Bony and Cartilaginous Tissues in Lumbosacral Lipomas.

PURPOSE: It is well known that bony and cartilaginous tissues can be present in lumbosacral lipomas; however, the relationship between their presence and clinical features has not been demonstrated. METHODS: Five (10.4%) out of 48 patients had osteochondral tissues in lipomas. We retrospectively analyzed the clinical, neuroradiological, and histological findings of these patients. RESULTS: Five (45.5%) of 11 patients with dorsal and transitional type lipomas had osteochondral tissues, while none with caudal and filar type lipomas had these tissues. Presurgical imaging demonstrated that the osteochondral tissue was located in a large subcutaneous lipoma dorsal to the bifid vertebral column. Histologically, mature bone with hematopoietic marrow and hyaline cartilage were observed in 3 and 2 patients, respectively. CONCLUSIONS: The high incidence of association of osteochondral tissues with dorsal and transitional type lipomas is thought to be the result of primary neurulation failure with invasion of mesenchymal tissues. Caudal and filar type lipomas, resulting from secondary neurulation failure, thus did not have osteochondral tissue.

Retained medullary cord extending to a sacral subcutaneous meningocele.

BACKGROUND: A retained medullary cord (RMC) is a rare closed spinal dysraphism with a robust elongated neural structure continuous from the conus and extending to the dural cul-de-sac. One case extending down to the base of a subcutaneous meningocele at the sacral level has been reported. CLINICAL PRESENTATION: We report on three cases of closed spinal dysraphism, in which a spinal cord-like tethering structure extended out from the dural cul-de-sac and terminated at a skin-covered meningocele sac in the sacrococcygeal region, which was well delineated in curvilinear coronal reconstructed images of 3D-heavily T2-weighted images (3D-hT2WI). Intraoperative neurophysiology revealed the spinal cord-like tethering structure was nonfunctional, and histopathology showed that it consisted of central nervous system tissue, consistent with RMC. The tethering structure histologically contained a glioneuronal core with an ependymal-like lumen and smooth muscle, which may indicate developmental failure during secondary neurulation. CONCLUSIONS: When the RMC extending to a meningocele is demonstrated with the detailed magnet resonance imaging including 3D-hT2WI, decision to cut the cord-like structure for untethering of the nervous tissue should be made under careful intraoperative neurophysiological monitoring.

Tadpole-shaped lateralized parietal atretic cephalocele associated with an ipsilateral lacrimal gland fistula and schizencephalic clefts.

BACKGROUND: Parietal atretic cephalocele (AC) and its associated intracranial venous anomalies, such as vertical embryonic positioning of the straight sinus (VEP of SS), have, in previous reports, been exclusively restricted to the midline. CLINICAL PRESENTATION: We report a patient with lateralized parietal AC on the right side. The AC was in the shape of a tadpole, with a large head and a long tail, extending to the proximity of the right external canthus, where a lacrimal gland fistula was observed. The superior sagittal sinus and VEP of SS were also displaced to the right side, although the sagittal suture was located at the midline. Schizencephalic clefts in the right posterior cortex were also observed. CONCLUSION: The parietal AC, which was initially located in the midline, could conceivably have been displaced to the right side by other developmental processes. However, the relationship between lateralized AC and associated multiple anomalies on the ipsilateral side is difficult to explain monogenetically. Our case study indicates that AC might have a broader spectrum of clinical symptoms than was once thought to be the case.

Sequential morphological change of Chiari malformation type II following surgical repair of myelomeningocele.

PURPOSE: To document long-term morphological changes of Chiari type II malformation (CM-II) following closure of spina bifida manifesta (SBM). METHODS: We retrospectively evaluated postnatal magnetic resonance images of the CM-II and posterior fossa (PF) in 28 consecutive cases. We measured changes in vertebral level and length of the cerebellar peg (CP), cerebrospinal fluid (CSF) spaces anterior and posterior to the cerebrospinal junction, PF area, and the anteroposterior diameters of the foramen magnum (FM) and C1 vertebra. We examined the morphological differences between the cases with and without ventriculoperitoneal (VP) shunting and derived predicted means by nonlinear mixed-effect modeling. RESULTS: At birth, there were significant differences in CP length, PF area, and FM and C1 diameters between those who underwent VP shunting and those who did not. In cases with a CP below C1, VP shunting was required in every case but one. In those with visible CSF space at birth, VP shunts were not required. In 17 of 18 cases with a CP below C1, the vertebral level ascended by mean two vertebral levels (range 0-5 levels) within 4-6 months of delivery. In the remaining case, slowly progressive hydrocephalus and delayed CP descent required VP shunting at 8 months. Predicted mean CP length and FM and C1 diameters were greater in those who underwent VP shunting, but there was no difference in predicted mean PF area. CONCLUSION: The morphology of CM-II and the presence of hydrocephalus influence each other in children who have undergone postnatal SBM repair.

Neuromagnetic evidence for hippocampal modulation of auditory processing.

The hippocampus is well known to be involved in memory, as well as in perceptual processing. To date, the electrophysiological process by which unilateral hippocampal lesions, such as hippocampal sclerosis (HS), modulate the auditory processing remains unknown. Auditory-evoked magnetic fields (AEFs) are valuable for evaluating auditory functions, because M100, a major component of AEFs, originates from auditory areas. Therefore, AEFs of mesial temporal lobe epilepsy (mTLE, n=17) with unilateral HS were compared with those of healthy (HC, n=17) and disease controls (n=9), thereby determining whether AEFs were indicative of hippocampal influences on the auditory processing. Monaural tone-burst stimuli were presented for each side, followed by analysis of M100 and a previously less characterized exogenous component (M400: 300-500ms). The frequency of acceptable M100 dipoles was significantly decreased in the HS side. Beam-forming-based source localization analysis also showed decreased activity of the auditory area, which corresponded to the inadequately estimated dipoles. M400 was found to be related to the medial temporal structure on the HS side. Volumetric analysis was also performed, focusing on the auditory-related areas (planum temporale, Heschl's gyrus, and superior temporal gyrus), as well as the hippocampus. M100 amplitudes positively correlated with hippocampal and planum temporale volumes in the HC group, whereas they negatively correlated with Heschl's gyrus volume in the mTLE group. Interestingly, significantly enhanced M400 component was observed in the HS side of the mTLE patients. In addition, the M400 component positively correlated with Heschl's gyrus volume and tended to positively correlate with disease duration. M400 was markedly diminished after hippocampal resection. Although volumetric analysis showed decreased hippocampal volume in the HS side, the planum temporale and Heschl's gyrus, the two major sources of M100, were preserved. These results suggested that HS significantly influenced AEFs. Therefore, we concluded that the hippocampus modulates auditory processing differently under normal conditions and in HS.

Clinical Significance of Prenatal and Postnatal Heavily T2-Weighted Magnetic Resonance Images in Patients with Myelomeningocele.

PURPOSE: To compare the utility and limitations of prenatal magnetic resonance (MR) imaging using half-Fourier acquisition single-shot turbo spin-echo (HASTE) with postnatal heavily T2-weighted imaging (hT2WI) for the evaluation of central nervous system abnormalities associated with myelomeningocele (MMC). METHODS: Sixteen patients with MMC who had undergone pre- and postnatal MR imaging were included in this study. MR imaging, including HASTE, was undertaken in the 3rd trimester, and hT2WI was performed immediately after delivery. The precision with which each could distinguish MMC, hindbrain herniation and ventriculomegaly was compared retrospectively. RESULTS: The skin defects and MMC sacs were clearly visible on prenatal HASTE images, although it was difficult to identify precisely the level of MMC compared with postnatal hT2WI, in which the detailed anatomical relationships of the spinal cord, neural placode and ventral nerve roots were evident in every case. Hindbrain herniation could be visualized on prenatal HASTE images, although its severity was difficult to evaluate because of the small size of the structures and neck flexion; again, the resolution was superior on postnatal hT2WI. For hydrocephalus, there were no significant differences in the diagnostic precision and ability to grade the severity between pre- and postnatal imaging. CONCLUSION: Prenatal HASTE imaging permits the diagnosis and understanding of the gross anatomy of MMC and associated hindbrain herniation and ventriculomegaly, but postnatal hT2WI is superior for evaluating detailed anatomy.

[Clinical and histological characteristics of ictal onset zone in cases of intractable epilepsy associated with dysembryoplastic neuroepithelial tumor].

Although the epileptogenic location of dysembryoplastic neuroepithelial tumors (DNTs) is controversial, it has recently been thought to be located within cortical dysplasia (CD) due to its frequent association with CD. Among the 84 resection surgeries for intractable epilepsy performed in our institution between January 2003 and April 2010, three patients had epileptogenic DNTs. In two cases, chronic subdural electrocorticography (ECoG) was performed, and the ictal onset zone was revealed to be in the cortex around the DNT. The ictal onset zone was resected along with the DNT, and good seizure outcome was achieved. Although histological examination of the ictal onset zone revealed mild gliosis, coexistence of CD was not noted. In the third case, the DNT was located in the left lateral temporal lobe and the intraoperative ECoG revealed frequent paroxysmal activity in the medial temporal lobe. Resection of the lateral temporal lobe involving the tumor did not result in good seizure control. The optimal surgical treatment of DNT is controversial. Some authors consider lesionectomy to be sufficient for good seizure control, whereas others advocate that additional resection of the epileptogenic zone beside the tumor improves outcome. Because the epileptogenic location of DNT varies among cases, it is important to identify its location by preoperative multimodal examinations, including chronic subdural ECoG recordings.

Neurological outcomes in Chiari type II malformations and their correlation to morphological findings and fetal heart rate patterns: a retrospective study.

BACKGROUND: Correlations among Chiari type II malformation (CMII) morphological findings, the proportion of fetal heart rate patterns corresponding to the quiet phase (QP), and neurological outcomes have yet to be investigated. FINDINGS: The correlations among the morphological findings (i.e., the degree of ventriculomegaly, myelomeningocele levels, and degree of cerebellar herniation), proportion of time spent in QP, and developmental quotients (DQs) were analyzed in 22 children. The proportion of time spent in QP was compared between children with poor neurological outcomes (n = 9) and those with good outcomes (n = 13). Pearson's correlations and the Mann-Whitney U-test were used to assess for statistical significance; P < 0.05 was considered statistically significant. No significant differences were observed between the DQs and morphological findings, but the DQs and the proportion of time spent in QP were significantly correlated (r = 0.287, P = 0.01). The proportion of time spent in QP was significantly different between children with poor outcomes and those with good outcomes (median, 11% [range, 0-32%] vs. 28% [range, 2-55%]; P = 0.006). CONCLUSIONS: The proportion of fetal heart rate patterns corresponding to the QP might be a useful predictor of neurological outcomes in 2-year-old children with CMII.

ILAE focal cortical dysplasia type IIIc in the ictal onset zone in epileptic patients with solitary meningioangiomatosis.

"Solitary" meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion of the cerebral cortex and frequently leads to epilepsy. However, the source of the epileptogenicity in meningioangiomatosis remains controversial. We report two surgically-treated meningioangiomatosis cases with medically intractable epilepsy. In both cases, chronic subdural electrocorticogram (ECoG) recordings identified the ictal onset zone on apparently normal cortex, adjacent to and/or above the meningioangiomatosis lesion, not on the meningioangiomatosis lesion itself. The ictal onset zone was resected, along with the MA lesion, and good seizure outcome was achieved. Histological examination of the ictal onset zone revealed the presence of ILAE focal cortical dysplasia (FCD) type IIIc. Our case studies suggest that in the surgical management of epilepsy with meningioangiomatosis, it is important to identify undetected, but epileptogenic, ILAE FCD Type IIIc, using preoperative multimodal examinations, including chronic ECoG recordings.

[Patients with intractable epilepsy who achieved good seizure control after craniotomy instead of vagal nerve stimulation].

Vagal nerve stimulation(VNS)is an effective adjunctive therapy for medically intractable epilepsy. However, VNS is a palliative therapy, and craniotomy should preferably be performed when complete seizure remission can be expected after craniotomy. We report here three patients who were referred for VNS therapy, but underwent craniotomy instead of VNS based on the results of a comprehensive preoperative evaluation, and achieved good seizure control. Case 1 was a 48-year-old woman with left temporal lobe epilepsy and amygdalar enlargement. Even though no left hippocampal sclerosis was observed on magnetic resonance imaging, she underwent left anterior temporal lobectomy and hippocampectomy. Case 2 was a 36-year-old woman with multiple bilateral subependymal nodular heterotopias, who underwent resection of the left medial temporal lobe including subependymal nodular heterotopias adjacent to the left inferior horn. Case 3 was a 25-year-old man with posttraumatic epilepsy. As the right hemisphere was most affected, multiple subpial transections were performed on the left frontal convexity. These three patients were referred to us for VNS therapy because there was a dissociation between the interictal electroencephalogram and magnetic resonance imaging findings, or because they had multiple or extensive epileptogenic lesions. Comprehensive preoperative evaluation including ictal electroencephalography can help to identify patients who are suitable candidates for craniotomy.

[Surgical complications of vagal nerve stimulation for intractable epilepsy: findings from 26 cases].

INTRODUCTION: Vagal nerve stimulation (VNS) is a less invasive palliative treatment for intractable epilepsy and was approved for use in Japan in July 2010. Surgical complications of VNS such as vagal nerve dysfunction, cardiac arrhythmia with asystole, and vocal cord palsy as well as complications arising from fracture of the leads or generator and infections are well known in the West. The aim of the present report is to describe the surgical complications encountered in our hospital and discuss their countermeasures. MATERIAL AND METHODS: We reviewed the clinical records of 26 patients who underwent VNS therapy between March 2011 and June 2013. The cases involved 17 male and 9 female patients, including 8 children(<15 years of age). RESULTS: Three patients(11.5%)experienced severe bradycardia and cardiac asystole following test stimulations of the vagal nerve with a stainless-steel surgical hook left in place, to extend the operative field. It was believed that the current spread through the hook and stimulated the cardiac branch of the vagal nerve. In an adult patient with severe intellectual disability, inappropriate dermatological therapy for a superficial purulent wound on the neck caused lead infection 10 months postoperatively. In a child with moderate intellectual disability, lead fracture was noted in association with rotation of the pulse generator at one month postoperatively. In the former case, the lead was cut off whilst the electrode and anchoring coil on the vagal nerve remained;the whole VNS system was removed in the latter case. Subfascial implantation of the generator was recommended. In an adult patient, disconnection between the leads and generator head was noted at 10 months postoperatively. CONCLUSIONS: During intraoperative test stimulations of the vagal nerve, stainless-steel surgical hooks should be removed to avoid the spread of current. In intellectually disabled patients, the pulse generator should be placed in the subfascial area instead of the subcutaneous area, especially children. The connection between the leads and the generator should be performed with the aid of a microscope, after removal of the fluid and tissue.

Minimum norm estimates in MEG can delineate the onset of interictal epileptic discharges: A comparison with ECoG findings.

The analysis of epileptic discharges in magnetoencephalography with minimum norm estimates (MNE) is expected to provide more precise localization of epileptic discharges compared with electroencephalographic estimations. However, the clinical feasibility of MNE remains unclear. In this study, we aimed to elucidate the onset and propagation patterns of interictal spikes using MNE. Seven patients with intractable epilepsy whose epileptogenicity was assumed to exist in the convexity of the cerebral cortex were studied. For MNE and electrocorticography (ECoG), we characterized the propagation patterns of interictal epileptic discharges according to the area in which they originated and where they extended; we then examined whether the propagation patterns observed in MNE were identified by ECoG. We also examined the relationship between the positions of spikes estimated by the equivalent current dipole (ECD) method and MNE. Among the seven patients, nine propagation patterns of epileptic discharges were observed by MNE, all of which were also identified by ECoG. In seven patterns, the epileptic activity propagated around the initial portion. However, in two patterns, the center of activities moved according to propagation with maintained activity of the initial portion. The locations of spikes identified by the ECD method were within the areas estimated by MNE when the epileptic activity propagated. However, the ECD method failed to detect onset activities identified by MNE in three of nine patterns. Thus, MNE is more useful as a means of presurgical evaluation for epilepsy than the ECD method because it can delineate the onset of epileptic activities as shown in ECoG.

Usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo and three-dimensional heavily T2-weighted images in preoperative evaluation of spinal dysraphism.

PURPOSE: The aim of this study was to evaluate the usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo (3D T1-GRE) images for the preoperative anatomical evaluation of lumbosacral lipoma, thick filum terminale, and myelomeningocele as a means of compensating for the drawbacks of 3D heavily T2-weighted (3D hT2-W) images. METHODS: Nine patients with lumbosacral lipomas, one patient with tight filum terminale, and five patients with myelomeningoceles were included in this study. 3D T1-GRE images were compared with 3D hT2-W images or conventional magnetic resonance images in terms of delineation of lipomas and other structures in the patients with lipomas and tight filum terminale. For patients with myelomeningoceles, 3D T1-GRE images were compared with 3D hT2-W images in terms of artifacts in the cerebrospinal fluid (CSF) space. RESULTS: The 3D T1-GRE images demonstrated lipomas with good contrast to the spinal cord and CSF space and more clearly delineated the anatomical relationship between lipomas and these structures than did the 3D hT2-W images. The 3D T1-GRE images delineated dural defects through which extradural lipomas penetrated into the intradural space. The 3D T1-GRE images also demonstrated the presence or absence of lipomas in the filum terminale and the absence of artifact in the myelomeningoceles. Furthermore, they were useful for differentiating artifacts observed on the 3D hT2-W images from nerve elements. CONCLUSIONS: The complementary use of 3D T1-GRE and 3D hT2-W images may compensate for the drawbacks of 3D hT2-W images and may eventually improve lesion visualization and surgical decision making.

Focal cortical dysplasia type IIa underlying epileptogenesis in patients with epilepsy associated with Sturge-Weber syndrome.

In patients with epilepsy associated with Sturge-Weber syndrome (SWS), epileptogenesis has been suggested to be caused by chronic ischemia in cortical areas affected by leptomeningeal angiomatosis or by ischemia-related cortical malformations. However, this has not been fully verified electrophysiologically. We herein present two cases of SWS with medically intractable epilepsy in which the epileptogenic area involved focal cortical dysplasia (FCD) type IIa near the region of leptomeningeal angiomatosis. In both cases, the ictal-onset zones were identified by chronic subdural electrodes, and the presence of FCD type IIa was shown histopathologically. In SWS, especially in association with focal leptomeningeal angiomatosis, FCD may thus play a major role in epileptogenesis. FCD should therefore be demonstrated by the collective findings of perioperative neurophysiologic examination, anatomic and functional neuroimaging, and histopathologic examination.

[Frequency of the use of vagus nerve stimulation for the treatment of intractable epilepsy during the first year of public health insurance coverage with in Kyushu Rosai Hospital and other areas in Japan].

Vagus nerve stimulation (VNS) is a palliative treatment for medically intractable epilepsy and has been covered by public health insurance in Japan since July 1, 2010. The frequency of the use of VNS during the first year of insurance coverage was determined by assessing the number of cases for which VNS was performed in Kyushu Rosai Hospital, the number of registered cases, and the questionnaire survey filled by 68 surgeons who are board certified as both epileptologists and neurosurgeons. VNS devices were placed in 98 patients from July 2010 to June 2011. These devices were placed in an average of 4.4 patients per month from July 2010 to November 2010 and in an average of 10.9 patients from December 2010 to June 2011. However, we did not observe an increasing trend. Almost all of the surgeries were performed in the Kanto (56 patients in 8 institutes) and Tokai (24 patients in 2 institutes) areas. VNS was not performed in many institutes primarily because VNS was not indicated for any of the patients. The questionnaire survey indicated that the use of VNS was likely to increase with an increase in the number of neurologists who decide on performing VNS preoperatively and regulate the conditions of the vagus nerve stimulator postoperatively. In conclusion, VNS is currently being applied in a limited number of institutes in the Kanto and Tokai areas, and a close association between the epileptologists and neurologists during preoperative and postoperative periods will increase the use of VNS.

Worldwide academic contributions of Japanese neurosurgeons.

Based on the data reported in the National Institute of Science and Technology Policy 2010, Japan is ranked in fourth place in the world in terms of the numbers of the articles in the fields of clinical medicine. However, there had not been any objective data regarding the numbers of publications by neurosurgeons. As it is important for neurosurgeons to realize the extent of academic contributions by the neurosurgeons in different countries, the numbers of publications in the major journals by the members of the Japan Neurosurgical Society and those from neurosurgical institutions around the world were analyzed using both the biomedical literature database PubMed and the publication database "ISI Web of Knowledge." Parts of the results were presented in the 69th Annual Meeting of the Japan Neurosurgical Society. As to the number of neurosurgical publications in English from the top 9 countries, the US has been consistently in first place and Japan in second. However, the number of publications from Japan has been decreasing since 2000. With regards to the "top 8 journals" such as the Lancet and the Journal of the American Medical Association, the number of first-author publications by Japanese neurosurgeons increased in the late 1980s and had been 2-9 articles per year until recently. In the "top 12 neuroscience journals" which include Stroke, Neuro-Oncology, Cancer Research, and others, Japan had been in the third next to the US and UK till 2004, but Germany surpassed Japan in 2005. In the "top 6 clinical journals" such as the Journal of Neurosurgery and Neurosurgery, the US has been consistently keeping first place and Japan second place since 1977. Searches using the key word elucidated that Japanese neurosurgeons are greatly contributing in the field of "aneurysm." Regarding the number of publications per neurosurgeon, Canada and UK are in the forefront and Japan is down to eighth place. Japanese neurosurgeons have been contributing greatly next to the Americans to the field of clinical neurosurgery and neuroscience by publishing in English. However, the number of publications by Japanese neurosurgeons has been declining since 2000. The Japan Neurosurgical Society must come up with countermeasures to address this problem.

Feasibility of intraoperative motor-evoked potential monitoring for skull base tumors with a high risk of postoperative motor deterioration.

OBJECTIVE: To establish the validity and utility of motor-evoked potential (MEPs) monitoring for skull base tumor resection, we explored the relationship between MEP monitoring results and postoperative motor function. METHODS: MEPs were successfully monitored during 76 operations in 68 patients with a high risk of motor morbidity. MEP monitoring data were correlated with perioperative clinical motor function. RESULTS: MEPs remained stable in 56 operations (73.7%), and no postoperative motor deterioration was observed. Transient or permanent deterioration of MEPs (<50% of the initial amplitude before surgery) occurred in 20 operations (26.3%). This deterioration was reversible after intervention in seven cases (9.2%). Irreversible deterioration in MEPs was seen in 13 cases (17.1%). In five cases, the final amplitude was greater than 10%. Two of these patients experienced transient loss of MEPs and moderate to severe hemiparesis. Both patients showed full recovery within 6 months after the operation. The other three patients experienced no postoperative worsening of motor function. The final MEP amplitude was less than 10% in the other eight patients, including five with permanent MEP loss. All of these patients experienced severe postoperative motor dysfunction. Recovery of motor function was worse in most participants in this group compared with those in the other groups. CONCLUSION: Intraoperative MEP monitoring is a valid indicator of pyramidal tract pathway function for skull base tumor surgery.

Focal cortical dysplasia coexisting with diffuse astrocytoma in childhood: a case report and reappraisal of the glial component in archival FCD cases.

We report a rare case of focal cortical dysplasia (FCD) concurring with diffuse astrocytoma and arachnoid cyst, and also re-evaluate the glial component in archival FCD cases for the differential diagnosis of diffuse gliomas. A 7-year-old boy with a 9-month history of psychomotor seizures disclosed a hyperintense area accompanied by a cystic lesion in the left temporal lobe on MRI. The surgical specimen displayed dyslamination of the cortices and ectopic neurons in the white matter, associated with dysmorphic neurons, indicating FCD type IIA. Additionally, the lesion showed diffuse proliferation and infiltration of glial cells, immunopositive for infiltrating glioma markers (nestin, doublecortin, MAP-2e) and p53, and MIB-1 index was 2.0%. These findings indicated coexisting diffuse astrocytoma. Coexistence of diffuse glioma with FCD is unusual, but we often notice increased population of small glial cells in FCD lesions. Re-evaluation of archival FCD cases with diverse markers revealed that reactive microglia significantly proliferate in the white matter lesions. Therefore, a careful pathological assessment has to be made to define a rare case of diffuse glioma occurring in FCD.