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Purpose: Overdrainage (OD) is one of the most frequent complications related to drainage of the cerebrospinal fluid (CSF). It is mostly associated with valve-bearing shunt systems but should probably be considered as a risk factor in any type of CSF diversion procedure. There is extreme variation in the reported incidence of OD due to the lack of consensus on defining criteria and an unclear perception of the pathophysiology. Hence, OD is probably underreported and underestimated. The objective of this paper was to establish a definition of OD, based on a systematic review of the literature., Methods: A systematic search was conducted in MEDLNE and EMBASE. Studies providing a definition or a description of diagnostic findings related to OD in ventriculoperitoneal shunt treated hydrocephalus were included. Non-English titles, abstracts and manuscripts were excluded. Extracted descriptions were graded into five groups (class I-V studies) based on how precise the terminology used to describe OD was. Class I studies were included for further analysis and characteristics of OD were extracted. The quality of included descriptions was assessed by a clinical expert panel., Results: A total of 1309 studies were screened, 190 were graded into groups, and 22, which provided specific definitions or descriptions of OD, were graded as class I studies. We extracted 32 different characteristics consistent with OD (e.g., clinical symptoms, radiological signs, and syndromes)., Conclusion: There was an overall agreement that CSF overdrainage following implantation of a ventriculoperitoneal shunt in a mixed pediatric and adult population is characterized as a persistent condition with clinically manifestations as postural dependent headache, nausea, and vomiting and/or radiological signs of slim ventricles and/or subdural collections., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

In patients with hydrocephalus, prognosis and intervention are based on multiple factors. This includes, but is not limited to, time of onset, patient age, treatment history, and obstruction of cerebrospinal fluid flow. Consequently, several distinct hydrocephalus classification systems exist. The International Classification of Diseases (ICD) is universally applied, but in ICD-10 and the upcoming ICD-11, hydrocephalus diagnoses incorporate only a few factors, and the hydrocephalus diagnoses of the ICD systems are based on different clinical measures. As a consequence, multiple diagnoses can be applied to individual cases. Therefore, similar patients may be described with different diagnoses, while clinically different patients may be diagnosed identically. This causes unnecessary dispersion in hydrocephalus diagnostics, rendering the ICD classification of little use for research and clinical decision-making. This paper critically reviews the ICD systems for scientific and functional limitations in the classification of hydrocephalus and presents a new descriptive system. We propose describing hydrocephalus by a system consisting of six clinical key factors of hydrocephalus: A (anatomy); S (symptomatology); P (previous interventions); E (etiology); C (complications); T (time-onset and current age). The "ASPECT Hydrocephalus System" is a systematic, nuanced, and applicable description of patients with hydrocephalus, with a potential to resolve the major issues of previous classifications, thus providing new opportunities for standardized treatment and research., (© 2022. The Author(s).)

Background: Intermittent theta-burst stimulation (iTBS), a novel repetitive transcranial magnetic stimulation (rTMS) technique, appears to have antidepressant effects when applied over left dorsolateral prefrontal cortex (DLPFC). However, its underlying neurobiological mechanisms are unclear. Proton magnetic resonance spectroscopy ( 1 H-MRS) provides in vivo measurements of cerebral metabolites altered in major depressive disorder (MDD) like N-acetyl-aspartate (NAA) and choline-containing compounds (Cho). We used MRS to analyse effects of iTBS on the associations between the shifts in the NAA and Cho levels during therapy and MDD improvement., Methods: In-patients with unipolar MDD (N = 57), in addition to treatment as usual, were randomized to receive 20 iTBS or sham stimulations applied over left DLPFC over four weeks. Single-voxel 1 H-MRS of the anterior cingulate cortex (ACC) was performed at baseline and follow-up. Increments of concentrations, as well as MDD improvement, were defined as endpoints. We tested a moderated mediation model of effects using the PROCESS macro (an observed variable ordinary least squares and logistic regression path analysis modeling tool) for SPSS., Results: Improvement of depressive symptoms was significantly associated with decrease of Cho/NAA ratio, mediated by NAA. iTBS had a significant moderating effect enhancing the relationship between NAA change and depression improvement., Conclusions: Our findings suggest a potential neurochemical pathway and mechanisms of antidepressant action of iTBS, which may moderate the improvement of metabolic markers of neuronal viability. iTBS might increase neuroplasticity, thus facilitating normalization of neuronal circuit function., Competing Interests: Declaration of competing interest MZ received in 2015 financial support for an educational program from Lundbeck, Servier, Actelion, MagVenture, Mag and More, Localite, Inomed, Sooma Oy, Brainsway/Tolko, and NeuroConn. However, it did not influence the content of this manuscript. All other authors report no financial or other relationship relevant to the subject of this article., (Copyright © 2020 The Author(s). Published by Elsevier Inc. All rights reserved.)

Many patients with epilepsy caused by hypothalamic hamartomas (HHs) have cognitive impairments during the course of the disease or following neurosurgical treatment. The purpose of this study was to assess cognitive function in these patients, as well as factors influencing preoperative cognitive performance and cognitive outcome after neurosurgical treatment. Using the two largest and most detailed neuropsychology datasets on HH and epilepsy from two centers, we retrospectively report on cognitive functions in 48 patients with structural epilepsy due to HH (mean age ± standard deviation [SD] 20 ± 12 years, range 5-53 years, median 16 years; disease duration mean 17 ± 11 years). Intelligence, verbal learning and recall, and speed and executive functions (processing speed and cognitive flexibility) were assessed before and on average 19 (±11) months after surgery (interstitial radiosurgery: N = 22; neurosurgical resection/disconnection: N = 26). Prior to neurosurgical treatment, 52% of patients showed impaired executive and 62% showed reduced verbal memory functions. A trend for a detrimental effect of higher drug load on cognitive functioning was found. After neurosurgical treatment, intellectual functions for the entire cohort tended to increase. This correlated with improved seizure frequency and decreased number of antiepileptic drugs (AEDs). However, postoperative outcomes for individual patients were highly variable, with significant deteriorations in 17% (processing speed) to 34% (cognitive flexibility and verbal learning), and performance increases in 17% (intellectual functioning) up to 39% (processing speed) of the patients. Higher levels of presurgical performance were significant predictors of cognitive decline after surgery. These results are highly relevant for patient consultation and may help with therapeutic decisions., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Aim: To report a successful treatment of an atypical case of pseudotumor cerebri., Background: Pseudotumor cerebri is a disorder of raised intracranial pressure. Multiple Case Series have been published about its treatment with sinus stenting. All these cases have had typical presentations., Case: An atypical fulminant case of pseudotumor cerebri which was treated successfully with venous sinus stenting., Conclusion: Venous sinus stenting can be done effectively in atypical cases of pseudotumor cerebri.

The outcome of pediatric hydrocephalus, including surgical complications, neurological sequelae and academic achievement, has been the matter of many studies. However, much uncertainty remains, regarding the very long-term and social outcome, and the determinants of complications and clinical outcome. In this paper, we review the different facets of outcome, including surgical outcome (shunt failure, infection and independence, and complications of endoscopy), clinical outcome (neurological, sensory, cognitive sequels, epilepsy), schooling and social integration. We then provide a brief review of the English-language literature and highlighting selected studies that provide information on the outcome and sequelae of pediatric hydrocephalus, and the impact of predictive variables on outcome. Mortality caused by hydrocephalus and its treatments is between 0 and 3%, depending on the duration of follow-up. Shunt event-free survival (EFS) is about 70% at one year and 40% at ten years. The EFS after endoscopic third ventriculostomy (ETV) appears better but likely benefits from selection bias and long-term figures are not available. Shunt infection affects between 5 and 8% of surgeries, and 15 to 30% of patients according to the duration of follow-up. Shunt independence can be achieved in 3 to 9% of patients, but the definition of this varies. Broad variations in the prevalence of cognitive sequelae, affecting 12 to 50% of children, and difficulties at school, affecting between 20 and 60%, attest of disparities among studies in their clinical evaluation. Epilepsy, affecting 6 to 30% of patients, has a serious impact on outcome. In adulthood, social integration is poor in a substantial number of patients but data are sparse. Few controlled prospective studies exist regarding hydrocephalus outcomes; in their absence, largely retrospective studies must be used to evaluate the long-term consequences of hydrocephalus and its treatments. This review aims to help to establish the current state of knowledge and to identify conflicting data and unanswered questions, in order to direct future studies.

Object: This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH)., Methods: Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death., Results: Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm(3). In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS. Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg)., Conclusions: Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.

Purpose: Hypothalamic hamartomas (HHs) are a malformation of the ventral hypothalamus and tuber cinereum, associated with gelastic seizures and epilepsy. We sought to determine the spectrum of electroencephalography (EEG) abnormalities in a large cohort of HH patients., Methods: Data was collected for HH patients undergoing evaluation between 2003 and 2007. Data included seizure history, prior treatment, and results of diagnostic studies. After informed consent, data were entered into a database., Key Findings: We reviewed 133 HH patients. Mean age at time of data analysis was 15.7 years (59.4% male). Most patients had gelastic (77%) and/or complex partial seizures (58%). Records for 102 EEG studies on 73 patients were reviewed. Interictal epileptiform abnormalities were seen in 77%, localizing predominately to the temporal and frontal regions. Records for 104 video-EEG (VEEG) studies on 65 patients were reviewed. Of 584 gelastic seizures (GS) captured, no ictal EEG change was noted in 438 (75%). Of GS with localizing features, 89% suggested onset from the temporal and/or frontal regions. There were 160 complex partial seizures (CPS). For those with localizing features, 100% localized to the temporal and/or frontal head regions. EEG and VEEG findings correlated with the side of HH attachment. VEEG did not influence outcome., Significance: EEG features in HH patients are diverse. The majority of gelastic seizures fail to demonstrate change in the EEG. The lack of EEG changes with many clinical seizures, and the false localization seen in those events with an ictal change suggest the utility of EEG is limited in the evaluation of these patients., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

The cellular mechanisms underlying intrinsic epileptogenesis in human hypothalamic hamartoma (HH) are unknown. We previously reported that HH tissue is composed predominantly of GABAergic neurons, but how GABAergic-neuron-rich HH tissue is intrinsically epileptogenic is unclear. Here, we tested the hypotheses that some HH neurons exhibit immature features and that GABA excites these neurons via activation of GABA(A) receptors (GABA(A)Rs). Gramicidin-perforated and cell-attached patch-clamp recordings were performed using freshly-dissociated HH neurons to evaluate GABA(A)R-mediated currents, Cl(-) equilibrium potentials, and intracellular Cl(-) concentrations. Single-cell RT-PCR and immunocytochemical techniques were used to examine cation-Cl(-) co-transporter (NKCC1 and KCC2) gene and KCC2 protein expression and molecular markers of maturation. From a total of 93 acutely-dissociated HH neurons from 34 patients, 76% were small (soma: 6-9 microm) and 24% were large (soma: >20 microm) in size. Under gramicidin-perforated patch recording conditions, GABA(A)R activation depolarized/excited large but hyperpolarized/inhibited small HH neurons in most cases. Compared to small HH neurons, large HH neurons exhibited more positive Cl(-) equilibrium potentials, higher intracellular Cl(-) concentrations, lower KCC2 expression, and an immature phenotype, consistent with GABA(A)R-mediated excitation. Taken collectively, we provide novel evidence for and mechanistic insights into HH epileptogenicity: GABA(A)R-mediated excitation.

We present an overview of the history, development, technological advancements, current application, and future trends of cranial endoscopy. Neuroendoscopy provides a safe and effective management modality for the treatment of a variety of intracranial disorders, either tumoral or non-tumoral, congenital, developmental, and degenerative, and its knowledge, indications, and limits are fundamental for the armamentarium of the modern neurosurgeon.

Abnormalities in GABA(A) receptor structure and/or function have been associated with various forms of epilepsy in both humans and animals. Whether this is true for patients with gelastic seizures and hypothalamic hamartoma (HH) is unknown. In this study, we characterized the pharmacological properties and native subunit composition of GABA(A) receptors on acutely dissociated single neurons from surgically resected HH tissues using patch-clamp, immunocytochemical, and RT-PCR techniques. We found that 1) GABA induced an inward current (I(GABA)) at a holding potential of -60 mV; 2) I(GABA) was mimicked by the GABA(A) receptor agonist muscimol and blocked by the GABA(A) receptor antagonist bicuculline, suggesting that I(GABA) was mediated principally through the GABA(A) receptor; 3) the EC(50) and Hill coefficient derived from the I(GABA) concentration-response curve were 6.8 muM and 1.9, respectively; 4) the current-voltage curve was linear at a reversal potential close to zero; and 5) I(GABA) exhibited low sensitivity to zinc and diazepam but higher sensitivity to pentobarbital and pregnanolone. Additionally, using Xenopus oocytes microtransplanted with normal human hypothalamic tissue, we confirmed that the functional properties of GABA(A) receptors were similar to those seen in small isolated HH neurons. Finally, the expression profile of GABA(A) receptor subunits obtained from normal control human hypothalamic tissue was identical to that from surgically resected human HH tissue. Taken together, our data indicate that GABA(A) receptors on small HH neurons exhibit normal pharmacosensitivity and subunit composition. These findings bear relevance to a broader understanding of inhibitory neurotransmission in human HH tissue.

The hypothalamic hamartoma (HH) is a rare developmental malformation commonly associated with gelastic seizures that are notoriously refractory to medical therapy. Recent evidence supports the intrinsic seizure propensity of HH. Despite increasing clinical recognition of this condition, the mechanisms of seizure genesis in HH tissue remain unclear. This review summarizes the histochemical and electrophysiological properties of HH neurons, and relates these findings to those characteristics identified in other types of epileptic tissue. Initial studies have revealed two distinct populations of neurons in surgically resected HH tissue. One group consisted of small gamma-aminobutyric acid (GABA)-expressing neurons that occurred principally in clusters and displayed spontaneous rhythmic firing. The second group was composed of large, quiescent, pyramidal-like neurons with more extensive dendritic and axonal arborization. We propose that the small, spontaneously firing GABAergic neurons send inhibitory projections to and drive the synchrony of large output neurons. These observations constitute the basis for future investigations aimed at elucidating the mechanisms of subcortical epileptogenesis.

The hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures, which are usually refractory to medical therapy. The mechanisms of epileptogenesis operative in this subcortical lesion are unknown. In this study, we used standard patch-clamp electrophysiological techniques combined with histochemical approaches to study individual cells from human HH tissue immediately after surgical resection. More than 90% of dissociated HH cells were small (6-9 microm soma) and exhibited immunoreactivity to the neuronal marker NeuN, and to glutamic acid decarboxylase, but not to glial fibrillary acidic protein. Under current-clamp, whole-cell recordings in single dissociated cells or in intact HH slices demonstrated typical neuronal responses to depolarizing and hyperpolarizing current injection. In some cases, HH cells exhibited a "sag-like" membrane potential change during membrane hyperpolarization. Interestingly, most HH cells exhibited robust, spontaneous "pacemaker-like" action potential firing. Under voltage-clamp, dissociated HH cells exhibited functional tetrodotoxin (TTX)-sensitive Na(+) and tetraethylammonium-sensitive K(+) currents. Both GABA and glutamate evoked whole-cell currents, with GABA exhibiting a peak current amplitude 10-fold greater than glutamate. These findings suggest that human HH tissues, associated with gelastic seizures, contained predominantly small GABAergic inhibitory neurons that exhibited intrinsic "pacemaker-like" behavior.

Shunt systems with differential pressure valves are prone to the complications of overdrainage. A programmable valve permits adjustment of the opening pressure of the valve. In this paper the authors report the incidence of subdural fluid collections in a randomized trial of programmable compared with conventional valves, and they describe methodologies used in management of this complication. A multiinstitutional, prospective, randomized trial of the Codman Hakim programmable valve and conventional fixed-pressure valves was undertaken. Two classes were defined: "new" and "replacement" valves. Randomization of the type of valve in each group was performed at each study site. Clinical and radiological studies were required at fixed intervals over a 104-week period. All complications were reported. The experimental valves were required to be reprogrammed after magnetic resonance imaging studies, but all other decisions regarding pressure setting were left to each investigator. Three hundred seventy-seven patients were randomized; 194 were treated with a programmable valve and 183 with a fixed-pressure valve. The two groups were statistically similar in demographic composition, as were the "new" and "replacement" categories. The investigators made 540 valve pressure changes (five per patient; range one-41 changes). More than half of the reprogramming adjustments were made in the first 3 months postplacement; 70% were made within 6 months. More than half of all reprogramming adjustments were required in a group of 30 patients. Four treatment modalities were observed: 1) 30% of the fluid collections resolved spontaneously (25% in the patients with programmable valves and 36.3% in those with conventional valves) and were largely found to be hygromas in infants and children; 2) four subdural fluid collections were unresolved and under observation; 3) the subdural hematoma was drained and the shunt removed (in 8.3% of patients with the programmable valve and 36.3% of those with the control valve); 4) the pressure of programmable valve was raised in 58% of patients (seven of 12), and this increase in opening pressure was a feature used by investigators to affect treatment. There was no significant difference in the incidence of subdural fluid collections between the programmable and fixed-pressure valve treatment groups. The programmable feature provided a considerable advantage in treatment when subdural collections occurred.

This report of 21 patients with cerebral injury resultant from child abuse, identifies a broad profile of central nervous sequelae from such injuries, 6 patients were admitted with whiplash shaken infants syndrome, all with severe signs of CS dysfunction. 3 children presented with acute cerebral confusions, 2 with severe cord injuries with resultant paresis and 10 with skull fractures or miscellaneous injuries. Computer tomography has proved to be of great assistance in precise identification of site and size of subdural/intracerebral hematomas in abused children and in identifying posterior fossa lesions and providing longitudinal assessment of sequelae of cerebral trauma. Also the role of the Child Protection Team in management of such acutely injured children is defined.

Caudal regression syndrome involves absence of the sacrococcygeal vertebrae with or without lumbar vertebral defects. Since the neurological condition of infants with this syndrome deteriorates, radiographic studies are important to rule out any surgically correctable lesion. A paraplegic male neonate with a vertebral canal ending at T11 is presented. This is the first report of using magnetic resonance imaging to evaluate the spinal cord and surrounding soft tissues involved in this syndrome. Magnetic resonance imaging provides more anatomical details than myelogram with computed tomography, which greatly aids in determining whether surgery is necessary.

Choroid plexus carcinomas in siblings aged 11 months and 10 years were examined by light microscopy and immunocytochemistry. One case was studied by electron microscopy. Choroid plexus carcinoma is rare, with approximately 24 reported cases in children. Predicting the behavior of choroid plexus tumors from the histology can be difficult. Neither mitoses nor necrosis were seen in one case, but evaluation of proliferation using Ki-67 monoclonal antibody showed 9% of the cells to be in proliferative phases of the cell cycle, a high value for a glial-derived neoplasm. Ki-67 activity may be a more sensitive measure of proliferation in malignant choroid plexus tumors than the presence of mitoses and necrosis, and additional studies may establish its role in distinguishing between choroid plexus carcinoma and papilloma when histologic classification is equivocal. Both tumors were immunoreactive for keratin, which confirmed previous studies. Both were nonreactive for glial fibrillary acidic protein, S-100, and carcinoembryonic antigen (CEA), unlike a previous study which reported that choroid plexus carcinoma, compared to papilloma, was uniquely S-100-negative and CEA-positive. Choroid plexus carcinoma in siblings has not been reported. Chance occurrence in siblings is extremely unlikely; thus a genetic basis for the neoplasms is likely, although environmental influences cannot be excluded.

Intracarotid chemotherapy has been suggested as an additional mode of therapy in patients with brain tumors. Seven comatose patients received intracarotid 5-fluorouracil and adriamycin after intracarotid infusion of 25% mannitol to open the blood-brain barrier at the tumor site. Five of seven patients became fully functional for 3-12 months. Another 11 patients entered the study, of which nine are currently receiving therapy and are functional, and two have died, one from brain herniation. The results are encouraging and support the need for further research of this therapeutic method.

The magnetic resonance (MR) imaging appearance of venous malformations, all angiographically verified, was evaluated in 11 patients. A venous malformation is characteristically depicted as a tubular area of decreased signal intensity in the white matter of the brain. In one patient, a histologically verified cavernous malformation was also present with a characteristic mixed signal-intensity core on the T2-weighted MR images. Care should be used when evaluating venous angiomas to exclude the presence of a lesion with associated prominent venous drainage, such as a glioma.

A review of 122 pediatric cases of vertebral column and spinal cord injuries is presented. These relatively uncommon injuries can be characterized by four distinct injury patterns: fracture only, fracture with subluxation, subluxation only, and spinal cord injury without radiographic abnormality. The immature pediatric spine has several anatomical and biomechanical features that distinguish it from the mature adolescent spine and, accordingly, the frequency of the injury type, the level of spine injury, and the incidence of neurological compromise were found to vary with the age of the patient. Follow-up data were obtained in 93% of the cases (median duration 44 months). No patient was made worse by treatment, 89% of the patients with incomplete myelopathy on admission were improved on their last examination, and 20% of the patients with a complete myelopathy had evidence of significant recovery of function. The authors conclude that the outcome after pediatric spinal trauma is good.

A child who developed generalized bleeding immediately after placement of a ventriculoperitoneal shunt was found to have evidence of disseminated intravascular coagulation (DIC). Infusion of fresh frozen plasma was followed promptly by improvement in laboratory values and cessation of bleeding. Complications of the acute bleeding episode included intraventricular hemorrhage, loss of 50% of the red cell volume into subcutaneous tissues, and transient peritoneal irritation. Defibrination syndrome (that is, DIC) due to release of tissue thromboplastin is a recognized complication of trauma, particularly with brain injury. Defibrination can be induced in experimental systems with administration of very small amounts of thromboplastin, which is present in high concentration in brain tissue. This has not been described previously with minor neurosurgical procedures. The diagnosis of DIC should be considered if excessive bleeding occurs after any brain insult, since early recognition and restoration of normal hemostasis by replacement of clotting factors should prevent major complications due to ongoing hemorrhage.