Early Hydrocephalus Research Articles

CFAP43 modulates ciliary beating in mouse and Xenopus

Malfunctions of motile cilia cause a variety of developmental defects and diseases in humans and animal model organisms. Defects include impaired mucociliary clearance of the airways, sperm immotility, hydrocephalus and organ laterality. Here, we characterize the evolutionary conserved Cfap43 gene by loss-of-function experiments in the mouse and the frog Xenopus laevis. Cfap43 is expressed in tissues carrying motile cilia and acts as a target gene of the transcription factor FOXJ1, which is essential for the induction of motile ciliogenesis. We show that CFAP43, a protein of unknown biochemical function, localizes to the ciliary axoneme. CFAP43 is involved in the regulation of the beating frequency of tracheal cilia and loss of CFAP43 causes severe mucus accumulation in the nasal cavity. Likewise, morphant and crispant frog embryos revealed impaired function of motile cilia of the larval epidermis, a model for airway mucociliary epithelia. CFAP43 participates in the formation of flagellar axonemes during spermatogenesis as mice mutant for Cfap43 display male infertility, consistent with observations in male sterile patients. In addition, mice mutant for Cfap43 display early onset hydrocephalus. Together, these results confirm the role of CFAP43 in the male reproductive tract and pinpoint additional functions in airway epithelia mucus clearance and brain development.

Does aneurysm side influence the infarction side and patients´ outcome after subarachnoid hemorrhage?

BackgroundThe prognostic factors and outcome of aneurysms appear to be dependent on its locations. Therefore, we compared left- and right- sided aneurysms in patients with aneurysmal subarachnoid hemorrhage (SAH) in terms of differences in outcome and prognostic factors.MethodsPatients with SAH were entered into a prospectively collected database. A total of 509 patients with aneurysmal subarachnoid hemorrhage were retrospectively selected and stratified in two groups depending on side of ruptured aneurysm (right n = 284 vs. left n = 225). Midline aneurysms of the basilar and anterior communicating arteries were excluded from the analysis. Outcomes were assessed using the modified Rankin Scale (mRS; favorable (mRS 0–2) vs. unfavorable (mRS 3–6)) six months after SAH.ResultsWe did not identify any differences in outcome depending on left- and right-sided ruptured aneurysms. In both groups, the significant negative predictive factors included clinical admission status (WFNS IV+V), Fisher 3- bleeding pattern in CT, the occurrence of delayed cerebral ischemia (DCI), early hydrocephalus and later shunt-dependence. The side of the ruptured aneurysm does not seem to influence patients´ outcome. Interestingly, the aneurysm side predicts the side of infarction, with a significant influence on patients´ outcome in case of left-sided infarctions. In addition, the in multivariate analysis side of aneurysm was an independent predictor for the side of cerebral infarctions.ConclusionThe side of the ruptured aneurysms (right or left) did not influence patients’ outcome. However, the aneurysm-side predicts the side of delayed infarctions and outcome appear to be worse in patients with left-sided infarctions.

Are primary and secondary types of brain anomalies exclusive factors affecting the attention networks in individuals with spina bifida?

Individuals with spina bifida myelomeningocele (SBM) frequently exhibit cognitive impairments on tasks mediated by brain regions involved in the posterior attention network. Although such deficits have been historically assumed to result from primary and secondary brain insults, there is a dearth of literature regarding whether sequential versus simultaneous surgical closure of neural folds and surgical shunt placement affect neuropsychological function and brain structure of attention networks that have been widely studied in individuals with SBM. The current study addressed these gaps in a large cohort of children and adults with SBM. White matter pathways and regional brain volumes of anterior and posterior attention networks were quantified through probabilistic tractography and automated segmentation, respectively. The Child Attention Network Test measured behavioral components of posterior and anterior attention networks. Sequential operations were associated with reduced orienting accuracy and smaller left superior parietal and dorsolateral prefrontal cortex volumes compared to simultaneous operations, controlling for a number of shunt revisions and age. Greater number of shunt revisions was associated with higher radial diffusivity values in the parietal tectocortical pathway. Older participants had greater accuracy and faster conflict resolution performance compared to younger participants, across operation type and number of shunt revisions. Shunt treatment and revision history related to brain structure and functions associated with the posterior attention network. Neurosurgical history also differentiated the harmful effects of early hydrocephalus on brain structure of the posterior from the anterior attention networks in SBM. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

Impaired neural differentiation and glymphatic CSF flow in the Ccdc39 rat model of neonatal hydrocephalus: genetic interaction with L1cam.

ABSTRACTNeonatal hydrocephalus affects about one child per 1000 births and is a major congenital brain abnormality. We previously discovered a gene mutation within the coiled-coil domain-containing 39 (Ccdc39) gene, which causes the progressive hydrocephalus (prh) phenotype in mice due to lack of ependymal-cilia-mediated cerebrospinal fluid (CSF) flow. In this study, we used CRISPR/Cas9 to introduce the Ccdc39 gene mutation into rats, which are more suitable for imaging and surgical experiments. The Ccdc39prh/prh mutants exhibited mild ventriculomegaly at postnatal day (P)5 that progressed into severe hydrocephalus by P11 (P<0.001). After P11, macrophage and neutrophil invasion along with subarachnoid hemorrhage were observed in mutant brains showing reduced neurofilament density, hypomyelination and increased cell death signals compared with wild-type brains. Significantly more macrophages entered the brain parenchyma at P5 before hemorrhaging was noted and increased expression of a pro-inflammatory factor (monocyte chemoattractant protein-1) was found in the cortical neural and endothelial cells in the mutant brains at P11. Glymphatic-mediated CSF circulation was progressively impaired along the middle cerebral artery from P11 as mutants developed severe hydrocephalus (P<0.001). In addition, Ccdc39prh/prh mutants with L1 cell adhesion molecule (L1cam) gene mutation, which causes X-linked human congenital hydrocephalus, showed an accelerated early hydrocephalus phenotype (P<0.05-0.01). Our findings in Ccdc39prh/prh mutant rats demonstrate a possible causal role of neuroinflammation in neonatal hydrocephalus development, which involves impaired cortical development and glymphatic CSF flow. Improved understanding of inflammatory responses and the glymphatic system in neonatal hydrocephalus could lead to new therapeutic strategies for this condition.This article has an associated First Person interview with the joint first authors of the paper.

Vitamin K Antagonist (Phenprocoumon) and Subarachnoid Hemorrhage: A Single-Center, Matched-Pair Analysis.

Demographic changes are leading to an aging society with a growing number of patients relying on anticoagulation, and vitamin K antagonists (VKA) are still widely used. As mortality and functional outcomes are worse in case of VKA-associated hemorrhagic stroke, phenprocoumon treatment seems to be a negative prognostic factor in case of subarachnoid hemorrhage (SAH). The purpose of this study was to analyze whether phenprocoumon treatment does worsen the outcome after non-traumatic SAH. All patients treated for non-traumatic SAH between January 2007 and December 2016 in our institution were retrospectively analyzed. After exclusion of patients with anticoagulant or antiplatelet treatment other than phenprocoumon, we analyzed 1040 patients. Thirty-three patients (3%) of those were treated with continuous phenprocoumon. In total, 132 out of all 1007 patients without anticoagulant treatment of the remaining patients were matched as control group (ratio = 1:4). Patients with phenprocoumon treatment were significantly older (66.5years vs. 53.9years; p < .0001), and admission status was significantly more often poor (66.7% vs. 41.8%, p = .007) compared to all patients without anticoagulant treatment. Further, bleeding pattern and rates of early hydrocephalus did not differ. Matched-pair analysis revealed a significant higher rate of angio-negative SAH in the study group (p = .001). Overall rates of hemorrhagic or thromboembolic complications did not differ (21.4% vs. 18.8%; NS) but were more often fatal, and 30-day mortality rate was significantly higher in the phenprocoumon group than in patients of the matched-pair control group (33% vs. 24%; p < .001). 30% of the phenprocoumon group and 37% of the matched-pair control group reached favorable outcome. However, poor outcome was strong associated with the reason for phenprocoumon treatment. Patients with phenprocoumon treatment at the time of SAH are significantly older, admission status is worse, and 30-day mortality rates are significantly higher compared to patients without anticoagulant treatment. However, outcome at 6months did not differ to the matched-pair control group but seems to be strongly associated with the underlying cardiovascular disease. Treatment of these patients is challenging and should be performed on an interdisciplinary base in each individual case. Careful decision-making regarding discontinuation and bridging of anticoagulation and close observation is mandatory.

Care management and contemporary challenges in spina bifida: a practice preference survey of the American Society of Pediatric Neurosurgeons.

Neurosurgical management preferences related to myelomeningocele (MMC) care demonstrate significant variability. The authors sought to evaluate variability in practice patterns across a group of senior pediatric neurosurgeons. The purpose of this study was to identify the extent of variability and of consensus with regard to neurosurgical management of MMC and associated hydrocephalus, Chiari II malformation, and tethered spinal cord. A 43-question survey was distributed electronically to the members of the American Society of Pediatric Neurosurgeons (ASPN). The survey covered domains such as clinic case volume, newborn management, hydrocephalus management, transition to adulthood, clinical indications for shunt revision, Chiari II malformation decompression (C2MD), and tethered cord release (TCR). Ninety responses were received from 200 active ASPN members, for an overall response rate of 45%. The majority (58%) of respondents closed 5-15 new cases of open MMC per year. Nearly all (98%) respondents perform back closure within 48 hours of birth, with the majority imbricating the placode and striving for a 3- to 4-layer closure. The most consistent indications for surgical intervention in early hydrocephalus were CSF leak from the back (92%), progressive ventricular enlargement (89%), and brainstem symptoms, including apnea/bradycardia (81%), stridor (81%), and dysphagia (81%). Eighty percent of respondents indicated that spina bifida care is delivered through multidisciplinary clinics, with neurosurgery, orthopedic surgery, urology, physical therapy, and social work as the most common disciplines included. One-third of clinics see both pediatric and adult patients, one-third offer a formal transition program to adult care, and one-third have no transition program. The vast majority of respondents offer prenatal counseling (95%), referral for in utero closure (66%), and endoscopic third ventriculostomy/choroid plexus cauterization (72%). Respondents were more willing to perform shunt revision for symptoms alone than for image changes alone. An asymptomatic broken shunt without ventricular enlargement produced responses evenly divided between observation, intervention, and further investigation. Operative shunt exploration was always performed before C2MD by 56% of respondents and performed sometimes by 40% of respondents. Symptoms of brainstem dysfunction were the strongest clinical triggers reported for C2MD, while declines in urinary continence, leg strength or sensation, or ambulation were the most consistent thresholds for TCR. Significant disparities exist surrounding key areas of decision making regarding treatment for patients with MMC, though there are central areas of agreement among ASPN members. Additionally, there is significant variation in the clinical management of chronic hydrocephalus, C2MD, and TCR, underscoring the need for further research into these specific areas.

Postoperative intraventricular blood: a new modifiable risk factor for early postoperative symptomatic hydrocephalus in children with posterior fossa tumors.

To analyze factors associated with the development of early symptomatic hydrocephalus following posterior fossa tumor (PFT) surgery in children. In this retrospective study, data from 148 children (age < 18years) who underwent primary resection of their PFTs without preoperative permanent CSF diversion procedures were collected. The incidence of symptomatic hydrocephalus within 30days of tumor resection was studied and its association with various demographic, tumor-related, and surgery-related risk factors was analyzed. At presentation, 131 (89%) of the 148 patients had symptomatic hydrocephalus. There were 99 males and 49 females (mean age 8.7years; range 1 to 17years). Postoperatively, 14 (9.4%) patients required shunt placement for symptomatic hydrocephalus. The indications for shunt surgery were persistent symptoms of raised intracranial pressure (n = 6, 43%), CSF leak from the wound (n = 7, 50%), and tense pseudomeningocele (n = 1, 7%). On multivariate analysis, age < 6years (OR 5.9, 95% CI 1.6-22.6, p = 0.009) and the presence of intraventricular blood (IVB) on postoperative CT (OR 6.4, 95% CI 1.7-23.7, p = 0.006) were independent risk factors for developing symptomatic hydrocephalus. The incidence of postoperative symptomatic hydrocephalus in our series (9.4%) is lower than that reported in most previous studies. Age < 6years and the presence of postoperative IVB were independent risk factors for developing symptomatic hydrocephalus. Of these, postoperative IVB is probably the only modifiable risk factor.

Predicting role of disturbance coefficient in post brain traumatic hydrocephalus

Objective To evaluate the predicting value of disturbance coefficient (DC) of noninvasive brain edema monitoring in patients with secondary hydrocephalus after traumatic brain injury. Methods Non-invasive brain edema monitoring technology was used clinically to perform dynamic monitor of 84 patients with traumatic brain injury admitted to Department of Neurosurgery, Nanfang Hospital, Southern Medical University from December 2016 to January 2018. Among them, 17 had hydrocephalus (hydrocephalus group) and 67 did not (control group). The patient’s age, gender, Glasgow coma scale at admission, brain CT status, DC and hydrocephalus situation were compared between the 2 groups. Results Hydrocephalus occurred in 17 patients and 8 of them had normal pressure hydrocephalus. The median DC of patients with hydrocephalus was decreased by 35.2(25.7-48.1) and that of patients with no hydrocephalus was decreased by 8.3(5.2-11.1). The difference was statistically significant (P=0.008). The DC decrease of more than 18.0 (sensitivity: 92.5%; specificity: 88.2%) indicated the occurrence of hydrocephalus. Conclusions The decrease of DC in non-invasive brain edema monitoring could be used to predict the occurrence of early hydrocephalus. Higher continuous decrease of DC was related to greater probability of occurrence of intracranial hydrocephalus. Key words: Craniocerebral trauma; Hydrocephalus; Disturbance coefficient

Cerebellar Hippocampal and Basal Nuclei Transient Edema with Restricted diffusion (CHANTER) Syndrome

BackgroundAbnormal restricted diffusion on magnetic resonance imaging is often associated with ischemic stroke or anoxic injury, but other conditions can present similarly. We present six cases of an unusual but consistent pattern of restricted diffusion in bilateral hippocampi and cerebellar cortices. This pattern of injury is distinct from typical imaging findings in ischemic, anoxic, or toxic injury, suggesting it may represent an under-recognized clinicoradiographic syndrome. Despite initial presentation with stupor or coma in the context of obstructive hydrocephalus, patients may have acceptable outcomes if offered early intervention.MethodsWe identified an ad hoc series of patients at our two institutions between years 2014 and 2017 who presented to the neurocritical care unit with severe, otherwise unexplained cerebellar edema and retrospectively identified several commonalities in history, presentation, and imaging.ResultsBetween two institutions, we identified six patients—ages 33–59 years, four male—with similar presentations of decreased level of consciousness in the context of intoxicant exposure, with acute cytotoxic edema of the cerebellar cortex, hippocampi, and aspects of the basal nuclei. All patients presented with severe cerebellar edema which led to obstructive hydrocephalus requiring aggressive medical and/or surgical management. The five patients who survived to discharge demonstrated variable degrees of physical and memory impairment on discharge and at follow-up.ConclusionsWe present findings of a potentially novel syndrome involving a distinct pattern of cerebellar and hippocampal restricted diffusion, with imaging and clinical characteristics distinct from ischemic stroke, hypoxic injury, and known toxidromes and leukoencephalopathies. Given the potential for favorable outcome despite early obstructive hydrocephalus, early identification and treatment of this syndrome are critical.

Is External Ventricular Drainage Life-Saving in Cerebellar Hemorrhages?

Background: The treatment of cerebellar hemorrhage (CH) may be different surgery or conservative according to the hematoma volume, compression of vital structures or hydrocephalus existence. In the present study, the authors investigated the risk factors, the indications and the situation of external ventricular drainage (EVD) on the treatment line. Methods: 63 pure cerebellar hemorrhage patients were enrolled in the study. 36 cases underwent surgery; the other 27 were received conservative treatment. 15 and 13 cases received EVD in both groups. Hospital stay and mortality rates were investigated. Results: 4 cases in the conservative group underwent surgery secondary to treatment failure. Both of the groups had equal rates of morbidity and mortality. On the other hand, the group that received surgical intervention had shorter median hospital stay. The EVD does not seem to be life-saving at first but it gives time for preparing for surgery. Conclusions: We found that CH was strongly associated with early hydrocephalus and mortality. The early diagnosis and surgical evacuation of the mass are mandatory and life-saving if hematoma is larger than 10 ml. The EVD may not being a life-saving instrument but majorly it may be a time earning device if acute hydrocephalus present.

Comparison of carotid and basilar bifurcation aneurysms versus non-T-angled bifurcations: the geometry is associated with the outcome.

Patients with ruptured aneurysms of carotid bifurcation artery seem to suffer less often from cerebral vasospasm and early brain injury and have a better clinical outcome. Aim of our study was to identify differences in clinical course and outcome in aneurysms of terminus segments (carotid bifurcation artery and basilar tip) compared to aneurysms of other aneurysm locations except carotid bifurcation artery and basilar tip. Patients with SAH were entered into a prospectively collected database (1999 to June 2014). A total of 471 patients ('T-shaped' aneurysms n = 63, 'non-T-shaped' aneurysms n = 408) were selected. Outcome was assessed by modified Rankin Scale (mRS) 6months after SAH. Mean age was 53.75years. Statistically, analysis showed a significant better outcome in 'T-shaped' aneurysms (p = 0.0001) and a significant lower mortality rate (p = 0.02) despite higher rates of Fisher 3 bleeding pattern and CVS. In 'T-shaped' aneurysms, no prognostic factors for outcome could be detected. In 'non-T-shaped' aneurysms admission status (p < 0.0001), early hydrocephalus (p < 0.0001), shunt-dependence (p = 0.001), and the occurrence of severe CVS (p = 0.01) statistically were factors influencing patients' outcome. Multivariate analysis showed 'non-T-shaped' aneurysms itself as independent prognostic factor for patients' outcome. Despite same rate of poor admission status, early hydrocephalus and shunt dependence 'T-shaped' aneurysms have a highly significantly better. Pathophysiological mechanism actually is not understood. Further studies are necessary to identify, which factors lead to the decreased outcome in "non-T-shaped"- aneurysms.

The Clinical Value of d-Dimer Level in Patients with Nonaneurysmal Subarachnoid Hemorrhage.

More nonaneurysmal subarachnoid hemorrhage (NaSAH) are found in clinical practice. However, the precise mechanisms in which d-dimer level is associated with clinical condition in patients with NaSAH remain unclear. But even more, the data assessing the risk of clinical course in the patients with NaSAH are scarce. Our objective was to investigate whether d-dimer levels correlated with complication and outcome in patients with NaSAH. Between February 2013 and May 2017, 92 patients suffering from NaSAH were treated in our hospital. Patient characteristics, radiologic features, laboratory findings, complications, and outcomes were analyzed retrospectively. Patients were stratified into the perimesencephalic (PM) subarachnoid hemorrhage (SAH) group and the nonperimesencephalic (NPM) SAH group according to the bleeding type, and the NPM-SAH group was further divided into 2 subgroups into the NPM-elevated group and the NPM-normal group based on the value of the d-dimer. After statistical analysis, the NPM-SAH and PM-SAH groups were compared; the same was true for the NPM-elevated group and the NPM-normal group. The rate of complications in the NPM-SAH group was higher than in the PM-SAH group, including early hydrocephalus, delayed cerebral ischemia, clinical vasospasm, pneumonia, and hyponatremia. Our results showed that d-dimer levels in patients with NPM-SAH were more elevated than in those with PM-SAH. Further analysis of subgroups demonstrated that patients with elevated d-dimer levels had a higher incidence of complications than those with normal d-dimer levels, especially the risks of shunt-dependent hydrocephalus and pneumonia. In addition, disability, and even death, could be seen in patients with higher levels of d-dimer, but the long-term outcomes were not particularly obvious between these groups. Elevated d-dimer levels on admission were significantly associated with complication in patients with NPM-SAH. In addition to conventional radiologic diagnosis, d-dimer levels can increase the ability of a rapid differential diagnosis between NPM-SAH and PM-SAH.

Early hydrocephalus in Listeria meningitis: Case report and review of the literature.

Listeria monocytogenes is a gram-positive bacterium with a predilection to infect the central nervous system, often affecting immunocompromised or elderly patients. The most common manifestations are meningitis and rhomboencephalitis. We report two cases of Listeria meningitis complicated by acute hydrocephalus several days after presentation and we further review the literature of similar cases. We conclude that acute hydrocephalus is a significant, not often recognized, complication of Listeria meningitis, usually occurring several days from onset when coverage did not include anti-Listeria antimicrobials. In high risk patients, meningitis combined with acute hydrocephalus is suggestive of LM infection.

A mutation in Ccdc39 causes neonatal hydrocephalus with abnormal motile cilia development in mice.

Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common congenital brain abnormalities. However, little is known about the molecular and cellular mechanisms regulating CSF flow in the developing brain. Through whole-genome sequencing analysis, we report that a homozygous splice site mutation in coiled-coil domain containing 39 (Ccdc39) is responsible for early postnatal hydrocephalus in the progressive hydrocephalus (prh) mouse mutant. Ccdc39 is selectively expressed in embryonic choroid plexus and ependymal cells on the medial wall of the forebrain ventricle, and the protein is localized to the axoneme of motile cilia. The Ccdc39prh/prh ependymal cells develop shorter cilia with disorganized microtubules lacking the axonemal inner arm dynein. Using high-speed video microscopy, we show that an orchestrated ependymal ciliary beating pattern controls unidirectional CSF flow on the ventricular surface, which generates bulk CSF flow in the developing brain. Collectively, our data provide the first evidence for involvement of Ccdc39 in hydrocephalus and suggest that the proper development of medial wall ependymal cilia is crucial for normal mouse brain development.

Neurocutaneous Melanosis with Bilateral Temporal Lobe Lesions without Leptomeningeal Enhancement: A Distinct Entity or Subtype.

Neurocutaneous melanosis (NCM) is a rare congenital disorder. Most of the cases described in literature for this entity have involvement of the leptomeninges and other structures of brain such as brain stem, temporal lobes, and spinal meninges and no involvement of leptomeninges and presence of lesions in bilateral temporal lobes. NCM without the involvement of leptomeninges should be considered a distinct entity as the prognosis is favorable as compared to cases with leptomeningeal involvement who develop early hydrocephalus and multiple cranial nerve palsies.

Myelomeningocele Repair: Surgical Management Based on a 30-Year Experience.

Myelomeningocele (MMC) is a rare but severe spinal defect resulting from a failed neurulation process. Surgical repair is a delicate procedure that needs accurate neuroanatomical knowledge and adequate surgical planning. The authors report on the main problems of the surgical management of this type of dysraphism. The technique used for reconstruction, and the management strategies, are analyzed according to the authors' experience with more than 600 cases, in comparison with the relevant literature. Tip and tricks of more than 30 years of MMC surgical management are reported, with special mention of the timing of surgery, the management of early hydrocephalus, and the principles of the repair, from the dissection to the closure of five separate layers (arachnoid, dura, muscular fascia, subcutaneous layer, skin). Accuracy and attention to the reconstruction are crucial for achieving good results and avoiding perioperative complications.

Evaluation of Late Onset Congenital Aqueductal Stenosis Hydrocephalus

Aqueductal stenosis is responsible for 20% of cases of hydrocephalus. Its incidence ranges from 0.5 to 1.0 in 1,000 births, with a recurrence risk in siblings of 1.0% to 4.5%. The onset of symptoms is usually insidious and can occur at any time from birth to adulthood. Associated malformations in neighboring structures are common The presence of these malformations helps to explain the intellectual, cognitive, and motor handicaps even after the hydrocephalus is compensated by shunting. Aqueductal obstruction constitutes three percent of adult hydrocephalus (1). Abstract Early onset hydrocephalus due to congenital aqueductal stenosis is well known and fully studied. However, late onset one is not so. The present study is a trial to give spotlight on the late onset type which may be clinically confusing with other syndromes leading sometimes to mismanagement. Our study included 25 patients with late onset congenital aqueductal stenosis hydrocephalus. They verified into 18 males (72%) and 7 females (28%). Their ages ranged from 5 years to 64 years. They presented with headache in 19 cases (76%), visual disturbance in 11 cases (44%), gait disturbance in 8 cases (32%) and disturbed level of consciousness in 4 cases (16%). Ventriculoperitoneal shunts were done for all cases with improvement in 20 cases (80%), no improvement in 3 cases (12%) and deterioration in 2 cases (8%). Post-shunt complications occurred in 9 cases (32%) and include 6 cases with subdural hematoma (24%), 2 cases with infection (8%) and one case with shunt obstruction (4%). Late onset aqueductal stenosis hydrocephalus is not uncommon cause of hydrocephalus in our locality. Proper diagnosis and management of such patients depended on the well manifest clinical data confirmed with the neuroimaging studies became easy.

Aneurysm Location as a Prognostic Outcome Factor After Subarachnoid Hemorrhage From Internal Carotid Artery Aneurysms and Potential Impact for Further Experimental Subarachnoid Hemorrhage Models

Prognostic factors of favorable and unfavorable clinical outcome after aneurysmal subarachnoid hemorrhage (SAH) are still not completely known. We retrospectively analyzed the aneurysm location as a factor for patients' outcome after aneurysmal SAH. We retrospectively selected patients from our prospectively collected database with aneurysm at the carotid bifurcation artery (n= 23) and posterior communicating artery (n= 170). Outcome was assessed using the modified Rankin Scale (mRS) (favorable [mRS score, 0-2] vs. unfavorable [mRS score, 3-6]) 6 months after SAH. A good clinical admission status (World Federation of Neurological Surgery grade I-III) has a significant influence on mRS score after SAH. In univariate analysis, advanced age, Fisher grade 3, early hydrocephalus, severity of cerebral vasospasm (CVS), longer stay on the intensive care unit, and posterior communicating artery aneurysms were associated with an unfavorable outcome. Multivariate analysis showed 4 prognostic factors for a favorable outcome: good admission status (odds ratio [OR], 10.8); aneurysms of carotid bifurcation artery (OR, 4.3); absence of mild or severe CVS (OR, 3.4); and patients age less than 55 years (OR, 2.1). Despite the usual prognostic factors (good admission status, younger age, absence of CVS) for a favorable outcome after SAH, the aneurysm location (carotidbifurcation artery) itself seems to be a prognostic factor. Also, aneurysms of the carotid bifurcation artery showed less occurrence of an early hydrocephalus, which is an indicator for the presence of an early brain injury. According to these results, we question if experimental animal models (especially the endovascular model using the perforation of the carotid bifurcation artery) have to be re-evaluated.

Early Idiopathic Normal Pressure Hydrocephalus Patients With Neuropsychological Impairment Are Associated With Increased Fractional Anisotropy in the Anterior Thalamic Nucleus.

In this study, we aimed to investigate the reactive changes in diffusion tensor imaging (DTI)-derived diffusion metrics of the anterior thalamic nucleus (AN), a relaying center for the Papez circuit, in early idiopathic normal pressure hydrocephalus (iNPH) patients with memory impairment, as well as its correlation with the patients’ neuropsychological performances. In total, 28 probable iNPH patients with symptom onset within 1 year and 17 control subjects were prospectively recruited between 2010 and 2013 for this institutional review board-approved study. Imaging studies including DTI and a neuropsychological assessment battery were performed in all subjects. Diffusion metrics were measured from the region of the AN using tract-deterministic seeding method by reconstructing the mammillo–thalamo–cingulate connections within the Papez circuit. Differences in diffusion metrics and memory assessment scores between the patient and control group were examined via the Mann–Whitney U test. Spearman correlation analyses were performed to examine associations between diffusion metrics of AN and neuropsychological tests within the patient group. We discovered that early iNPH patients exhibited marked elevations in fractional anisotropy, pure diffusion anisotropy, and axial diffusivity (all P < 0.01), as well as lower neuropsychological test scores including verbal and nonverbal memory (all P < 0.05) compared with normal control. Spearman rank correlation analyses did not disclose significant correlations between AN diffusion metrics and neuropsychological test scores in the patient group, whereas ranked scatter plots clearly demonstrated a dichotic sample distribution between patient and control samples. In summary, our study highlighted the potential compensatory role of the AN by increasing thalamocortical connectivity within the Papez circuit because memory function declines in early iNPH when early shunt treatment may potentially reverse the memory deficits.

Leukoencephalopathy with Vanishing White Matter (VWM) Presenting with Early Myoclonic Encephalopathy (EME) and Rapid Progressive Internal Hydrocephalus

Leukoencephalopathy with Vanishing White Matter (VWM) Presenting with Early Myoclonic Encephalopathy (EME) and Rapid Progressive Internal Hydrocephalus