Unique findings of subependymal giant cell astrocytoma within cortical tubers in patients with tuberous sclerosis complex: a histopathological evaluation.

INTRODUCTION: Tuberous sclerosis is associated with three central nervous system pathologies: cortical/subcortical tubers, subependymal nodules (SENs), and subependymal giant cell astrocytomas (SEGAs). Tubers are associated with epilepsy, which is often medication-resistant and often leads to resective surgery. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be effective reducing seizure burden in some patients with tuberous sclerosis complex (TSC)-related refractory epilepsy. mTORi have also been shown to be an alternative for surgery treating SEGAs. We describe several cases of resected tubers that contained SEGA tissue without an intraventricular SEGA. METHODS: After institutional review board (IRB) protocol approval, we retrospectively reviewed the surgical-pathological data for all TSC patients who underwent cortical resections for treatment of refractory epilepsy at NYU Langone Medical Center and Tel Aviv Medical Center between 2003 and 2013. Data included demographics, epilepsy type, MRI characteristics, epilepsy outcome, and histopathological staining. RESULTS: We reviewed cortical resections from 75 patients with complete pathological studies. In three patients, cortical lesions demonstrated histopathological findings consistent with a SEGA within the resected tuber tissue, with no intraventricular SEGA. All lesions were cortically based and none had any intraventricular extension. No patient had been treated before surgery with an mTORi. Two of the three patients remain Engel grade I-II. All lesions stained positive for glial fibrillary acidic protein (GFAP), synaptophysin, and neuronal nuclear antigen (NeuN). CONCLUSION: This is the first description of cortical tubers harboring SEGA tissue. This observation though preliminary may suggest a subgroup of patients with intractable epilepsy in whom mTORi may be considered before surgical intervention.

Open and endoscopic excision of calvarial dermoid and epidermoid cysts: a single center experience on 128 consecutive cases.

OBJECTIVE: Dermoid and epidermoid cysts rank among the most common pediatric tumors. We analyzed the outcomes of surgical excision of dermal and epidermal inclusion cysts in a large consecutive series of children. METHODS: We retrospectively reviewed 128 consecutive children who underwent calvarial inclusion cyst resection between 2000 and 2010 at NYU Langone Medical Center. Demographic information, neurological exam, lesion location, lesion diameter, type of treatment, extent of resection, time of follow-up, and recurrence were collected. RESULTS: The cohort includes 67 girls (52.3 %) and 61 boys (47.7 %). Age at diagnosis ranged from birth to 6.5 years (mean of 1.2 years) with surgical intervention between 1 month and 20 years of age (1.5 ± 2.1). Of the 128 patients, 107 underwent open resection. Surgical approach was determined by the senior surgeon. Location, postoperative cosmesis, and family preference were the determining factors. Endoscopic resection was favored with supraorbital and glabellar lesions (75 % endoscopic versus 25 % open) using a rigid scope via a single incision. Erosion of the outer table and involvement of the inner table was noted in 20 patients (15 %), 14 of which were reconstructed using a split thickness calvarial graft. These lesions were noted to be significantly larger than lesions where cranioplasty was not used (1.9 ± 2.81 cm versus 1.23 ± 0.98 cm, p = 0.022). Gross total resection was achieved in all cases. DISCUSSION: Complete removal and cure from dermoid and epidermoid inclusion cysts are possible. Complications are few. Endoscopic approaches are useful to improve cosmesis and limit tissue damage for lesions near the orbits.

Outcome comparison of atlantoaxial fusion with transarticular screws and screw-rod constructs: meta-analysis and review of literature.

STUDY DESIGN: Literature review and meta-analysis. OBJECTIVE: To compare clinical and radiographic outcomes of patients treated with transarticular screws (TASs) and screw-rod constructs (SRCs) for posterior atlantoaxial fusion. BACKGROUND: Modern techniques for C1-C2 fusions include Magerl and Seeman's TAS and SRC using C1 lateral mass screws and C2 pars/pedicle screws as described by Goel and Laheri and later modified by Harms and Melcher. MATERIALS AND METHODS: Online databases were searched for English-language articles between 1986 and April 2011 describing posterior atlantoaxial instrumentation with C1-C2 TAS or SRC. Forty-five studies (2073 patients) treated with TAS and 24 studies (1073 patients) treated with SRC fulfilled inclusion criteria. Standard and formal meta-analysis techniques were used to compare the outcomes. RESULTS: All studies provided class III evidence. There were no differences in 30-day mortality (0.8% vs. 0.6%) or neurological injury (0.2% vs. 0%). There was a higher incidence of vertebral artery injury [4.1% (95% confidence interval (CI), 2.8%-5.4%) vs. 2.0% (95% CI, 1.1%-3.4%); P=0.02] and malpositioned screws [7.1% (95% CI, 5.7%-8.8%) vs. 2.4% (95% CI, 1.1%-4.1%); P<0.001] and a slightly lower rate of fusion with the TAS technique [97.5% (95% CI, 95.9%-98.5%) vs. 94.6% (95% CI, 92.6%-96.1%); P<0.001]. CONCLUSIONS: TAS and SRC are safe and effective treatment options for C1-C2 instability but require a thorough knowledge of atlantoaxial anatomy for successful insertion of screws. Slightly higher rates of fusion and less risk of injury to the vertebral artery during screw placement were observed with the SRC technique. However, differences in graft material and techniques were noted. Prospective, randomized studies with validated radiographic and clinical outcome metrics are necessary for proper comparison of these techniques.

Comparison of screw malposition and vertebral artery injury of C2 pedicle and transarticular screws: meta-analysis and review of the literature.

STUDY DESIGN: Literature review and meta-analysis. OBJECTIVES: To compare the incidence of screw malposition and vertebral artery injury (VAI) with transarticular screws (TAS) and C2 pedicle screws (C2PS) using meta-analysis techniques. SUMMARY OF BACKGROUND DATA: Posterior instrumentation for atlantoaxial fusions can be challenging and risky. Some centers report a higher incidence of VAI with the implantation of TAS compared with C2PS, whereas other data do not support this. METHODS: Online databases were searched for English language articles between 1994 and April 2011 describing the clinical and radiographic outcomes after insertion of C2PS or TAS. Forty-one studies reporting on 3627 TAS and 33 studies describing 2979 C2PS met inclusion criteria for VAI or clinically significant misplacements (VAI, neurological deficits, or misplacements requiring surgical revision), and 36 studies reporting on 3280 TAS and 28 studies describing 2532 C2PS met inclusion criteria for radiographic misplacement outcomes. RESULTS: All studies comprised class III evidence. VAI occurred in 26 of 3627 (0.72%) implanted TAS and in 10 of 2979 (0.34%) implanted C2PS (P=0.01). Clinically significant misplacements occurred in 67 TAS (1.84%) and in 10 C2PS (0.34%; P<0.0001). The point estimate of VAI for TAS was 1.68% [confidence interval (CI), 1.23%-2.29%] and was higher than C2PS (1.09%; CI, 0.73%-1.63%; P=0.01). The point estimate of clinically significant screw malposition for TAS was 2.33% (CI, 1.61%-3.37%) and was higher than that of C2PS (1.15%; CI, 0.77%-1.70%; P<0.001). CONCLUSIONS: With training, experience, and anatomic knowledge, both TAS and C2PS can be inserted accurately and safely. However, improper insertion and VAI can have catastrophic consequences. Our review identified a higher risk of VAI, neurological injury, and clinically significant malpositions with TAS compared with C2PS. These data provide preliminary support for the supposition that C2PS have a lower risk of morbidity.

Epilepsy control following intracranial monitoring without resection in young children.

PURPOSE: Intracranial monitoring (IM) is a key diagnostic procedure for select patients with treatment-resistant epilepsy (TRE). Seizure focus resection may improve seizure control in both lesional and nonlesional TRE. IM itself is not considered to have therapeutic potential. We describe a cohort of patients with improved seizure control following IM without resective surgery. METHODS: Over 12.5 years, 161 children underwent 496 surgeries including intracranial monitoring. We retrospectively reviewed the patients' charts, operative reports, and radiologic scans, under an institutional review board-approved protocol. KEY FINDINGS: Seventeen patients underwent only IM, without additional resective surgery, and seven had a dramatic improvement in their epilepsy; six of the seven patients are seizure-free (Engel class I), and one rarely has seizures (Engel class II). All seven patients had frequent seizures that led to IM: either daily (five patients) or 1-2 per week (two patients). The mean age (± standard deviation, SD) at seizure onset was 1.6 ± 1.3 years (range 0.5-4 years). Etiologies were tuberous sclerosis (3 patients), trauma (1 patient), and unknown (3 patients). Mean age at surgery (± SD) was 4.1 ± 2 years (range 1-7 years), and duration of epilepsy 2.5 ± 1.1 years (range 0.5-4 years). Duration of IM was 11.7 ± 5.6 days (5-19 days). Six patients had bilateral and one unilateral invasive electrodes. At last follow-up, four patients required fewer antiepileptic drugs (AEDs), one had the same medication but a higher dose, and two patients were taking additional AEDs. Follow-up was 30.6 ± 9.5 months (range 19-41 months). SIGNIFICANCE: Although uncommon, patients with TRE may improve after IM alone. The explanation for this observation remains unclear; however, perioperative medications including steroids, direct cortical manipulation, or other factors may influence the epileptogenic network.

Improvement in quality of life after adenotonsillectomy in a child with Prader Willi syndrome.

INTRODUCTION: We report a child with Prader Willi syndrome who developed obstructive sleep apnea (OSA). This patient underwent surgical treatment for OSA. There was improvement not only on her OSA but in her quality of life score as well. This report highlights the need for a comprehensive assessment in the management of patients with Prader Willi syndrome.

PmLT, a C-type lectin specific to hepatopancreas is involved in the innate defense of the shrimp Penaeus monodon.

A diverse class of proteins called lectins plays a major role in shrimp innate immunity. In this study, the cDNA encoding a C-type lectin of Penaeus monodon (PmLT) was cloned, and its potential role examined. Despite the low overall amino acid sequence identity with other animal lectins, PmLT includes conserved carbohydrate recognition domains (CRDs) characteristic of animal C-type lectins. Unlike the other two P. monodon lectin-like proteins described to date that have one CRD, PmLT has two CRDs. The first CRD contains a QPD motif with specificity for binding galactose, while the second CRD contains a EPN motif for binding mannose. PmLT transcripts can be detected in the hepatopancreas but not in other tissues. Expression studies showed that PmLT mRNA transcript level decreased initially and then gradually increased after whole shrimp or hepatopancreas tissue fragments were treated with white spot syndrome virus (WSSV) extract but were not affected by bacteria. Using anti-rPmLT antibody, PmLT was detected only in the hepatopancreas specific F cells (Hpf). In vitro encapsulation assay showed that agarose beads coated with rPmLT were encapsulated by hemocytes indicating a role in innate immune response. In summary, PmLT is produced in the hepatopancreas and may act as a pattern recognition protein for viral pathogens and also activates the innate immune responses of the shrimp to bacteria. The dual-CRD structure of PmLT may assist the recognition of diverse pathogens.

Pre-optimization of spinal surgery patients: Development of a neurosurgical enhanced recovery after surgery (ERAS) protocol.

OBJECTIVE: Despite surgical, technological, medical, and anesthetic improvements, patient outcomes following elective neurosurgical procedures can be associated with high morbidity. Enhanced recovery after surgery (ERAS) protocols are multimodal care pathways designed to optimize patient outcomes by addressing pre-, peri-, and post-operative factors. Despite significant data suggesting improved patient outcomes with the adoption of these pathways, development and implementation has been limited in the neurosurgical population. METHODS/RESULTS: This study protocol was designed to establish the feasibility of a randomized controlled trial to assess the efficacy of implementation of an ERAS protocol on the improvement of clinical and patient reported outcomes and patient satisfaction scores in an elective inpatient spine surgery population. Neurosurgical patients undergoing spinal surgery will be recruited and randomly allocated to one of two treatment arms: ERAS protocol (experimental group) or hospital standard (control group). The experimental group will undergo interventions at the pre-, peri-, and post-operative time points, which are exclusive to this group as compared to the hospital standard group. CONCLUSIONS: The present proposal aims to provide supporting data for the application of these specific ERAS components in the spine surgery population and provide rationale/justification of this type of care pathway. This study will help inform the design of a future multi-institutional, randomized controlled trial. RESULTS: of this study will guide further efforts to limit post-operative morbidity in patients undergoing elective spinal surgery and to highlight the impact of ERAS care pathways in improving patient reported outcomes and satisfaction.

Chinese translation and validation of the Sport Concussion Assessment Tool 3 (SCAT3).

OBJECTIVE: The aim of this study was to translate, adapt and validate the Sport Concussion Assessment Tool 3rd edition (SCAT3), a test for assessing athletes for concussion, into the Chinese context. METHODS: Translation and adaptation were performed in several stages, which included forward translation by two independent teams, translation merging, backward translation, reviews by both native Cantonese-speaking and Mandarin-speaking multidisciplinary expert panels (n=49) for semantic and conceptual equivalence and reviews by pitch-side physiotherapists (n=18) as end-users of the SCAT3 and rugby players (n=11) for face validity. The Serial 3 s subtraction test was used as a substitute for the Months Backward Test (MBT) for measures of concentration in the Standardized Assessment of Concussion subscale. English-speaking and Chinese-speaking rugby players (n=52) were recruited to perform these tests to assess the level of difficulty, time for completion and accuracy. Inter-rater and test-retest reliability were assessed in 33 and 38 healthy young individuals, respectively. RESULTS: Despite the longer mean completion time (p<0.05) for the Serial 3 s test, no significant difference was found in the percentage accuracy between MBT and the Serial 3 s test. No significant difference was found in either the percentage accuracy or completion time between English-speaking and Cantonese-speaking rugby players. All subscales in the Chinese SCAT3 had excellent levels of inter-rater reliability for all items (ICC2,1 range: 0.96-0.99) but a low to moderate test-retest reliability (ICC3,2 range: 0.32-0.65). The mean completion time of the Chinese SCAT3 was 10.6±1.1 min. CONCLUSION: Chinese SCAT3 is a valid instrument for pitch-side assessment of concussed Chinese-speaking athletes.

A simplified pressure adjustment clinical pathway for programmable valves in NPH patients.

OBJECTIVE: The goal of this study is to provide a clinical pathway for shunt valve adjustment in the treatment of normal pressure hydrocephalus (NPH) patients. PATIENT AND METHODS: The authors conducted a single-center retrospective study of 102 patients (mean age 74 years, 66 men, 36 women) diagnosed with NPH. In all cases, a Medtronic Strata Adjustable Pressure valve set initially at 1.5 was implanted. Outcome was based on the clinical status of the patient at the last contact point with the senior author. Patients were adjusted with reductions or increases of 0.5 per follow-up visit to achieve the best clinical outcome and avoid complications. Complications were categorized as infection, shunt malfunction, subdural hygroma/hematoma, or any adverse event able to be attributed to a change in shunt setting or surgical procedure. RESULTS: Of the 102 patients, 60% had the triad of clinical symptoms, 5% had gait dysfunction only, 2% had dementia only, 4% had urine incontinence and gait dysfunction, 1% had urine incontinence and dementia, and 28% had gait dysfunction and dementia. Over a mean clinical follow-up of 19 months, 71 patients had improvement or resolution of NPH symptoms at the last point of contact with the senior author. Of the 71 patients, 24% improved in all 3 symptoms, 8% improved in urine incontinence only, 17% improved in gait dysfunction only, 15% improved in dementia only, 15% improved in urine incontinence and gait dysfunction only, 4% improved in urine incontinence and dementia only, and 15% improved in gait dysfunction and dementia only. Valve pressure adjustment was required at least once in 85 patients (mean number of 1.68 adjustments, 7 maximum); 10% had 0.5 as the final setting, 47% had 1.0 as the final setting, 36% had 1.5 as the final setting, 7% had 2.0 as the final setting, and 0% had 2.5 as the final setting. There were 41 (40%) complications overall: 28 subdural hygromas/hematomas, of which 5 required surgical evacuation; 9 distal obstructions requiring surgical revision; 4 seizures; 2 infections; 1 exposed shunt tubing; 1 intraparenchymal hematoma of unknown etiology. CONCLUSION: Standardization of post-operative care for patients with NPH is possible. The present manuscript offers a safe and effective pathway for treatment of NPH patients with the Strata Adjustable Pressure Valve.

Direct versus indirect revascularization procedures for moyamoya disease: a comparative effectiveness study.

OBJECTIVE Moyamoya disease (MMD) is a chronic cerebrovascular disease that can lead to devastating neurological outcomes. Surgical intervention is the definitive treatment, with direct, indirect, and combined revascularization procedures currently employed by surgeons. The optimal surgical approach, however, remains unclear. In this decision analysis, the authors compared the effectiveness of revascularization procedures in both adult and pediatric patients with MMD. METHODS A comprehensive literature search was performed for studies of MMD. Using complication and success rates from the literature, the authors constructed a decision analysis model for treatment using a direct and indirect revascularization technique. Utility values for the various outcomes and complications were extracted from the literature examining preferences in similar clinical conditions. Sensitivity analysis was performed. RESULTS A structured literature search yielded 33 studies involving 4197 cases. Cases were divided into adult and pediatric populations. These were further subdivided into 3 different treatment groups: indirect, direct, and combined revascularization procedures. In the pediatric population at 5- and 10-year follow-up, there was no significant difference between indirect and combination procedures, but both were superior to direct revascularization. In adults at 4-year follow-up, indirect was superior to direct revascularization. CONCLUSIONS In the absence of factors that dictate a specific approach, the present decision analysis suggests that direct revascularization procedures are inferior in terms of quality-adjusted life years in both adults at 4 years and children at 5 and 10 years postoperatively, respectively. These findings were statistically significant (p < 0.001 in all cases), suggesting that indirect and combination procedures may offer optimal results at long-term follow-up.

Ultrasonographic Evaluation of Peripheral Nerves.

There are a variety of imaging modalities for evaluation of peripheral nerves. Of these, ultrasonography (US) is often underused. There are several advantages of this imaging modality, including its cost-effectiveness, time-efficient assessment of long segments of peripheral nerves, ability to perform dynamic maneuvers, lack of contraindications, portability, and noninvasiveness. It can provide diagnostic information that cannot be obtained by electrophysiologic or, in some cases, magnetic resonance imaging studies. Ideally, the neurosurgeon can use US as a diagnostic adjunct in the preoperative assessment of a patient with traumatic, neoplastic, infective, or compressive nerve injury. Perhaps its most unique use is in intraoperative surgical planning. In this article, a brief description of normal US nerve anatomy is presented followed by a description of the US appearance of peripheral nerve disease caused by trauma, tumor, infection, and entrapment.

Vitellogenesis in the red crab, Charybdis feriatus: contributions from small vitellogenin transcripts (CfVg) and farnesoic acid stimulation of CfVg expression.

During reproductive maturation of the female red crab, Charybdis feriatus, the oocytes rapidly accumulate 110- and 78-kDa major polypeptides. Although the hepatopancreas expresses a high level of vitellogenin (CfVg) mRNA, tissue proteins and secreted proteins of the hepatopancreas consist of only small polypeptides. In addition to the 8.0-kb transcripts, many smaller mRNAs specific to the CfVg gene can be detected. These results suggest that the hepatopancreas also produces smaller CfVg transcripts for small CfVg subunits. Using an RT-PCR cloning approach, a population of the small cDNA clones were isolated. Determining the DNA sequence of these clones revealed that these transcripts were most likely the result of alternative splicing and/or alternative expression of the CfVg gene. In vitro treatment of the hepatopancreas fragments with low levels of farnesoic acid stimulated the expression of CfVg.

Resective Epilepsy Surgery for Tuberous Sclerosis in Children: Determining Predictors of Seizure Outcomes in a Multicenter Retrospective Cohort Study.

BACKGROUND: There are no established variables that predict the success of curative resective epilepsy surgery in children with tuberous sclerosis complex (TSC). OBJECTIVE: We performed a multicenter observational study to identify preoperative factors associated with seizure outcome in children with TSC undergoing resective epilepsy surgery. METHODS: A retrospective chart review was performed in eligible children at New York Medical Center, Miami Children's Hospital, Cleveland Clinic Foundation, BC Children's Hospital, Hospital for Sick Children, and Sainte-Justine Hospital between January 2005 and December 2013. A time-to-event analysis was performed. The "event" was defined as seizures after resective epilepsy surgery. RESULTS: Seventy-four patients (41 male) were included. The median age of the patients at the time of surgery was 120 months (range, 3-216 months). The median time to seizure recurrence was 24.0 ± 12.7 months. Engel Class I outcome was achieved in 48 (65%) and 37 (50%) patients at 1- and 2-year follow-up, respectively. On univariate analyses, younger age at seizure onset (hazard ratio [HR]: 2.03, 95% confidence interval [CI]: 1.03-4.00, P = .04), larger size of predominant tuber (HR: 1.03, 95% CI: 0.99-1.06, P = .12), and resection larger than a tuberectomy (HR: 1.86, 95% CI: 0.92-3.74, P = .084) were associated with a longer duration of seizure freedom. In multivariate analyses, resection larger than a tuberectomy (HR: 2.90, 95% CI: 1.17-7.18, P = .022) was independently associated with a longer duration of seizure freedom. CONCLUSION: In this large consecutive cohort of children with TSC and medically intractable epilepsy, a greater extent of resection (more than just the tuber) is associated with a greater probability of seizure freedom. This suggests that the epileptogenic zone may include the cortex surrounding the presumed offending tuber.

PmPPAF is a pro-phenoloxidase activating factor involved in innate immunity response of the shrimp Penaeus monodon.

One of the major steps in the innate immune response of shrimp includes the activation of serine proteinases of the pro-phenoloxidase pathway by the prophenoloxidase activation enzyme (PPAF). In this study, the cDNA encoding a serine proteinase homologue (SPH) with prophenoloxidase activating activity of Penaeus monodon (PmPPAF) was cloned and characterized. PmPPAF cDNA consists of 1444 nucleotides encoding a protein with 394 amino acid residues. The estimated molecular weight of PmPPAF is 43.5 kDa with an isoelectric point of 5.19. PmPPAF consists of a signal peptide, a CLIP domain and a carboxyl-terminal trypsin-like serine protease domain. It is highly similar to the masquerade-like protein 2A (61% similarity) of the crayfish Pacifastacus leniusculus, other serine proteases (42.9-67% identity) of P. monodon, and the PPAF of the crab (61% similarity). Unlike other SPH of P. monodon, which express mainly in the hemocytes, PmPPAF transcripts were detected in the hemocytes, eyestalk, hypodermis, gill, swimming leg and brain. Similar to the crab PPAF, PmPPAF transcript level is high in shrimp at the premolt stages and PmPPAF expression is up-regulated in shrimp infected with white spot syndrome virus (WSSV). Gene silencing of PmPPAF decreased expression of a prophenoloxidase-like gene and injection of Anti-PmPPAF antibody causes a decrease in PO activity. Taken together, these results provided evidence that PmPPAF is a serine proteinase homologue, and is involved in the pro-PO activation pathway of the shrimp innate immune system.

Atlantoaxial fusion with screw-rod constructs: meta-analysis and review of literature.

OBJECTIVE: To review published series describing C1-2 posterior instrumented fusions and summarize clinical and radiographic outcomes of patients treated with screw-rod constructs (SRC). METHODS: Online databases were searched for English-language articles published between 1991 and April 2011 describing posterior atlantoaxial instrumentation with C1-2 SRC. There were 24 studies including 1073 patients treated with SRC that fulfilled inclusion criteria. Meta-analysis techniques were used to compare outcomes. RESULTS: All studies provided class III evidence. The 30-day perioperative mortality rate was 0.6%, and neurologic injury occurred in two patients with vertebral artery injury (VAI) from screw malpositions (0.2%). The incidence of clinically significant screw malpositions was 2.4% (confidence interval [CI], 1.1%-4.1%), the incidence of VAI was 2.0% (CI, 1.1%-3.4%), and the rate of fusion with the SRC technique was 97.5% (CI, 95.9%-98.5%). CONCLUSIONS: SRC is a safe and effective treatment option for C1-2 instability. The low but nonzero incidence of screw malposition and VAI emphasizes the necessity of having a thorough knowledge of atlantoaxial anatomy for successful insertion of screws.

Atlantoaxial fusion with transarticular screws: meta-analysis and review of the literature.

OBJECTIVE: To review published series describing C1-2 posterior instrumented fusions and summarize clinical and radiographic outcomes of patients treated with transarticular screw (TAS) fixation. METHODS: Online databases were searched for English-language articles published between 1986 and April 2011 describing posterior atlantoaxial instrumentation with C1-2 TAS fixation. There were 45 studies including 2073 patients treated with TAS that fulfilled inclusion criteria. Meta-analysis techniques were used to calculate outcomes. RESULTS: All studies provided class III evidence. The 30-day perioperative mortality rate was 0.8%, and the incidence of neurologic injury was 0.2%. The incidence of clinically significant malpositioned screws was 7.1% (confidence interval [CI], 5.7%-8.8%), the incidence of vertebral artery injury was 3.1% (CI, 2.3%-4.3%), and the rate of fusion with the TAS technique was 94.6% (CI, 92.6%-96.1%). CONCLUSIONS: TAS fixation is a safe and effective treatment option for C1-2 instability with high rates of fusion (approximately 95%). Screw malposition and vertebral artery injury occurred in approximately 5% of patients. The successful insertion of TAS requires a thorough knowledge of atlantoaxial anatomy.

Is external cervical orthotic bracing necessary after posterior atlantoaxial fusion with modern instrumentation: meta-analysis and review of literature.

BACKGROUND: No guidelines exist regarding external cervical orthoses (ECO) after atlantoaxial fusion. We reviewed published series describing C1-2 posterior instrumented fusions with screw-rod constructs (SRC) or transarticular screws (TAS) and compared rates of fusion with and without postoperative ECO. METHODS: Online databases were searched for English-language articles between 1986 and April 2011 describing ECO use after posterior atlantoaxial instrumentation with SRC or TAS. Eighteen studies describing 947 patients who had SRC (± ECO: 254 of 693 patients), and 33 studies describing 1424 patients with TAS (± ECO: 525 of 899 patients) met inclusion criteria. Meta-analysis techniques were applied to estimate rates of fusion with and without ECO use. RESULTS: All studies provided class III evidence, and no studies directly compared outcomes with or without ECO use. There was no significant difference in the proportion of patients who achieved successful fusion between patients treated with ECO and without ECO for SRC or TAS patients. Point estimates and 95% confidence intervals (CI) for rates of fusion ± ECO were 97.4% (CI: 95.2% to 98.6%) versus 97.9% (CI: 93.6% to 99.3%) for SRC and 93.6% (CI: 90.7% to 95.6%) versus 95.3% (CI: 90.8% to 97.7%) for TAS. There was no correlation between duration of ECO treatment and fusion (dose effect). CONCLUSIONS: After C1-2 fusion with modern instrumentation, ECO may be unnecessary (class III). Some centers recommend ECO use with patients with softer bone quality (class IV). Prospective, randomized studies with validated radiographic and clinical outcome metrics are necessary to determine the utility of ECO after C1-2 fusion and its impact on patient comfort and cost.

Electrocorticographic evidence of perituberal cortex epileptogenicity in tuberous sclerosis complex.

OBJECT: Tuberous sclerosis complex (TSC) is a multisystem autosomal dominant disorder resulting in hamartomas of several organs. Cortical tubers are the most prominent brain lesions in TSC. Treatment-resistant epilepsy often develops early in life in patients with TSC and is associated with severe intellectual and behavioral impairments. Seizures may remit following epilepsy surgery in selected cases, yet it remains unclear whether the tuber or the perituberal cortex is the source of seizure onset. In this study, the authors reviewed the onset of seizures in patients in whom depth electrodes had been placed within or adjacent to cortical tubers. METHODS: After obtaining institutional review board approval, the authors retrospectively reviewed data from 12 pediatric patients with multifocal TSC and treatment-resistant epilepsy who had undergone invasive intracranial electroencephalographic monitoring. Tubers were identified on postimplantation MRI, and all depth electrodes were located. Depth electrode contacts were classified visually as either tuber/perituberal cortex or nontuber/nonperituberal cortex. Board-certified clinical neurophysiologists reviewed the seizures to identify all electrodes involved in the ictal onset. RESULTS: Among 309 recorded seizures, 104 unique ictal onset patterns were identified. Of the 11 patients with electrodes recording in a tuber, 9 had seizure onsets involving the tuber. Similarly, of the 9 patients with perituberal recording electrodes, 7 had perituberal ictal onsets. Overall, there was no difference in the percentage of contacts involved in seizure onset between the tuber and perituberal cortex. In a subset of 7 patients in whom at least 1 depth electrode contact was within the tuber and 1 was in the perituberal cortex, there was no difference between the percentage of tuber and perituberal onsets. CONCLUSIONS: Findings demonstrated heterogeneity in the ictal onset patterns as well as involvement of the tuber and perituberal cortex within and between patients. Although the data are limited by the restricted region(s) sampled with intracranial electrodes, they do suggest that cortical hyperexcitability in TSC may derive from the tuber or surrounding cortex.

Personality traits and social behaviors predict the psychological adjustment of Chinese people with epilepsy.

Very little is known about the psychosocial correlates of psychological morbidity among Chinese people with epilepsy (PWE). No studies have investigated the association between social relationships and psychological morbidity, while most studies examined only the negative impact of maladaptive personalities on psychological adjustment in PWE. This study examined the association of psychological morbidity with a broad array of personality traits and social skills in a sample of 54 Chinese PWE. Respondents completed the Temperament and Character Inventory (TCI), the Social Performance Survey Schedule (SPSS), and the Hospital Anxiety and Depression Scale (HADS) via semi-structured interview. Regression analyses revealed that, independent of demographic and medical variables and perceived impact, Harm Avoidance was positively associated with anxiety and depression whereas Self-Directedness was negatively associated with anxiety and depression; that Cooperativeness was inversely associated with anxiety. Social skills were inversely associated with depression whereas negative social skills were inversely associated with anxiety. Clinical implications of adaptive personality traits and social skills functioning are discussed.