Brain and Spinal Tumors Originating from the Germ Line Cells.

Primary central nervous system germ cell tumors (CNS GCTs) are part of the GCTs in children and adults. This tumor entity presents with geographic variation, age, and sex predilection. There are two age peaks of incidence distribution at the first few months of life and in adolescence. CNS GCTs are heterogeneous in histopathological subtypes, locations, and tumor marker (AFP, ß-hCG) secretions. In the WHO CNS tumor classification, GCTS are classified as germinoma and nongerminomatous GCT (NGGCT) with different subtypes (including teratoma). Excluding mature teratoma, the remaining NGGCTs are malignant (NGMGCT). In teratoma, growing teratoma syndrome and teratoma with somatic-type malignancy should be highlighted. The common intracranial locations are pineal region, neurohypophysis (NH), bifocal pineal-NH, basal ganglia, and cerebral ventricle. Above 50% of intracranial GCTs (IGCTs) present obstructive hydrocephalus. Spinal tumors are rare. Age, locations, hydrocephalus, and serum/CSF titer of ß-hCG correlate with clinical manifestations. Delayed diagnosis is common in tumors arising in neurohypophysis, bifocal, and basal ganglia resulting in the increasing of physical dysfunction and hormonal deficits. Staging work-up includes CSF cytology for tumor cells and contrast-enhanced MRI of brain and spine for macroscopic metastasis before treatment commences. The therapeutic approach of CNS GCTs integrates locations, histopathology, staging, tumor marker level, and therapeutic classification. Treatment strategies include surgical biopsy/excision, chemotherapy, radiotherapy (single or combination). Secreting tumors with consistent imaging may not require histopathological diagnosis. Primary germinomas are highly radiosensitive and the therapeutic aim is to maintain high survival rate using optimal radiotherapy regimen with/without chemotherapy combination. Primary NGNGCTs are less radiosensitive. The therapeutic aim is to increase survival utilizing more intensive chemotherapy and radiotherapy. The negative prognostic factors are residue disease at the end of treatment and serum or CSF AFP level >1000 ng/mL at diagnosis. In refractory or recurrent NMGGCTs, besides high-dose chemotherapy, new therapy is necessary. Molecular profiling and analysis help for translational research. Survivors of pediatric brain tumors frequently experience cancer-related cognitive dysfunction, physical disability, pituitary hormone deficiency, and other CNS complications after cranial radiotherapy. Continuous surveillance and assessment may lead to improvements in treatment protocols, transdisciplinary interventions, after-treatment rehabilitation, and quality of life.

Correlation between intraoperative mapping and monitoring and functional outcomes following supratentorial glioma surgery.

OBJECTIVES: Intraoperative neurophysiological monitoring (IONM) has long been regarded as the "gold standard" when resecting a supratentorial glioma, as it facilitates the goals of maximal tumor resection and preservation of sensorimotor function. The purpose of the present study was to evaluate the ability of motor evoked potentials (MEPs) monitoring or subcortical mapping (SCM), alone or in combination, to predict postoperative functional outcomes in glioma surgery. MATERIALS AND METHODS: We retrospectively reviewed patients with supratentorial glioma that underwent craniotomy for tumor removal with IONM. Statistical analyses were used to evaluate whether the following criteria correlated with postoperative functional outcomes: Reduced amplitude (>50% reduction) or disappearance of MEPs (criterion 1), SCM with a stimulation intensity threshold less than 3 mA (criterion 2), the presence of both two phenomena (criterion 3), or either one of the two phenomena (criterion 4). RESULTS: Ninety-two patients were included in this study, of whom 15 sustained new postoperative deficits, 4 experienced improved functional status, and 73 were unchanged. Postoperative functional status correlated significantly with all four criteria, and especially with criterion 3 (r = 0.647, P = 0.000). Sensitivity of IONM was better if using criteria 2 and 4, but specificity was better if using criteria 1 and 3. Criterion 3 had the most favorable overall results. CONCLUSION: Using statistical methodology, our study indicates that concomitant interpretation of MEPs and SCM is the most accurate predictor of functional outcomes following supratentorial glioma surgery. However, accurate interpretations of the monitoring results by experienced neurophysiologists are essential.

Impact of extent of internal acoustic meatus tumor removal using translabyrinthine approach for acoustic neuroma surgery.

OBJECTIVES: Many studies have investigated the surgical outcome and predictive factors of acoustic neuroma using different approaches. The present study focused on large tumors due to the greater likelihood of internal acoustic meatus involvement and the greater application of surgical intervention than radiosurgery. There have been no previous reports on outcomes of internal acoustic meatus tumor removal. We investigated the impact of the extent of internal acoustic meatus tumor removal using a translabyrinthine approach for large acoustic neuroma surgery and predictive factors of tumor control. METHODS: This retrospective study reviewed 104 patients with large cerebellopontine angle tumor >3 cm treated by translabyrinthine approach microsurgery. Predictive factors of postoperative facial palsy, tumor control, and extent of internal acoustic meatus tumor removal were assessed. RESULTS: The mean tumor size was 38.95 ± 6.83 mm. Postoperative facial function showed 76.9% acceptable function (House-Brackmann grade 1 or 2) six months after surgery. The extent of internal acoustic meatus tumor removal was a statistically significant predictor factor of poor postoperative facial function. Younger age, larger tumor size needing radiosurgery, and more extensive removal of tumor were associated with better tumor control. CONCLUSION: More extensive internal acoustic meatus tumor removal was associated with poor postoperative facial function and better tumor control.

Blink synkinesis monitoring during microvascular decompression for hemifacial spasm.

BACKGROUND: In the present study, we investigated whether blink synkinesis monitoring during microvascular decompression (MVD) is effective for predicting long-term outcomes in patients with hemifacial spasm (HFS). METHODS: This retrospective study included 69 patients who had undergone MVD for HFS at a tertiary hospital. All patients underwent intraoperative monitoring of blink synkinesis, lateral spread responses (LSRs), and facial nerve motor-evoked potentials (FNMEPs). Baseline signals were compared to those obtained following decompression with Teflon, and postoperative outcomes were recorded. RESULTS: A total of 65 patients were observed with complete relief of symptoms after 1 year after MVD, while 61 patients were observed with initial disappearance of blink synkinesis, 57 patients were observed with initial elimination of the LSR, and 45 patients with initial decreases in FNMEP amplitude (>50%). The highest sensitivity and accuracy values were observed for blink synkinesis. Chi-square tests comparing the sensitivity of the three methods revealed that FNMEP monitoring was associated with significantly lower sensitivity values than the remaining methods. Combined use of blink synkinesis and LSRs did not significantly increase sensitivity (61/65 vs 62/65) or accuracy (62/69 vs 63/69). CONCLUSION: Our results demonstrate that blink synkinesis monitoring is safe during MVD for HFS. Furthermore, blink synkinesis was associated with the highest sensitivity and predictive values among the three methods evaluated. These findings suggest that blink synkinesis can be regarded as the first choice for intraoperative monitoring during MVD. Concurrent use of blink synkinesis and LSR monitoring may maximize the ability to predict patient prognosis and determine the extent of decompression.

Health-related quality of life in children and adolescent with different types of scoliosis: A cross-sectional study.

BACKGROUND: The health-related quality of life (HRQoL) was affected in children and adolescents with scoliosis. However, there was lack of study to compare the HRQoL among patients with different types of scoliosis. We aimed to investigate whether the HRQoL differs among patients with idiopathic, congenital, neuromuscular, and syndromic scoliosis. METHODS: Children and adolescents with scoliosis were recruited from a single tertiary hospital. The HRQoL, as assessed by the child health questionnaire 50-item parent form, was compared with a reference health sample group using the effect size (ES). Intergroup differences related to scoliosis subtype and severity were explored. RESULTS: A total of 67 participants with scoliosis (24 idiopathic, 15 congenital, 15 neuromuscular, and 13 syndromic) were analyzed. The HRQoL in patients with neuromuscular scoliosis was affected the most, in both physical (ES range: 0.97-2.4) and psychosocial domains (ES range: 0.92-2.58). To a lesser extent, the physical (ES range: 0.99-1.13) and psychosocial (ES range: 0.8-1.18) domains were also affected in patients with syndromic scoliosis. The domains of family activities (ES = 1.1), role/social-emotional/behavioral (ES = 0.99), general health perception (ES = 0.94), and self-esteem (ES = 0.87) were affected in patients with idiopathic scoliosis. In contrast, only the general health perception domain (ES = 1.27) was affected in patients with congenital scoliosis. Scoliosis severity correlated with scores in the physical domains and some psychosocial domains, while treatment type correlated with scores in the physical domains only. Scoliosis subtype and severity both affected the physical and psychosocial domains, with a stronger impact for subtype. CONCLUSION: Differences in the HRQoL exist among scoliosis subtypes, with neuromuscular scoliosis being most affected. Although the scoliosis subtype and severity both affect the HRQoL, the subtype is more influential than severity.

Early and anticipatory postural adjustments in healthy subjects under stable and unstable sitting conditions.

Clinicians frequently incorporate unstable sitting devices into training plans for improving proximal postural muscle control; however, the effect of unstable sitting conditions on postural adjustments during dynamic activities has not been fully explored. The aim of this study was to characterize early postural adjustments (EPAs) and anticipatory postural adjustments (APAs) under stable and unstable sitting conditions. Using a cross-sectional laboratory study design, 13 healthy college student volunteers used their dominant hand to reach forward and push a target under stable and unstable sitting conditions; subjects sat on an air-filled rubber cushion for the unstable condition. EPAs and APAs were quantified by recording muscle activation of the trunk and lower extremity muscles using electromyography (EMG). The center of pressure (COP) was measured using a force plate. The resulting EMG integral of the ipsilateral gastrocnemius muscle was larger during the EPA phase and smaller during the APA phase under unstable conditions (p = 0.014 and p = 0.041, respectively). COP amplitude in the anterior-posterior direction, path length, and velocity, was larger during the APA phase (p = 0.035, p = 0.023, and 0.023, respectively). This suggests greater distal muscle activation during EPAs in unstable sitting conditions, specifically in the ipsilateral gastrocnemius muscle. In addition, APAs adjusted by reducing the activity of the ipsilateral gastrocnemius muscle and increasing the anterior-posterior shift in the COP to compensate for the expected additional perturbation due to an unstable surface.

Using subdural strip electrodes to define functional sensory nerves and the most inferior functional portion of the conus medullaris during detethering surgeries for tethered cord syndrome: a pilot study.

OBJECTIVE: The aim of this study was to investigate the feasibility of using subdural strip electrodes, placed just rostral to the surgical field, to record sensory evoked potentials (SEPs) from the lumbosacral sensory nerves and define the most inferior functional portion of the conus medullaris during detethering surgery for spinal dysraphism and/or tethered cord syndrome (TCS). METHODS: Six patients, 2 boys and 4 girls, aged 0.5 to 16 years, were enrolled in this study. One patient had lipomyelomeningocele-related, 4 had myelomeningocele-related, and 1 had diastematomyelia and lipomyelomeningocele-related TCS. In addition to the routine preparations that are needed for performing functional mapping and monitoring during surgery for spinal dysraphism and TCS, the patients had a 1 × 4 strip of electrodes placed rostral to the surgical field, where it was secured by a surgeon after opening the dura. With the patient under total intravenous anesthesia, the sensory nerves and conus medullaris were stimulated with a concentric bipolar electrode over the surgical field while SEPs were recorded with the strip electrodes to identify any possible sensory roots with remaining function and the most inferior functional portion of the conus medullaris. RESULTS: The SEP amplitudes that were recorded with the subdural strip electrodes ranged from 4 to 400 µV, and the responses to sensory nerve stimulation were frequently much larger than were those to conus stimulation. Use of the SEP recordings for sensory mapping along with the routine mapping and monitoring techniques allowed detethering to be completed such that none of the patients sustained any new functional deficit after surgery. CONCLUSIONS: Recording SEPs from the functional sensory nerves and conus medullaris through subdural strip electrodes proved to be a feasible and valuable tool during detethering surgery in young patients. This approach may help surgeons achieve maximal detethering while preserving important sensory functions, consequently retaining the patient's quality of life.

Pattern of Corticospinal Projections Defined by Brain Mapping During Resective Epilepsy Surgery in a Patient with Congenital Hemiparesis and Intractable Epilepsy.

BACKGROUND: Congenital or early-onset brain structural lesions often cause contralateral hemiparesis, cognitive deficits, developmental delays, and seizures. Seizure is the most debilitating condition, as it greatly impairs quality of life in both the affected individuals and their caregivers and prevents them from active social participation. CASE DESCRIPTION: A 34-year-old man with hemiparesis and early-onset seizures since childhood owing to a congenital brain lesion developed intractable seizures in the last 2 years and was subsequently admitted for resective epileptic surgery. During the operation, we employed an innovative intraoperative neurophysiologic monitoring technique. In contrast to routine application for transcranial stimulation, we recorded compound muscle action potentials over the bilateral limb muscles simultaneously, instead of over the contralateral muscles only, to determine the patterns of the corticospinal projections. Transcranial stimulation over the bilateral hemispheres was applied before craniotomy, and direct cortical stimulation over the lesioned hemisphere was applied after craniotomy. By integrating both approaches, we could first identify the pattern of corticospinal projections before craniotomy and then accurately define the noneloquent area, which guided the resection to successfully accomplish the surgical goal. CONCLUSIONS: This technique is simple because no patient participation is required. We believe that it has the potential to replace conventional preoperative functional magnetic resonance imaging and transcranial magnetic stimulation in resective epilepsy surgery, particularly for young patients. Not only can it improve the safety of surgical procedures, but also it can help predict functional outcome.

Localization of the trunk muscles using musculoskeletal ultrasound guidance for pedicle screw stimulation during spine surgery.

The precise placement of recording electrodes at the relevant myotome is mandatory while performing pedicle screw stimulation (PSS) during spine surgery; however, their placement at trunk muscles is challenging. This study aimed to determine whether ultrasound guidance is useful for trunk muscle localization for PSS during spine surgery. A retrospective clinical study was conducted from a prospective database. Eighty-four patients eligible for spine surgery were recruited. Ultrasound was used to localize the intercostal, rectus abdominis, and internal oblique and psoas muscles if pedicle screw placement was performed at T3 to L1. After the operation, patients were examined for any new neurological deficits related to this procedure, and computed tomography was performed to check screw position if indicated. Four to 22 pedicle screws were used for spinal fixation. The threshold of stimulus to obtain a compound muscle action potential ranged from 1.29 to >20mA during PSS. Six of our patients sustained new postoperative deficits, and only one case was related directly to pedicel screw misplacement. Loss of motor evoked potential (MEP) over both the lower limbs was noted during pedicle screw placement, and the stimulus threshold during PSS were 1.29mA at the left T9 and 3.8mA at the right T5 level. MEP remained absent at the end of surgery despite removal of those two screws. The patient woke with significant weakness in both lower limbs (muscle power 0/0) and voiding difficulty. Fortunately, he regained walking ability 4.5months later after intensive rehabilitation therapy.

Acute Sensory Neuronopathy following Enterovirus Infection in a 3-Year-Old Girl.

Acute sensory neuronopathy (SNN) is a rapidly developing peripheral nervous system disease that primarily affects sensory neurons in the dorsal root ganglion or trigeminal ganglion, leading to the impairment of sensory axons. SNN is notably uncommon in childhood; only three cases of childhood or adolescent SNN have been reported to date. Moreover, SSN has never been reported in association with enterovirus infection. Here, we report the case of a 3-year-old girl who was initially diagnosed with enterovirus infection based on the presentation of fevers, rashes on all extremities, and ulceration over the posterior pharynx. Nine days later, she presented with ataxic and wide-based gait and dysmetria affecting the extremities, with an absence of sensory nerve action potentials in the upper and lower limbs. The patient was diagnosed with acute SNN based on the criteria developed by Camdessanché et al in 2009. To our knowledge, this is the youngest case of SNN reported to date. In addition, this case reveals that enterovirus infection can be associated with acute SNN in children in rare cases. Accurate diagnosis relies on clinical suspicion, comprehensive knowledge of the patient's history, and careful characterization of abnormal findings in electrodiagnostic studies.

Efficacy of Combined Ultrasound-Guided Steroid Injection and Splinting in Patients With Carpal Tunnel Syndrome: A Randomized Controlled Trial.

OBJECTIVE: To compare the effectiveness of local steroid injection plus splinting with that of local steroid injection alone using clinical and electrophysiological parameters in patients with carpal tunnel syndrome (CTS). DESIGN: Randomized controlled study with 12 weeks of follow-up. SETTING: Tertiary care center. PARTICIPANTS: Volunteer sample of patients (N=52) diagnosed with CTS. INTERVENTIONS: Participants were randomly assigned to the steroid injection group (n=26) or the steroid injection-plus-splinting group (n=26). Patients of both groups received ultrasound-guided steroid injection with 1mL of 10mg (10mg/mL) triamcinolone acetonide (Shincort) and 1mL of 2% lidocaine hydrochloride (Xylocaine). Participants in the second group also wore a volar splint in the neutral position while sleeping and also during daytime whenever possible for the 12-week intervention period. MAIN OUTCOME MEASURES: Participants were evaluated before the treatment and at 6 and 12 weeks after the onset of treatment. The primary outcome measure was Boston Carpal Tunnel Questionnaire scores. The secondary outcome measures were as follows: scores on the visual analog scale for pain; electrophysiological parameters, including median nerve distal motor latency, sensory nerve conduction velocity (SNCV), and compound muscle action potential and sensory nerve action potential (SNAP) amplitudes; and patient's subjective impression of improvement. RESULTS: At 12-week follow-up, improvements in symptom severity and functional status scores on the Boston Carpal Tunnel Questionnaire as well as SNCV and SNAP amplitudes were greater in the group that received steroid injection combined with splinting than in the group that received steroid injection alone. The between-group difference was .48 points (95% confidence interval [CI], .09-.88 points; P=.032) in the Symptom Severity Scale score, .37 points (95% CI, .06-.67 points; P=.019) in the Functional Status Scale score, 3.38m/s (95% CI, 0.54-6.22m/s; P=.015) in the SNCV amplitude, and 3.21µV (95% CI, 0.00-6.46µV; P=.025) in the SNAP amplitude. CONCLUSIONS: In people with CTS, steroid injection combined with splinting resulted in modestly greater reduction of symptoms, superior functional recovery, and greater improvement in nerve function at 12-week follow-up as compared with steroid injection alone. However, these small differences are of unclear clinical significance.

Cortical Modulation of Motor Control Biofeedback among the Elderly with High Fall Risk during a Posture Perturbation Task with Augmented Reality.

The cerebral cortex provides sensorimotor integration and coordination during motor control of daily functional activities. Power spectrum density based on electroencephalography (EEG) has been employed as an approach that allows an investigation of the spatial-temporal characteristics of neuromuscular modulation; however, the biofeedback mechanism associated with cortical activation during motor control remains unclear among elderly individuals. Thirty one community-dwelling elderly participants were divided into low fall-risk potential (LF) and high fall-risk potential (HF) groups based upon the results obtained from a receiver operating characteristic analysis of the ellipse area of the center of pressure. Electroencephalography (EEG) was performed while the participants stood on a 6-degree-of-freedom Stewart platform, which generated continuous perturbations and done either with or without the virtual reality scene. The present study showed that when there was visual stimulation and poor somatosensory coordination, a higher level of cortical response was activated in order to keep postural balance. The elderly participants in the LF group demonstrated a significant and strong correlation between postural-related cortical regions; however, the elderly individuals in the HF group did not show such a relationship. Moreover, we were able to clarify the roles of various brainwave bands functioning in motor control. Specifically, the gamma and beta bands in the parietal-occipital region facilitate the high-level cortical modulation and sensorimotor integration, whereas the theta band in the frontal-central region is responsible for mediating error detection during perceptual motor tasks. Finally, the alpha band is associated with processing visual challenges in the occipital lobe.With a variety of motor control demands, increment in brainwave band coordination is required to maintain postural stability. These investigations shed light on the cortical modulation of motor control among elderly participants with varying fall-risk potentials. The results suggest that, although elderly adults may be without neurological deficits, inefficient central modulation during challenging postural conditions could be an internal factor that contributes to the risk of fall. Furthermore, training that helps to improve coordinated sensorimotor integration may be a useful approach to reduce the risk of fall among elderly populations or when patients suffer from neurological deficits.

Profuse unilateral hyperhidrosis induced by urinary retention in a stroke patient.

INTRODUCTION: The diagnosis of urinary retention in stroke patients is challenging because their symptoms may be subtle, or go unnoticed. Furthermore, patients suffering from aphasia or impaired cognition often have difficulties communicating urination problems, leading to their urinary retention being overlooked. CASE REPORT: We report a 53-year-old man with bilateral pontine hemorrhage resulting in incomplete locked-in syndrome. During the patient's hospital course, he developed 3 episodes of rapid onset of profuse unilateral hyperhidrosis. All 3 episodes were found to be induced by an overly distended bladder and each time the unilateral hyperhidrosis resolved promptly after bladder drainage. CONCLUSIONS: This case provides the first description of unilateral hyperhidrosis as a manifestation of urinary retention in a stroke patient. It is important for clinicians to recognize this condition so that appropriate management can be implemented in time to prevent potential detrusor injury and renal impairment caused by bladder overdistension.

Localization of the cricothyroid muscle under ultrasound guidance for vagal nerve mapping.

During surgical removal of tumors of the skull base or cerebellopontine angle with brainstem compression, the vagus nerve is at a high risk for injury that can result in permanent or transient swallowing and speech dysfunction. Intramuscular recording of cricothyroid muscle can be used for vagal nerve mapping during intraoperative neurophysiologic monitoring so as to prevent the above complications. However, it is a small muscle that lies beneath the strap muscles over the anterior neck and is not easily accessible by a blind approach. Here, we present a case in which cricothyroid muscle was identified for precise electrode placement under ultrasound guidance during preparation for intraoperative monitoring. We concluded that localization of the cricothyroid muscle by ultrasonography proved to be a feasible and easy technique, and the compound muscle action potential recorded by this approach is clearly recognizable during intraoperative vagal nerve mapping.

Fluorescence-assisted visualization of facial nerve during mastoidectomy: A novel technique for preventing iatrogenic facial paralysis.

OBJECTIVE: Mastoidectomy can be risky due to the chance of iatrogenic facial nerve dysfunction. Avoiding injuries to the mastoid segment of the facial nerve is mandatory when drilling the bone. With advancements in intraoperative near-infrared indocyanine green (ICG) video angiography, we describe the application of a novel fluorescent guidance technique during mastoidectomies to identify the facial canal with safety. METHODS: Mastoidectomies were performed as the key step in the presigmoid, petrosal or translabyrinthine approaches in 16 patients with different pathologies located at the cerebellopontine angle or petroclival region. After the facial canal was drilled to paper thin, ICG was injected via the central venous catheter. Compared with the dark bony portion, the vessels inside the vasa nervorum were highlighted as a result. The fluorescence guides the operator through the course of the facial nerve and facilitates opening of the internal auditory canal and the dissection of tumors. RESULTS: All 16 facial nerves were recognized during mastoidectomies under fluorescence guidance for varied periods of enhancing time (range, 23-50s). In all, one to four attempts after repeated drilling works to enhance the facial nerve were required before these nerves could be clearly seen. The tumor resection procedure yielded the following results: grossly total removal in seven patients, near total removal in five, and subtotal removal in three. Complete obliteration of a giant vertebral artery aneurysm in one patient was seen in the follow-up angiogram. The post-mastoidectomy facial nerve function, examined by triggered EMG, was preserved in all 16 patients, and no patients had postoperative facial palsy worse than House-Brackmann grade IV after 6 months of follow-up. CONCLUSION: With this novel technique, the course of the facial nerve can be confirmed during mastoidectomy, which reduces the possibility of iatrogenic facial nerve dysfunction. This fluorescence technique is especially helpful in establishing confidence and shortening the learning curve for beginners at mastoidectomies.

Pediatric aquatic therapy on motor function and enjoyment in children diagnosed with cerebral palsy of various motor severities.

This study investigates the effects of pediatric aquatic therapy on motor function, enjoyment, activities of daily living, and health-related quality of life for children with spastic cerebral palsy of various motor severities. Children with spastic cerebral palsy were assigned to a pediatric aquatic therapy group (n = 11; mean age = 85.0 ± 33.1 months; male : female = 4 : 7) or a control group (n = 13; mean age = 87.6 ± 34.0 months; male : female = 9 : 4). The statistic results indicate that the pediatric aquatic therapy group had greater average 66-item Gross Motor Function Measure following intervention than the control group (η(2) = 0.308, P = .007), even for children with Gross Motor Function Classification System level IV (5.0 vs 1.3). The pediatric aquatic therapy group had higher Physical Activity Enjoyment Scale scores than the control group at post-treatment (P = .015). These findings demonstrate that pediatric aquatic therapy can be an effective and alternative therapy for children with cerebral palsy even with poor Gross Motor Function Classification System level.

Cognitive and epilepsy outcomes after epilepsy surgery caused by focal cortical dysplasia in children: early intervention maybe better.

BACKGROUND: Focal cortical dysplasia (FCD) is a specific malformation of cortical development harboring intrinsic epileptogenicity, and most of the patients develop drug-resistant epilepsy in early childhood. The detrimental effects of early and frequent seizures on cognitive function in children are significant clinical issues. In this study, we evaluate the effects of early surgical intervention of FCD on epilepsy outcome and cognitive development. METHODS: From 2006 to 2013, 30 children younger than 18 years old underwent resective surgery for FCDs at Taipei Veterans General Hospital. The mean age at surgery was 10.0 years (range 1.7 to 17.6 years). There were 21 boys and 9 girls. In this retrospective clinical study, seizure outcome, cognitive function, and quality of life were evaluated. To evaluate the effects to outcomes on early interventions, the patients were categorized into four groups according to age of seizure onset, duration of seizure before surgery, and severity of cognitive deficits. RESULTS: Eleven of 22 (50 %) patients demonstrated developmental delay preoperatively. The Engel seizure outcome achievements were class I in 21 (70 %), class II in 2 (7 %), class III in 6 (20 %), and class IV in 1 (3 %) patients. The locations of FCDs resected were in the frontal lobe in 18 cases, temporal lobe in 7, parietal lobe in 2, and in bilobes including frontoparietal lobe in 2 and parieto-occipital lobes in 1. Eight cases that had FCDs involved in the rolandic cortex presented hemiparesis before surgical resection. Motor function in four of them improved after operation. The histopathological types of FCDs were type Ia in 1, type Ib in 7, type IIa in 7, type IIb in 12, and type III in 3 patients. FCDs were completely resected in 20 patients. Eighteen (90 %) of them were seizure free (p < 0.001) with three patients that received more than one surgery to accomplish complete resection. The patients who had early seizure onset, no significant cognitive function deficit, and early surgical intervention with complete resection in less than 2 years of seizure duration showed best outcomes on seizure control, cognitive function, and quality of life. CONCLUSION: Delay in cognitive development and poor quality of life is common in children treated for FCDs. Early surgical intervention and complete resection of the lesion help for a better seizure control, cognitive function development, and quality of life. FCDs involved eloquent cortex may not prohibit complete resection for better outcomes.

Intraoperative brain mapping to identify corticospinal projections during resective epilepsy surgery in children with congenital hemiparesis.

PURPOSE: The purpose of the study is to determine corticospinal organization using intraoperative neurophysiologic monitoring (IONM) during resective epilepsy surgery for patients with congenital hemiparesis and intractable epilepsy. METHODS: Ten patients, aged 3-17, with intractable epilepsy underwent resective surgery. Transcranial stimulation (TCS) was achieved using a pair of cork screws at Cz and C3/C4, respectively. A 1 × 4 stimulating electrode strip was placed on the presumed motor cortex of the affected hemisphere for direct cortical stimulation (DCS) after craniotomy. Multipulse TCS and DCS train stimulation was delivered, with simultaneous recordings from bilateral abductor pollicis brevis and abductor halluces, to determine the corticospinal projection pattern of the paretic limbs. RESULTS: The above mapping techniques revealed ipsilateral corticospinal projections from the contralesional hemisphere to target muscles in the paretic limbs in three patients, projections from both hemispheres to target muscles in three, and preserved crossed projections from the affected hemisphere in four. Nine patients were seizure free after surgery. Five had unchanged postoperative functional status, and three showed minimally improved use of the paretic hand. Two developed new motor deficits after surgery, which may have been due to a premotor syndrome in one patient, since it completely resolved within 2 weeks. The other experienced increased weakness of the paretic lower limb because a small part of the eloquent cortex was removed for better seizure control. CONCLUSIONS: Using IONM to define the corticospinal projection pattern is a valuable technique that can potentially replace preoperative fMRI and transcranial magnetic stimulation in resective epilepsy surgery, particularly for younger patients.