Shigeki Kameyama

Patients with temporal lobe epilepsy show an increase in brain-derived neurotrophic factor protein and its correlation with neuropeptide Y

Model studies on animal seizures have proposed potential involvement of the neurotrophins, BDNF and NGF, in human epilepsy. However, their biological significance in this disease itself remains to be evaluated. Here we demonstrate that patients with intractable temporal lobe epilepsy show a marked increase in protein levels of BDNF (2.6-fold, p<0.01) but not other neurotrophins. Moreover, the specific BDNF increase was significantly correlated with contents of neuropeptide Y. Thus, these results indicate the activity-dependent expression of BDNF in human subjects and its potential contribution to the pathophysiology of human epilepsy via neuropeptide Y.

Somatic Mutations in the MTOR gene cause focal cortical dysplasia type IIb

Focal cortical dysplasia (FCD) type IIb is a cortical malformation characterized by cortical architectural abnormalities, dysmorphic neurons, and balloon cells. It has been suggested that FCDs are caused by somatic mutations in cells in the developing brain. Here, we explore the possible involvement of somatic mutations in FCD type IIb.

Bilateral periventricular nodular heterotopia due to filamin 1 gene mutation: widespread glomeruloid microvascular anomaly and dysplastic cytoarchitecture in the cerebral cortex

Abstract. Bilateral periventricular nodular heterotopia (BPNH) is a neuronal migration disorder that is characterized by subependymal nodules of gray matter. Recently, a causative gene for BPNH, filamin 1, has been identified, and possible roles of the translated protein in cell migration and blood vessel development have been proposed. We report here the histopathological features of an autopsy case of BPNH with widespread glomeruloid microvascular anomaly and dysplastic cytoarchitecture in the cerebral cortex, in whom we found a novel exon 11 (Val528Met) filamin 1 mutation. Within the periventricular nodules, well-differentiated pyramidal neurons were randomly oriented. A small proportion of neurons were immunolabeled with antibodies raised against calbindin D-28k, parvalbumin, or calretinin. We used a carbocyanine dye (DiI) tracing technique to investigate the extent of fiber projections within and outside the nodules. The labeled fibers formed bundles that extended into the surrounding white matter. Connections between adjacent nodules were evident. Connections between the nodules and the cerebral cortex were also seen, with a small number of labeled fibers reaching the cortex. In the cerebral cortex, small closely packed vessels ran in a parallel fashion throughout all of the layers. Immunohistochemically, the inner rim of individual vessel lumina was labeled by an antibody against factor VIII, and the vessel walls were labeled by antibodies against actin and laminin. Astrocyte processes, labeled with an antibody to glial fibrillary acidic protein, invaded these vascular channels. Ultrastructurally, a network of basal lamina-like materials lined with endothelial cells was evident. The cytoarchitecture of the cerebral cortex was disturbed, in that the columnar neuronal arrangement was distorted around the malformed vessels. This case appears to represent an example of BPNH manifesting widespread developmental anomalies within the blood vessels and the cortical cytoarchitecture in the cerebrum.

Stereotaxy for hypothalamic hamartoma with intractable gelastic seizures: technical case report

OBJECTIVE AND CLINICAL IMPORTANCE: We report a patient with a hypothalamic hamartoma treated by stereotactic radiofrequency thermocoagulation. CLINICAL PRESENTATION: A 15-year-old girl presented with a hypothalamic hamartoma associated with intractable gelastic and tonic seizures. There were no clinical signs of precocious puberty. Magnetic resonance imaging revealed an isointense suprasellar mass, about 1 cm in diameter. TECHNIQUE: A depth electrode was placed into the hamartoma using a computed tomography-compatible stereotactic frame. Depth electroencephalographic studies allowed us to record the seizure onset from the lesion, and stereotactic radiofrequency thermocoagulation produced seizure remission. CONCLUSION: These findings suggest that hypothalamic hamartoma itself has intrinsic epileptogenicity. We believe that this surgical treatment is effective for the relatively small hypothalamic hamartoma associated with intractable gelastic seizures.

Surgical strategy and outcomes for epileptic patients with focal cortical dysplasia or dysembryoplastic neuroepithelial tumor.

Summary: u2002Purpose: The purpose of this study was to clarify and compare the influence of surgical strategy on relief from seizures in patients with focal cortical dysplasia (FCD) and those with dysembryoplastic neuroepithelial tumor (DNT).

The long-term growth rate of residual acoustic neurinomas

SummaryThe growth rate of 19 residual acoustic neurinomas was examined in a long-term follow-up study (median, 10 years; range, 5 to 17 years) following intracapsular removal. Of these, 10 (53%) had regrowth, three (16%) showed regression, and six (32%) were unchanged. The 10 acoustic neurinomas showing regrowth were divided into two categories, either solid or cystic, according to computed tomographic findings. Five acoustic neurinomas with cyst formation showed rapid regrowth, with the tumour doubling time ranging from 0.15 to 5.0 years (median, 4.5 years), and required re-operation. Five solid tumours showed slow regrowth, with the tumour doubling time ranging from 9 to 34 years (median, 15 years). Although cyst formation is a major factor in rapid regrowth, residual acoustic neurinomas without cyst formation have a slower growth potential. In this study, 74% of the residual acoustic neurinomas have never required re-operation. It is advisable to choose intracapsular removal if there is major risk of neurological deficits.

Contribution of subtraction ictal SPECT coregistered to MRI to epilepsy surgery: a multicenter study

ObjectiveA multicenter prospective study was performed to assess the additional value of a subtraction ictal SPECT coregistered to MRI (SISCOM) technique to traditional side-by-side comparison of ictal- and interictal SPECT images in epilepsy surgery.MethodsOne hundred and twenty-three patients with temporal and extratemporal lobe epilepsy who had undergone epilepsy surgery after evaluation of scalp ictal and interictal electroencephalogram (EEG), MRI, and ictal and interictal SPECT scans were followed up in terms of postsurgical outcome for a period of at least 1xa0year. Three reviewers localized the epileptogenic focus using ictal and interictal SPECT images first by side-by-side comparison and subsequently by SISCOM. Concordance of the localization of the epileptogenic focus by SPECT diagnosis with the surgical site and inter-observer agreement between reviewers was compared between side-by-side comparison and SISCOM. Logistic regression analysis was performed in predicting the surgical outcome with the dependent variable being the achievement of a good postsurgical outcome and the independent variables using the SISCOM, side-by-side comparison of ictal and interictal SPECT images, MRI, and scalp ictal EEG.ResultsThe SISCOM presented better concordance in extratemporal lobe epilepsy and less concordance in temporal lobe epilepsy than side-by-side comparison. Inter-observer concordance was higher in SISCOM than in side-by-side comparison. Much higher concordance of the epileptogenic focus by SPECT diagnosis with the surgical site was obtained in patients with good surgical outcome than in those with poor surgical outcome. These differences in concordance between good and poor surgical outcomes were greater in SISCOM than in side-by-side comparison. Logistic regression analysis showed the highest odds ratio of 12.391 (95% confidence interval; 3.319, 46.254) by SISCOM evaluation for concordance of the epileptogenic focus with the surgical site in predicting good surgical outcome.ConclusionsA SISCOM technique of ictal and interictal SPECT images provides higher predictive value of good surgical outcome and more reliability on the diagnosis of the epileptogenic focus than side-by-side comparison in medically intractable partial epilepsy.

Ganglioglioma with a tanycytic ependymoma as the glial component

We studied a cystic ganglioglioma (GG) located in the right frontal lobe of the brain. Interestingly, the fibrillary spindle glial cells were often arranged in a fascicular pattern, and the generally uniform, round-to-oval delicate nuclei appeared to resemble those of ependymoma; and the neoplastic neurons often contained neurofibrillary tangles (NFTs). The glial component was positive for glial fibrillary acidic protein and occasionally contained granular or microvesicular structures positive for epithelial membrane antigen. Ultrastructural investigation revealed that the glial cells were ependymal in nature; intracytoplasmic lumina and intercellular microrosettes lined with cilia and microvilli, as well as long zonulae adherentes, were evident. In addition, chromogranin A-positive granular staining, neurosecretory-granule-like structures, and parallel arrays of microtubules were sometimes associated with the blood vessels. We considered the present case to be an unusual example of GG with an ependymoma, more precisely a tanycytic ependymoma, as the glial component; to our knowledge, the existence of ependymoma as the main glial component of this particular tumor has not been described before. The occurrence of NFTs, which has been reported in several cases of GG, was an additional, unusual feature.

Abnormal muscle response monitoring during microvascular decompression for hemifacial spasm.

SummaryBackground. Several studies have investigated the relation between intraoperative abnormal muscle response (AMR) findings and postoperative results in patients undergoing microvascular decompression (MVD) for hemifacial spasm (HFS). However, there is some debate over the reliability of AMR as an indicator of postoperative outcome. We investigated whether AMR findings obtained during MVD reflect postoperative outcome in patients with HFS.Method. Subjects were 60 HFS patients who underwent AMR monitoring during MVD. AMR recordings were obtained from the mentalis muscle by electrical stimulation of the temporal branch of the facial nerve and from the orbicularis oculi muscles by stimulation of the marginal mandibular branch. Surgical outcome was compared with AMR findings at the completion of MVD. Mean follow-up was 61 months.Findings. HFS resolved completely in 50 patients in whom AMR disappeared intraoperatively and in 5 patients in whom the AMR amplitude was decreased at the end of MVD. Four patients showed HFS at the final follow-up examination despite cessation or decrease of AMR during surgery. In 1 patient, preoperative AMR waveforms persisted throughout MVD, but the postoperative outcome was excellent.Conclusions. Our findings suggest that intraoperative cessation or decreased amplitude of AMR at the end of surgery indicates a high likelihood of postoperative relief of HFS. We believe that intraoperative AMR monitoring is useful in MVD surgery for HFS.

Long-term follow-up of the residual intracanalicular tumours after subtotal removal of acoustic neurinomas

SummaryWe examined growth potential of residual intracanalicular tumours left from subtotal removal of large acoustic neurinomas. Eleven patients were followed-up by magnetic resonance (MR) imaging. The interval between surgery and MR study ranged from 12 to 29 years (median, 16 years). MR images of two patients showed no evidence of tumour remnant, and in six a small tumour was localized in the internal auditory canal. The other three showed an intracanalicular tumour protruding slightly towards the intracranial portion. This result suggests that the intracanalicular residual tumours have less risk of regrowth after subtotal removal of acoustic neurinomas. It is advisable to choose intracapsular subtotal removal without opening the internal auditory canal in the treatment of acoustic neurinoma, if it is large in size and there is a high risk of nerve injury.