Severe hyperglycorrhachia and status epilepticus after endoscopic aqueductoplasty: illustrative case.

BACKGROUND: While hypoglycorrhachia is observed and managed frequently, there are few reports in the literature of clinically significant hyperglycorrhachia after neurosurgery. Understanding the effects and management of severe hyperglycorrhachia is important to the neurosurgeon and neurocritical care teams who care for patients in these rare scenarios. OBSERVATIONS: The authors present the case of a 3-month-old male with congenital hydrocephalus who faced profound hyperglycorrhachia and status epilepticus after an endoscopic aqueductoplasty using an irrigant composed of lactated Ringer's solution with dextrose 5% in water. A multidisciplinary approach was developed to monitor and treat the patient's seizures and cerebrospinal fluid (CSF) osmolytes. LESSONS: This case provides several learning opportunities for understanding CSF physiology, pathogenesis of common brain injuries related to osmotic shifts and inflammatory states, as well as clinical management of hyperglycorrhachia. It also reiterates the significance of meticulous intraoperative assessment to avoid preventable medical errors.

Deep Brain Stimulation for Cockayne Syndrome-Associated Movement Disorder.

Cockayne syndrome (CS) is a rare progeroid disorder characterized by multisystem degeneration, including neurological dysfunction, for which deep brain stimulation (DBS) is a proposed treatment. This study represents only the third case of DBS for CS-associated movement disorder and the first in which both proposed targets had devices implanted, allowing for direct comparison. A case of DBS for CS-associated movement disorder is presented. Previous literature documents two cases with one targeting the ventral intermediate nucleus of the thalamus (VIM) and the other targeting the globus pallidus interna (GPi). Our patient underwent stimulation of GPi nuclei followed by repositioning to VIM nuclei with improved symptom control using VIM stimulation. In all cases, there was a significant clinical benefit without off-target effects. CS-associated movement disorder exhibits phenotypic variability for which DBS is a viable treatment. Target selection should be driven by clinical phenotype.

Fulminant non-infectious peri-electrode edema after deep brain stimulation system implantation in a pediatric patient.

Non-infectious peri-electrode edema is a rare complication after implantation of a deep brain stimulation (DBS) electrode. DBS is frequently used in the management of movement disorders with increasing interest surrounding its value in more rare disorders associated with movement abnormalities. This is the report of a 10-year-old male with Cockayne syndrome who acutely developed symptomatic non-infectious, non-hemorrhagic peri-electrode edema 18 days postoperatively from implantation of a DBS system targeting the bilateral globus pallidus internus. CT head confirmed extensive vasogenic edema along the entire length of the left electrode, and infectious workup was negative. The patient required admission to the pediatric intensive care unit for management utilizing steroid, hypertonic, and hyperosmolar therapy due to the amount of mass effect. Symptoms reduced over a 7-day hospital stay and were completely resolved at 1 month without removal of the DBS system. Management of this rare entity remains controversial and often involves the use of steroids and anti-epileptic prophylaxis. This represents the first case of non-infectious peri-electrode edema reported in a pediatric patient and is especially notable for its fulminant nature.

The efficacy and tolerability of auto-stimulation-VNS in children with Lennox-Gastaut syndrome.

OBJECTIVE: Lennox-Gastaut syndrome (LGS) is a severe drug-resistant epilepsy (DRE) of childhood. The Vagus Nerve Stimulator (VNS) is established as a safe and effective treatment for DRE. This study assesses efficacy and tolerability of the auto-stimulation VNS models in pediatric patients with LGS. METHODS: This is a retrospective chart review of a cohort of pediatric patients (Age 1-18 years old) with LGS implanted with an auto-stimulation VNS model at a single level four pediatric epilepsy center. Patient responder's rate was measured as seizure reduction over baseline and improvements in five quality-of-life measures as reported by the patients and families. Efficacy and tolerability were assessed at 1, 3, 6, 12, 18 and 24 months compared to baseline. RESULTS: This cohort includes 71 consecutive children with Lennox-Gastaut syndrome who underwent implantation with one of two models of the auto-stimulation VNS. The average age of the children at implantation was 20.82 months. Of those patients, 55 % of patients achieved greater than 50 % seizure reduction at six months, 67.7 % at 12 months, and 65 % at 24 months. At 12 months 11 % of the patients were completely seizure free and at 24 months 17 % were seizure free. By 24 months post implantation most of the patient families reported at least a 50 % improvement rate in one or more of the quality-of-life measures. The most commonly reported adverse events were dysphonia, paresthesia, and shortness of breath, all of which were tolerated and subsided by 24 months. SIGNIFICANCE: This study provides evidence that VNS models with the auto-stimulation paradigm based on detection of tachycardia are well tolerated and effective in a pediatric population with LGS. Furthermore, this study shows that for this population, the auto-stimulation models of the VNS may provide additional benefits over the earlier VNS versions.

The Efficacy and Safety of Rapid Cycling Vagus Nerve Stimulation in Children With Intractable Epilepsy.

PURPOSE: Vagus nerve stimulation (VNS) is an effective adjunctive therapy for drug-resistant epilepsy. Nevertheless, information is lacking regarding optimization of stimulation parameters to improve efficacy. Our study examines the safety and efficacy of rapid duty cycle VNS (OFF time ≤1.1 minute keeping duty cycle less than 50%) in pediatric cohort with intractable epilepsy. METHODS: Retrospective chart review of 50 patients (one to 17 years) with drug-resistant epilepsy treated with VNS between 2010 and 2015 at a single pediatric epilepsy center. Safety and tolerability data were aggregated across all patient visits to determine frequency of adverse events between differing duty cycles. We also compared seizure reduction rates for each patient at (1) last regular duty cycle visit, (2) first rapid duty cycle visit, and (3) last recorded rapid duty cycle visit. RESULTS: Rapid duty cycle was well tolerated, with no adverse events reported in 96.6% patient encounters. At the last visit before switching to rapid duty cycle 45.5% patients were showing response to VNS (seizure reduction rates ≥50%). This rate increased to 77.3% after switching to rapid duty cycle and remained at 77.4% at the last rapid duty cycle visit. Fifteen patients (34.1%) became responders to VNS after switching to rapid cycling; another 19 (43.2%) maintained their response with mostly improved seizure reduction rates. In only a few instances, responders became nonresponders after switching to rapid duty cycle. CONCLUSIONS: Rapid duty cycle VNS is probably safe and well tolerated; it may also be more efficacious than regular cycling VNS in some patients. This study highlights the necessity of prospective, long-term, double-blinded studies for understanding the advantages of this VNS modality.

Intracranial subdural empyemas and epidural abscesses in children

OBJECTIVE: The authors conducted a retrospective analysis of a consecutive series of children with intracranial subdural empyemas (SEs) and epidural abscesses (EAs) to highlight the important clinical difference between these two entities. They describe the delays and pitfalls in achieving accurate diagnoses and make treatment recommendations based on clinical and imaging findings. METHODS: They reviewed their experience with children who had presented with intracranial SE and/or EA in the period from January 2013 to May 2018. They recorded presenting complaint, date of presentation, age, neurological examination findings, time from presentation to diagnosis, any errors in initial image interpretation, timing from diagnosis to surgical intervention, type of surgical intervention, neurological outcome, and microbiology data. They aimed to assess possible causes of any delay in diagnosis or surgical intervention. RESULTS: Sixteen children with SE and/or EA had undergone evaluation by the authors' neurosurgical service since 2013. Children with SE (n = 14) presented with unmistakable evidence of CNS involvement with only one exception. Children with EA alone (n = 2) had no evidence of CNS dysfunction. All children older than 1 year of age had sinusitis. The time from initial presentation to a physician to diagnosis ranged from 0 to 21 days with a mean and median of 4.5 and 6 days, respectively. The time from diagnosis to neurosurgical intervention ranged from 0 to 14 days with a mean and median of 3 and 1 day, respectively. Delay in treatment was due to misinterpretation of images, a failure to perform timely imaging, progression on imaging as an indication for surgical intervention, or the managing clinician's preference. Among the 14 cases with SE, initial imaging studies in 6 were not interpreted as showing SE. Four SE collections were dictated as epidural even on MRI. The only fatality was associated with no surgical intervention. Endoscopic sinus surgery was not associated with reducing the need for repeat craniotomy. CONCLUSIONS: Regardless of the initial imaging interpretation, any child presenting with focal neurological deficit or seizures and sinusitis should be assumed to have an SE or meningitis, and a careful review of high-resolution imaging, ideally MRI with contrast, should be performed. If an extraaxial collection is identified, surgical drainage should be performed expeditiously. Neurosurgical involvement and evaluation are imperative to achieve timely diagnoses and to guide management in these critically ill children. ABBREVIATIONS: EA = epidural abscess; SE = subdural empyema.

Novel Helicobacter species H.japonicum isolated from laboratory mice from Japan induces typhlocolitis and lower bowel carcinoma in C57BL/129 IL10-/- mice.

A novel Helicobacter species Helicobacter japonicum was isolated from the stomach and intestines of clinically normal mice received from three institutes from Japan. The novel Helicobacter sp. was microaerobic, grew at 37°C and 42°C, was catalase and oxidase positive, but urease negative. It is most closely related to the 16S rRNA gene of H.muridarum (98.6%); to the 23S rRNA gene of H.hepaticus (97.9%); to the hsp60 gene of H.typhlonius (87%). The novel Helicobacter sp. has in vitro cytolethal distending toxin (CDT) activity; its cdtB gene sequence has 83.8% identity with that of H.hepaticus The whole genome sequence of H.japonicum MIT 01-6451 has a 2.06-Mb genome length with a 37.5% G + C content. When the organism was inoculated into C57BL/129 IL10-/- mice, it was cultured from the stomach, colon and cecum of infected mice at 6 and 10 weeks post-infection. The cecum had the highest H.japonicum colonization levels by quantitative PCR. The histopathology of the lower bowel was characterized by moderate to severe inflammation, mild edema, epithelial defects, mild to severe hyperplasia, dysplasia and carcinoma. Inflammatory cytokines IFNγ, TNFα and IL17a, as well as iNOS were significantly upregulated in the cecal tissue of infected mice. These results demonstrate that the novel H.japonicum can induce inflammatory bowel disease and carcinoma in IL10-/- mice and highlights the importance of identifying novel Helicobacter spp. especially when they are introduced from outside mouse colonies from different geographic locations.

Impact of epilepsy surgery on seizure control and quality of life: a 26-year follow-up study.

PURPOSE: The short-term efficacy and safety of epilepsy surgery relative to medical therapy has been established, but it remains underutilized. There is a lack of data regarding the long-term seizure-control rates and quality of life outcomes after epilepsy surgery. This study represents the longest follow-up study to date, with a mean follow-up duration of 26 years. METHODS: We studied the seizure and health-related quality of life outcomes of patients who underwent epilepsy surgery by Dr. Sidney Goldring from 1967 to 1990. Retrospective clinical chart reviews gathered perioperative data and surveys obtained follow-up data. Seizure outcome was evaluated using the Engel classification system. KEY FINDINGS: Of 361 patients, 117 (32.4%) completed follow-up interviews. Fifty-six patients (48%) were Engel class I. Mean overall Quality of Life in Epilepsy (QOLIE-31) questionnaire score for the cohort was 68.2 ± 16. Eighty percent of patients reported their overall quality of life now as being better than before surgery. Seizure freedom was associated with better quality of life. We did not observe a statistically significant association between postoperative complications and long-term outcome. Patients who underwent temporal lobe resection achieved better seizure outcomes than those who underwent other types of procedures. Astatic seizures and bilateral surgery were associated with a worse Engel class outcome. SIGNIFICANCE: Our study demonstrates that the beneficial effects of epilepsy surgery are sustained over decades, and that these beneficial effects are correlated with an improved quality of life. The confirmation of its durability makes us optimistic that the outcomes from modern epilepsy surgery will be even better and that our present enthusiasm for this treatment modality is not misplaced.

Bilateral deep brain stimulation for treatment of medically refractory paroxysmal nonkinesigenic dyskinesia.

The authors present the case of a 26-year-old man with a 10-15-year history of worsening bilateral dystonia and baseline chorea occurring up to 20 times per day that was exacerbated by stress and anxiety and was refractory to medical management. Paroxysmal nonkinesigenic dyskinesia was diagnosed, which is a rare, hyperkinetic movement disorder that is episodic and does not respond to nonbenzodiazepine antiepileptics. The patient was significantly debilitated by his disease, lived in a group home, and suffered from frequent falls, necessitating the wearing of a protective helmet and face mask at all times. The patient underwent implantation of bilateral deep brain stimulation quadripolar electrodes in the globus pallidus internus with the aid of image-guided stereotactic neurosurgery and microelectrode recording without complication. At his 1-month postoperative follow-up, the patient reported a subjective 90% improvement in his symptoms; the only notable side effect was a slight increased slurring in his baseline dysarthria. Objective reporting and recording forms maintained by the patient's caretakers over the following 18 months suggested a significant and sustained improvement in his overall balance, ambulation, and gross motor function with a substantial decrease in the incidence of reported falls. The authors conclude that pallidal deep brain stimulation may be successfully applied to patients suffering from refractory paroxysmal nonkinesigenic dyskinesia with promising results. This treatment strategy deserves further prospective investigation, clinical consideration, and refinement.

Accuracy of the freehand pass technique for ventriculostomy catheter placement: retrospective assessment using computed tomography scans.

OBJECTIVES: The standard method of ventriculostomy catheter placement is a freehand pass technique using surface anatomical landmarks. This study was undertaken to determine the accuracy of successful ventriculostomy procedures performed at a single institution's intensive care unit (ICU). The authors hypothesized that use of surface anatomical landmarks alone with successful results frequently do not correlate with desirable catheter tip placement. METHODS: Retrospective evaluation was performed on the head computed tomography (CT) scans of 97 patients who underwent 98 freehand pass ventriculostomy catheter placements in an ICU setting. Using the postprocedure CT scans of the patients, 3D measurements were made to calculate the accuracy of ventriculostomy catheter placement. RESULTS: The mean distance (+/- standard deviation [SD]) from the catheter tip to the Monro foramen was 16 +/- 9.6 mm. The mean distance (+/- SD) from the catheter tip to the center of the bur hole was 87.4 +/- 14.0 mm. Regarding accurate catheter tip placement, 56.1% of the catheter tips were in the ipsilateral lateral ventricle, 7.1% were in the contralateral lateral ventricle, 8.2% were in the third ventricle, 6.1% were within the interhemispheric fissure, and 22.4% were within extraventricular spaces. CONCLUSIONS: The accuracy of freehand ventriculostomy catheterization at the authors' institution typically required 2 passes per successful placement, and, when successful, was 1.6 cm from the Monro foramen. More importantly, 22.4% of these catheter tips were in nonventricular spaces. Although many neurosurgeons believe that the current practice of ventriculostomy is good enough, the results of this study show that there is certainly much room for improvement.

Ecological and developmental dynamics of a host-parasite system involving a sea anemone and two ctenophores.

The lined sea anemone Edwardsiella lineata has evolved a derived parasitic life history that includes a novel body plan adapted for life inside its ctenophore hosts. Reputedly its sole host is the sea walnut, Mnemiopsis leidyi, a voracious planktivore and a seasonally abundant member of many pelagic ecosystems. However, we have observed substantially higher E. lineata prevalence in a second ctenophore species, the ctenophore predator Beroë ovata. The interplay among these 3 species has important conservation consequences as M. leidyi introductions are thought to be responsible for the severe depletion of numerous commercial fisheries in the Mediterranean basin, and both E. lineata and B. ovata have been proposed as biological controls for invasive M. leidyi. Over a 3-yr period (2004-2006), we collected 8,253 ctenophores from Woods Hole, Massachusetts, including M. leidyi, B. ovata, and a third ctenophore, Pleurobrachia pileus, and we recorded E. lineata infection frequencies, parasite load, and parasite location. We also conducted laboratory experiments to determine the likely mechanisms for parasite introduction and the effect of each host on parasite development. We observed peak E. lineata infection frequencies of 0% in P. pileus, 59% in M. leidyi, and 100% in B. ovata, suggesting that B. ovata could be an important natural host for E. lineata. However, in laboratory experiments, E. lineata larvae proved far more successful at infecting M. leidyi than B. ovata, and E. lineata parasites excised from M. leidyi exhibited greater developmental competence than parasites excised from B. ovata. Although we show that E. lineata is efficiently transferred from M. leidyi to B. ovata when the latter preys upon the former, we conclude that E. lineata larvae are not well adapted for parasitizing the latter species and that the E. lineata parasite is not well adapted for feeding in B. ovata; these developmental and ecological factors underlie the host specificity of this recently evolved parasite.

The "clamshell" craniotomy technique in treating sagittal craniosynostosis in older children.

OBJECT: Although most patients with sagittal craniosynostosis are recognized and treated in infancy, some children are not referred to craniofacial centers until later in childhood. In this paper the authors describe a novel operative technique for calvarial reconstruction in older children with previously untreated sagittal craniosynostosis. METHODS: The authors report a clinical series of eight patients who were treated using novel single-stage calvarial reconstruction, and they assess the complications and outcomes. The patient is placed supine for the procedure, which consists of a coronal incision, bifrontal craniotomy without orbital osteotomy, and multiple interlocking midline parietooccipital osteotomies and recontouring. Fixation is achieved using a bioabsorbable plate system. Cranial indices were calculated from measurements obtained before and after the reconstructive procedures. Preoperative, intraoperative, and postoperative photographs and three-dimensional computed tomography scans are presented for review. Between November 2003 and April 2005, the authors treated seven boys (age range approximately 1-10 years, mean age 4.2 years) with uncorrected sagittal craniosynostosis and one with bicoronal and sagittal synostosis. The mean operating time was 5.13 hours (range 4.3-8 hours), with a mean blood loss of 425 ml (range 200-800 ml). As a percentage of the estimated circulating blood volume, the mean operative blood loss was 33.5% (range 17-57%). The mean hospital stay was 4.9 days. The cranial index significantly improved from a mean of 65.6 to 71.3% (p = 0.001). No acute or delayed complications have been noted. Follow-up examinations performed at an average of 12 months (range 1-17 months) have confirmed early patient and family satisfaction. CONCLUSIONS: An approach of aggressive calvarial reconstruction with multiple interleaving osteotomies crossing the midline achieves improvements in biparietal narrowing. Combined with a bifrontal reconstruction, early outcomes are excellent, with an acceptable amount of intraoperative blood loss and no significant complications.