material obtained from another site. Methods This method involves packing a small wedge-shaped piece of bone obtained from the bone flap of the craniotomy into the nasal recess of the exposed sinus. The large opened frontal sinus is reconstructed as a new minimized frontal sinus with mucous membrane and bony roof in the nasal recess. Results Eleven patients with large frontal sinus opening during craniotomy (7 cases of bifrontal craniotomy for the basal interhemispheric approach) underwent frontal sinus reconstruction by packing of a small bone piece into the nasal recess. No patients suffered postoperative complications related to the opened frontal sinus such as pneumocephalus or cerebrospinal fluid rhinorrhea. Conclusion Packing of a small bone piece from the bone flap is a quick and reliable method to reconstruct the frontal sinus opened during craniotomy.

Among the various disorders manifesting dementia, gait disturbance, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. After the publication of the first edition of the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus in 2004 (the English version was published in 2008), clinical awareness of iNPH has risen dramatically, and the number of shunt surgeries has increased rapidly across Japan. Clinical and basic research on iNPH has increased significantly, and more high-level evidence has since been generated. The second edition of the Japanese Guidelines was thus published in July 2011, to provide a series of timely evidence-based recommendations related to iNPH. The revision of the Guidelines has been undertaken by a multidisciplinary expert working group of the Japanese Society of Normal Pressure Hydrocephalus in conjunction with the Japanese Ministry of Health, Labour and Welfare research project on "Studies on the epidemiology, pathophysiology, and treatment of normal pressure hydrocephalus." This English version of the second edition of the Guidelines was made to share these ideas with the international community and to promote international research on iNPH.

Objective Cerebrospinal fluid (CSF) shunting can improve symptoms of elderly patients' idiopathic normal pressure hydrocephalus (iNPH). However, adjunctive means for confirming the diagnosis remain unavailable. We have previously reported the specific increase of leucine-rich alpha-2-glycoprotein (LRG) in iNPH CSF, and the present study investigates its potential clinical applications. Methods We performed CSF tap test (TT) on 90 patients (mean age 73.4 years) and shunting in 52 patients (mean age 73.5 years), evaluating symptom improvement and higher cerebral functions—mini-mental state examination (MMSE) and Frontal Assessment Battery (FAB) before and 12 months after shunting. LRG and tau protein concentrations in TT CSF were simultaneously measured using enzyme-linked immunosorbent assay. We then compared the predictive value of these concentrations with TT results regarding successful shunting outcomes. Results Positive combinations of TT and LRG concentrations of 67 ng/ml or higher, gave 81.6% sensitivity and 78.6% specificity. Therefore we used LRG (67 ng/ml) and tau (200 pg/ml) cut-off values, dividing patients into four groups. In group A (LRG ≥ 67 ng/ml and tau

The mechanism of epilepsy in Sturge–Weber syndrome (SWS) has not been elucidated, since it is difficult to detect epileptic discharges on scalp EEG records. To determine the electrophysiological characteristics of SWS, we evaluated epileptic discharges using intracranial electrocorticography (ECoG). We report a 1-year and 3-month-old boy diagnosed with SWS whose psychomotor development was impaired despite good control of his seizures with several antiepileptic drugs (AEDs). Prolonged video ECoG monitoring was performed to determine whether subclinical seizures had induced his developmental delay. Frequent seizures were detected as motionless staring and respiratory distress. His seizures originated from multiple areas along the Sylvian fissure, propagated very slowly, and lasted for 10 to 30 min. Left hemispherotomy was performed, and he was confirmed to be completely seizure-free after surgery. Our findings indicate that long-lasting and barely discernible seizures may continue in patients with SWS who appear to have good control of epilepsy with AEDs.

BACKGROUND Endodermal cysts usually develop in the subdural space in the anterior spinal cord and rarely occur inside the cranium. Most intracranial endodermal cysts develop in the posterior fossa. We report the first case of an endodermal cyst in the quadrigeminal cistern. CASE DESCRIPTION The patient was a 71-year-old man who suffered from gait disturbance for 6 months. Although head computed tomography (CT) scanning 4 years previously did not show any cystic lesion, CT and magnetic resonance imaging (MRI) on admission showed a cystic lesion extending from the quadrigeminal cistern to the right ambient cistern. The cyst was subtotally removed via a suboccipital transtentorial approach. The cyst wall consisted of a layer of columnar epithelium and connective tissue. Based on the results of immunostaining, it was diagnosed as an endodermal cyst. CONCLUSIONS It is possible that the increase of secretion from the cells lining the cyst may have caused a difference in osmotic pressure between the cerebrospinal fluid and the cyst contents, leading to rapid enlargement of the cyst. An endodermal cyst should be removed as completely as possible because its cells have the ability to grow and produce secretions.

Background Epileptic myoclonus is generally treated by valproate monotherapy, and this therapy has confirmed efficacy. However, almost 30% of patients with juvenile myoclonic epilepsy (JME) are valproate-resistant. Case Description A 23-year-old man with a diagnosis of JME had resistance to multiple antiepileptic drugs (AEDs). Stepwise synchronization of bilateral spikes on electroencephalography (EEG) was found shortly before the clinical myoclonus. The anterior two thirds of the corpus callosum were divided. Desynchronization of spikes by the corpus callosotomy arrested the patient's myoclonus. Conclusions Interhemispheric recruitment of epileptic spikes through the corpus callosum may induce synchronization of spikes and myoclonus.