Hypermotor seizure arising from insular cortex.
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Cerebral Cortex↗
Biomedical subjects
Taisuke Otsuki
Publications and source records attributed to Taisuke Otsuki.
Encapsulation cell therapy for mucopolysaccharidosis type VII using genetically engineered immortalized human amniotic epithelial cells.
Mucopolysaccharidosis type VII (MPSVII) is a lysosomal storage disease resulted from a deficiency of the enzyme beta-glucuronidase (GUSB), which is necessary for degradation of glycosaminoglycans (GAGs). The deficiency of GUSB causes progressive accumulation of GAGs and subsequent lysosomal distension in multiple tissues, including the central nervous system (CNS). In murine experiments, bone marrow transplant, enzyme replacement, viral vectors, and genetically modified cells were successfully used for correction of the visceral accumulation of GAGs, but little improvement was seen in the brain, because these therapeutic agents cannot cross the blood-brain barrier (BBB). Although direct intracerebral injection of GUSB-encoding viral vectors has been developed to bypass the BBB, the possibility of tumor formation and the toxicity of over-expressed GUSB have been reported. In this study, we generated immortalized human amniotic epithelial (IHAE) cells to maintain the effect of implantation, and encapsulated these cells to prevent harmful immunological response and tumor formation and to regulate the level of GUSB expression within the host. Moreover, we generated IHAE cells that over-express and secrete human GUSB following transduction with an adenoviral vector encoding human GUSB. Therapeutic efficacy for MPSVII was evaluated in and ex vivo experiments using these encapsulated genetically engineered GUSB-encoding IHAE cells. We confirmed that encapsulated genetically engineered IHAE cells could secrete significant amounts of GUSB outside the capsule in vitro and into the cerebral parenchyma of C3H mice seven days after the capsule implantation. Thus, encapsulation cell therapy using genetically engineered IHAE cells is an effective armamentarium for the treatment of MPSVII.
Amnion↗
[Recent advances in diagnosis and treatment of epilepsy in Japan].
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Child↗
Glutamate metabolism in epilepsy: 13C-magnetic resonance spectroscopy observation in the human brain.
To clarify changes in glutamate metabolism in the brain with chronic epileptic activities, 13C-magnetic resonance spectroscopy observation of glutamate and glutamine synthesis after oral administration of [1-13C] glucose (Glc C1) (0.75 g/kg) was performed in intractable occipital lobe epilepsy patients (n=5) and controls (n=10). 1H[13C]-spectra were obtained from two voxels of 64 ml placed on the bilateral parieto-occipital lobes of the study participants. Time courses for 13C-incorporation into 4-glutamate and 3-glutamate (Glu C4, C3) and 4-glutamine (Gln C4) were obtained and the concentrations of Glu C4, C3 and Gln C4 at the time between 120 and 150 min after Glc C1 administration was calculated. Concentration of Gln C4 was increased in the epilepsy patients [control: 0.39 mM (SD 0.14), epilepsy: 0.60 mM (SD 0.15), P<0.05], whereas those of Glu C4 and Glu C3 were not. The present study revealed increased glutamine synthesis compared with glutamate formation in a widespread cortical area with sustained epileptiform activities, possibly a result of chronic excessive glutamate release from neurons and subsequent uptake into astrocytes.
Adult↗
Complex behavioral automatism arising from insular cortex.
We describe two cases of complex partial seizures with ictal violent movements arising from the insular cortex. The first patient, a 14-year-old girl, presented with hyperkinetic behavior such as rolling, thrashing, and pedaling, and the second case, a 38-year-old woman, had been suffering from frequent daytime hyperkinetic seizures characterized by bizarre vocalization, jumping, and violent bimanual movements. Both patients showed a slight high signal change in the right posterior ventral insular cortex in fluid-attenuated inversion recovery (FLAIR) studies involving magnetic resonance imaging, and extensive subdural electroencephalographic monitoring revealed EEG seizure onset from the temporal lobe. The posterior ventral insular and lateral temporal cortices were resected, resulting in complete seizure freedom in both cases. The histological diagnoses were focal cortical dysplasia in the first case and gliosis in the second case. There may exist a group of patients with complex partial seizures with ictal violent automatism that can be ameliorated by the resection of epileptogenic lesions in the insular cortex. Careful inspection of the insular cortex is necessary to diagnose this type of epileptic seizure.
Adolescent↗
Carbon 13-labeled magnetic resonance spectroscopy observation of cerebral glucose metabolism: metabolism in MELAS: case report.
BACKGROUND: Carbon 13-labeled magnetic resonance spectroscopy ((13)C-MRS) with [(1-13)C]-glucose administration, the (13)C atom that behaves as a radio inactive tracer in the brain, can differentiate aerobic and anaerobic glucose metabolism by detecting [(4-13)C]-glutamate (Glu C4) and [(3-13)C]-lactate (Lac C3). OBJECTIVE: To investigate the cerebral metabolic derangement resulting from mitochondrial dysfunction in mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). DESIGN: Application of a new (13)C-MRS technique to a patient with MELAS compared with control subjects (n = 7). PATIENT: A 19-year-old woman with an A3243G mitochondrial mutation who underwent (13)C-MRS for 30 minutes after oral administration of [(1-13)C]-glucose (0.75 g/kg). RESULT: Decreased Glu C4-labeling (P<.001) and increased Lac C3 synthesis (>2 SDs) compared with controls were demonstrated in the patient with MELAS. CONCLUSIONS: This first report on (13)C-MRS observation of cerebral glucose metabolism in a patient with MELAS demonstrated the presence of low glutamate production via the tricarboxylic acid cycle compared with high lactate synthesis by glycolysis. The present findings suggest that the clinical use of (13)C-MRS can be extended to diagnose mitochondrial dysfunction and monitor cerebral glucose metabolism in a variety of mitochondrial disorders.
Adult↗
Neuroimaging and presurgical evaluation of symptomatic epilepsies.
The goal of presurgical evaluation of intractable epilepsy is to identify epileptogenic regions in the brain. From our experience of 38 cases of resective epilepsy surgery from the last 3 years, ictal SPECT was considered the most sensitive at detecting focal changes relating to seizures compared to other neuroimaging modalities, such as MRI, FDG-PET, SPECT and MEG. At interictal state, on the other hand, FDG-PET was most sensitive, especially in cases with focal cortical dysplasia, which is often MRI-invisible. In dysplastic tumors, MRI showed the highest concordance rate to clinically verified epileptogenic regions. Activation studies using functional neuroimaging such as PET and fMRI is useful to evaluate brain functions at epileptogenic regions presurgically. The role of functional brain imaging in epilepsy surgery is considered to be: (i). case selection for resective surgery, (ii). case selection for invasive EEG monitoring, and (iii). navigation of electrode placement and cortical resection.
Brain↗
A comparison of (13)C NMR measurements of the rates of glutamine synthesis and the tricarboxylic acid cycle during oral and intravenous administration of [1-(13)C]glucose.
13C-labeled glucose is increasingly used in conjunction with magnetic resonance spectroscopy to measure rates of metabolic pathways in the brain in vivo. Most studies of human subjects have used intravenous infusions to administer the labeled compounds, but the procedure is cumbersome and can be uncomfortable for patients with neurological or psychiatric disorders. It may be possible to improve the practicality of the method by administering the glucose orally instead of intravenously. This report describes the performance and comparison of the oral and intravenous protocols in the same subjects. The conclusion is that oral administration does yield the same result as intravenous administration but with lower precision. That sensitivity of the oral protocol may be improved by several ways that are available today.
Administration, Oral↗
Neural basis of the retrieval of people's names: evidence from brain-damaged patients and fMRI.
The aim of this study was to identify the neuroanatomical basis of the retrieval of people's names. Lesion data showed that patients with language-dominant temporal lobectomy had impairments in their ability to retrieve familiar and newly learned people's names, whereas patients with language-nondominant temporal lobectomy had difficulty retrieving newly learned people's names. Functional magnetic resonance imaging experiments revealed activations in the left temporal polar region during the retrieval of familiar and newly learned people's names, and in the right superior temporal and bilateral prefrontal cortices during the retrieval of newly learned information from face cues. These data provide new evidence that the left anterior temporal region is crucial for the retrieval of people's names irrespective of their familiarity and that the right superior temporal and bilateral prefrontal areas are crucial for the process of associating newly learned people's faces and names.
Adolescent↗
Anaplastic ganglioglioma with sarcomatous component: an immunohistochemical study and molecular analysis of p53 tumor suppressor gene.
The present case report describes a case of ganglioglioma with a distinct sarcomatous component in the left temporal lobe of a 59-year-old Japanese man. Neoplastic neuroglial tissue contained both benign and anaplastic glial components with a MIB-1 labeling index of 0.1% and 12.0%, respectively. Sarcomatous tissue adjacent to the anaplastic glial tissue was dominated by pleomorphic fibroblastic cells with a MIB-1 labeling index of 10.8%. They were immunoreactive for smooth muscle actin, type IV collagen, and alpha 1 antitrypsin, but not for desmin and CD34. Interestingly, some of the sarcomatous cells were double-positive for smooth muscle actin and GFAP. The p53 protein had accumulated in the anaplastic astrocytes and sarcomatous cells, but direct DNA sequencing of PCR products failed to detect any mutation in the p53 gene (from exon 4 to exon 10).
Actins↗
Visual activation positron emission tomography for presurgical evaluation of occipital lobe epilepsy--case report.
A 14-year-old boy suffered from daily epileptic seizures originating from the left polymicrogyric occipital cortex. Visual activation positron emission tomography (PET) was used to map the function of the occipital cortex presurgically. Loss of visual function in the left occipital cortex was suggested by both visual activation PET and electrical cortical stimulation. Left occipital lobectomy resulted in a completely seizure-free status without deterioration in the visual function. Preoperative evaluation of the visual function in the epileptogenic occipital cortex by activation studies using PET or functional magnetic resonance imaging is the key to the successful surgical treatment of occipital lobe epilepsy.
Adolescent↗