95 results on '"Mukae N"'
Search Results
2. Degradation of chromosomal DNA during apoptosis
- Author
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Nagata, S, primary, Nagase, H, additional, Kawane, K, additional, Mukae, N, additional, and Fukuyama, H, additional
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- 2003
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3. The human caspase-activated DNase gene (hCAD): genomic structure, exonic single-nucleotide polymorphisms, and a highly polymorphic dinucleotide repeat at the hCAD locus
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Sugimoto, N., primary, Fukuda, Y., additional, Saito-Ohara, F., additional, Kamiyama, R., additional, Nakagawara, A., additional, Mukae, N., additional, Nagata, S., additional, and Inazawa, J., additional
- Published
- 1999
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4. An ECG electrode using a long 23G needle in burn patients
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Shidayama, R., primary, Mukae, N., additional, and Furukawa, M., additional
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- 1994
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5. EC syndrome In a girl with paracentric inversion (7)(q22A;q36.3)
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AKITA, S., primary, KURATOMI, H., additional, ABE, K., additional, HARADA, N., additional, MUKAE, N., additional, and NIIKAWA, N., additional
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- 1993
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6. Diagnostic value of serum antibody to candida in an extensively burned patient
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Kobayashi, K., primary, Mukae, N., additional, Matsunag, Y., additional, and Hotchi, M., additional
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- 1990
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7. EC syndrome In a girl with paracentric inversion (7)(q22A;q36.3)
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Mukae N, Harada N, Sadanori Akita, Niikawa N, Kuratomi H, and Abe K
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Chromosome 7 (human) ,medicine.medical_specialty ,Ectrodactyly ,business.industry ,Locus (genetics) ,General Medicine ,medicine.disease ,Penetrance ,Dermatology ,Pathology and Forensic Medicine ,stomatognathic diseases ,Bilateral cleft lip ,Pediatrics, Perinatology and Child Health ,Medicine ,Anatomy ,Allele ,Genomic imprinting ,business ,Genetics (clinical) ,Chromosomal inversion - Abstract
Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome associated with a paracentric inversion of chromosome 7 in a 2-year-old Japanese girl is reported. She had sparse and light-brown hair, bilateral cleft lip and palate, fused lower incisors, a pigmented skin lesion at the neck, accessory nipples, limited extension of elbow joints and bilateral ectrodactyly of hands and feet. Cytogenetic studies demonstrated a balanced inv(7)(q22.1;q36.3) in the patient and her father. The association of EEC syndrome and inv(7) in the patient suggested a putative locus of the EEC syndrome gene either at 7q22.1 or 7q36.3, although a coincidental occurrence of the two conditions is an alternative explanation. A comparison with reported karyotypes in patients with EEC or isolated ectrodactyly favoured 7q22.1 as the locus. A normal phenotype of the father in our family might reflect reduced penetrance of the EEC syndrome or, possibly, reduced expression of a maternally-derived allele of the EEC syndrome gene through a genomic imprinting mechanism.
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- 1993
8. Clinical effects of allograft.
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Namba K, Koga Y, Mukae N, Ogata S, and Nakamura K
- Published
- 1984
9. A novel activation mechanism of caspase-activated DNase from Drosophila melanogaster.
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Yokoyama, H, Mukae, N, Sakahira, H, Okawa, K, Iwamatsu, A, and Nagata, S
- Abstract
Caspase-activated DNase (CAD) is an enzyme that cleaves chromosomal DNA in apoptotic cells. Here, we identified a DNase in Drosophila Schneider cells that can be activated by caspase 3, and purified it as a complex of two subunits (p32 and p20). Using primers based on the amino acid sequence of the purified proteins, a cDNA coding for Drosophila CAD (dCAD) was cloned. The polypeptide encoded by the cDNA contained 450 amino acids with a calculated M(r) of 52,057, and showed significant homology with human and mouse CAD (22% identity). Mammalian CADs carry a nuclear localization signal at the C terminus. In contrast, dCAD lacked the corresponding sequence, and the purified dCAD did not cause DNA fragmentation in nuclei in a cell-free system. When dCAD was co-expressed in COS cells with Drosophila inhibitor of CAD (dICAD), a 52-kDa dCAD was produced as a heterotetrameric complex with dICAD. When the complex was treated with human caspase 3 or Drosophila caspase (drICE), the dICAD was cleaved, and released from dCAD. In addition, dCAD was also cleaved by these caspases, and behaved as a (p32)(2)(p20)(2) complex in gel filtration. When a Drosophila neuronal cell line was induced to apoptosis by treatment with a kinase inhibitor, both dCAD and dICAD were cleaved. These results indicated that unlike mammalian CAD, Drosophila CAD must be cleaved by caspases to be activated.
- Published
- 2000
10. Venous drainage through bone marrow after replantation: an experimental study
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Tanaka, K., Kobayashi, K., Murakami, R., Tasaki, Y., Fujii, T., and Mukae, N.
- Abstract
Venous drainage is vital for successful replantation, but it is not always possible to reconstruct because of missing or damaged veins. We devised an experimental model to study venous drainage through bone marrow while the new subcutaneous venous system regenerated. Adult male Wistar rats were placed into three groups. Group A rats had their hindlimbs amputated at thelower leg, but the tibia and sural and saphenous artery connections were preserved. Group B rats were prepared the same as Group A, except that a step-cut osteotomy was performed in the tibia. The bone ends were then realigned and kept in place with stainless steel wire. Group C rats were prepared the sameas Group B, except that the ends of the bone were not aligned. All unoperated limbs served as controls for evaluations of blood flow. Experimental limbs were evaluated for skin colour and viability, blood flow and dye injection. Skin colour was investigated daily. Blood flow was measured postoperatively during three phases: immediate (up to 1 h), early (from 1 h to 24 h), and late (from 1 day to 7 days after operation). Survival of limbs varied in Groups A and B, while all limbs in Group C necrosed by day 7. Blood flow was returning to near control (normal) levels by day 7 in Group A and B limbs. India inkwas observed in the medullary cavity at day 7. After replantation, bone marrow plays a critical role in venous drainage until the subcutaneous venous drainage system regenerates. es.
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- 1998
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11. Glial fibrillary acidic protein immunopositive neuroglial tissues with or without ependyma-lined canal in spinal lipoma of filar type: Relationship with retained medullary cord.
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Murakami N, Morioka T, Kurogi A, Suzuki SO, Shimogawa T, Mukae N, and Yoshimoto K
- Abstract
Background: Retained medullary cord (RMC) and filar lipomas are believed to originate from secondary neurulation failure; filar lipomas are reported to histopathologically contain a central canal-like ependyma-lined lumen with surrounding neuroglial tissue with ependyma-lined central canal (NGT w/E-LC) as a remnant of the medullary cord, which is a characteristic histopathology of RMC. With the addition of glial fibrillary acidic protein (GFAP) immunostaining, we reported the presence of GFAP-positive NGT without E-LCs (NGT w/o E-LCs) in RMC and filar lipomas, and we believe that both have the same embryopathological significance., Methods: We examined the frequency of GFAP-positive NGT, with or without E-LC, in 91 patients with filar lipoma., Results: Eight patients (8.8%) had NGT w/E-LC, 25 patients (27.5%) had NGT w/o E-LC, and 18 patients (19.8%) had tiny NGT w/o E-LC that could only be identified by GFAP immunostaining. Combining these subgroups, 56% of the patients ( n = 51) with filar lipoma had GFAP immunopositive NGT., Conclusion: The fact that more than half of filar lipomas have NGT provides further evidence that filar lipoma and RMC can be considered consequences of a continuum of regression failure that occurs during late secondary neurulation., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)
- Published
- 2024
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12. Expanding Ventricular Diverticulum Overlying the Cerebral Hemisphere through an Open-Lip Schizencephalic Cleft: A Report of Two Pediatric Cases.
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Murakami N, Kurogi A, Shono T, Torio M, Shimogawa T, Mukae N, Morioka T, and Yoshimoto K
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- Humans, Male, Female, Diverticulum surgery, Diverticulum diagnostic imaging, Magnetic Resonance Imaging, Hydrocephalus surgery, Hydrocephalus diagnostic imaging, Hydrocephalus etiology, Infant, Cerebral Ventricles diagnostic imaging, Cerebral Ventricles surgery, Schizencephaly diagnostic imaging, Schizencephaly surgery, Schizencephaly complications
- Abstract
Introduction: Open-lip-type schizencephaly is characterized by trans-cerebral clefts filled with cerebrospinal fluid (CSF) between the subarachnoid space at the hemisphere surface and the lateral ventricles. Disorders related to CSF retention, including hydrocephalus and arachnoid cysts, have reportedly been associated with open-lip schizencephaly and have induced intracranial hypertension in some cases. However, detailed neuroimaging and surgical treatment findings have rarely been described., Case Presentation: We report 2 cases of open-lip schizencephaly with an expanding CSF-filled cavity overlying the ipsilateral cerebral hemisphere that manifested as signs of intracranial hypertension. Detailed three-dimensional heavily T2-weighted imaging revealed thin borders between the CSF-filled cavity and the subarachnoid space, but no separating structures between the cavity and the lateral ventricle, suggesting that the cavity was directly connected to the lateral ventricle through the schizencephalic cleft but not to the subarachnoid space. Neuroendoscopic observation in case 1 confirmed this finding. Endoscopic fenestration of the cavity to the prepontine cistern was ineffective in case 1. Shunting between the lateral ventricle (case 1) or CSF-filled cavity (case 2) and the peritoneal cavity slightly decreased the size of the CSF-filled cavity., Discussion: We speculate that the thin borders along the margin of the CSF-filled cavity are membranes that previously covered the schizencephalic cleft and are now pushed peripherally. In addition, we believe that the cavity is a ventricular diverticulum protruding through the cleft and that shunting operation is effective against such expanding cavity. Detailed magnetic resonance imaging can be useful for evaluating patients with schizencephaly associated with CSF retention disorders., (© 2024 The Author(s). Published by S. Karger AG, Basel.)
- Published
- 2024
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13. Retained medullary cord and caudal lipoma with histopathological presence of terminal myelocystocele in the epidural stalk.
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Kurogi A, Murakami N, Suzuki SO, Shimogawa T, Mukae N, Yoshimoto K, and Morioka T
- Abstract
Background: The retained medullary cord (RMC), caudal lipoma, and terminal myelocystocele (TMCC) are thought to originate from the failed regression spectrum during the secondary neurulation, and the central histopathological feature is the predominant presence of a central canal-like ependyma-lined lumen (CC-LELL) with surrounding neuroglial tissues (NGT), as a remnant of the medullary cord. However, reports on cases in which RMC, caudal lipoma, and TMCC coexist are very rare., Case Description: We present two patients with cystic RMC with caudal lipoma and caudal lipoma with an RMC component, respectively, based on their clinical, neuroradiological, intraoperative, and histopathological findings. Although no typical morphological features of TMCC were noted on neuroimaging, histopathological examination revealed that a CC-LELL with NGT was present in the extraspinal stalk, extending from the skin lesion to the intraspinal tethering tract., Conclusion: This histopathological finding indicates the presence of TMCC that could not be completely regressed and further supports the idea that these pathologies can be considered consequences of a continuum of regression failure during secondary neurulation., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
- Published
- 2023
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14. Virtual reality environments to train soft skills in medical and nursing education: a technical feasibility study between France and Japan.
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Le Duff M, Michinov E, Bracq MS, Mukae N, Eto M, Descamps J, Hashizume M, and Jannin P
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- Humans, Feasibility Studies, Japan, Health Personnel education, Clinical Competence, Virtual Reality, Education, Nursing
- Abstract
Purpose: To meet the urgent and massive training needs of healthcare professionals, the use of digital technologies is proving increasingly relevant, and the rise of digital training platforms shows their usefulness and possibilities. However, despite the impact of these platforms on the medical skills learning, cultural differences are rarely factored in the implementation of these training environments., Methods: By using the Scrub Nurse Non-Technical Skills Training System (SunSet), we developed a methodology enabling the adaptation of a virtual reality-based environment and scenarios from French to Japanese cultural and medical practices. We then conducted a technical feasibility study between France and Japan to assess virtual reality simulations acceptance among scrub nurses., Results: Results in term of acceptance do not reveal major disparity between both populations, and the only emerging significant difference between both groups is on the Behavioral Intention, which is significantly higher for the French scrub nurses. In both cases, participants had a positive outlook., Conclusion: The findings suggest that the methodology we have implemented can be further used in the context of cultural adaptation of non-technical skills learning scenarios in virtual environments for the training and assessment of health care personnel., (© 2023. CARS.)
- Published
- 2023
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15. Mandibular and chin electrodes as a supplemental recording for detection of epileptiform discharges in mesial temporal lobe epilepsy.
- Author
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Shimogawa T, Sakata A, Watanabe E, Mukae N, Shigeto H, Mukaino T, Okadome T, Yamaguchi T, Yoshimoto K, and Morioka T
- Abstract
Background: We previously demonstrated the usefulness of periorbital electrodes in supplemental recording to detect epileptiform discharges in patients with mesial temporal lobe epilepsy (MTLE). However, eye movement may disturb periorbital electrode recording. To overcome this, we developed mandibular (MA) and chin (CH) electrodes and examined whether these electrodes could detect hippocampal epileptiform discharges., Methods: This study included a patient with MTLE, who underwent insertion of bilateral hippocampal depth electrodes and video-electroencephalographic (EEG) monitoring with simultaneous recordings of extra- and intracranial EEG as part of a presurgical evaluation. We examined 100 consecutive interictal epileptiform discharges (IEDs) recorded from the hippocampus and two ictal discharges. We compared these IEDs from intracranial electrodes with those from extracranial electrodes such as MA and CH electrodes in addition to F7/8 and A1/2 of international EEG 10-20 system, T1/2 of Silverman, and periorbital electrodes. We analyzed the number, rate of laterality concordance, and mean amplitude of IEDs detected in extracranial EEG monitoring and characteristics of IEDs on the MA and CH electrodes., Results: The MA and CH electrodes had nearly the same detection rate of hippocampal IEDs from other extracranial electrodes without contamination by eye movement. Three IEDs, not detected by A1/2 and T1/2, could be detected using the MA and CH electrodes. In two ictal events, the MA and CH electrodes detected the ictal discharges from the hippocampal onset as well as other extracranial electrodes., Conclusion: The MA and CH electrodes could detect hippocampal epileptiform discharges as well as A1/A2, T1/T2, and peri-orbital electrodes. These electrodes could serve as supplementary recording tools for detecting epileptiform discharges in MTLE., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
- Published
- 2023
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16. Severe type of segmental spinal dysgenesis with complete disconnection of the spinal cord and vertebra associated with open neural tube defect.
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Kurogi A, Murakami N, Shimogawa T, Mukae N, Suzuki SO, Yamaguchi T, Yoshimoto K, and Morioka T
- Abstract
Background: Severe type of segmental spinal dysgenesis (SSD) is a rare and complex anomaly in which the spinal cord completely disconnects at the portion of the spinal dysgenesis. Although closed spinal dysraphisms have been associated with SSD, to the best of our knowledge, the association between open neural tube defect (ONTD) and SSD is significantly rare, with only one case being reported to date., Case Description: We report a case of an infant with severe SSD and a disconnected spinal cord and spinal column at the thoracolumbar junction associated with myelomeningocele (MMC) in the lumbosacral region. The patient presented severe neurological deficits in the legs and impaired bowel function. The spinal column of L1-L3 was absent. The lower spinal segment consisted of neural placode at the L5-S1 level and no connecting structure between the upper and lower spinal cords. A repair surgery for MMC, including cord untethering and dura plasty, was performed. Histopathological findings revealed a neural placode consisting of a neuroglial tissue and leptomeninges., Conclusion: The management of severe SSD during the perinatal period is more challenging when it is associated with ONTD. We report detailed neuroradiological, intraoperative, and histological findings of such a case and discuss the embryopathogenesis of the associated ONTD and the treatment strategies., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
- Published
- 2023
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17. Detection of ictal and periictal hyperperfusion with subtraction of ictal-interictal 1.5-Tesla pulsed arterial spin labeling images co-registered to conventional magnetic resonance images (SIACOM).
- Author
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Abe K, Shimogawa T, Mukae N, Ikuta K, Shono T, Tanaka A, Sakata A, Shigeto H, Yoshimoto K, and Morioka T
- Abstract
Background: Our recent report showed that 1.5-T pulsed arterial spin labeling (ASL) magnetic resonance (MR) perfusion imaging (1.5-T Pulsed ASL [PASL]), which is widely available in the field of neuroemergency, is useful for detecting ictal hyperperfusion. However, the visualization of intravascular ASL signals, namely, arterial transit artifact (ATA), is more remarkable than that of 3-T pseudocontinuous ASL and is easily confused with focal hyperperfusion. To eliminate ATA and enhance the detectability of (peri) ictal hyperperfusion, we developed the subtraction of ictal-interictal 1.5-T PASL images co-registered to conventional MR images (SIACOM)., Methods: We retrospectively analyzed the SIACOM findings in four patients who underwent ASL during both (peri) ictal and interictal states and examined the detectability for (peri) ictal hyperperfusion., Results: In all patients, the ATA of the major arteries was almost eliminated from the subtraction image of the ictal-interictal ASL. In patients 1 and 2 with focal epilepsy, SIACOM revealed a tight anatomical relationship between the epileptogenic lesion and the hyperperfusion area compared with the original ASL image. In patient 3 with situation-related seizures, SIACOM detected minute hyperperfusion at the site coinciding with the abnormal electroencephalogram area. SIACOM of patient 4 with generalized epilepsy diagnosed ATA of the right middle cerebral artery, which was initially thought to be focal hyperperfusion on the original ASL image., Conclusion: Although it is necessary to examine several patients, SIACOM can eliminate most of the depiction of ATA and clearly demonstrate the pathophysiology of each epileptic seizure., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
- Published
- 2023
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18. Reflection of the Ictal Electrocorticographic Discharges Confined to the Medial Temporal Lobe to the Scalp-Recorded Electroencephalogram.
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Mukae N, Shimogawa T, Sakata A, Uehara T, Shigeto H, Yoshimoto K, and Morioka T
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- Humans, Electrocorticography, Scalp, Temporal Lobe, Electroencephalography, Epilepsy, Temporal Lobe diagnosis
- Abstract
Objective: Previous reports on the simultaneous recording of electroencephalography (EEG) and electrocorticography (ECoG) have demonstrated that, in patients with temporal lobe epilepsy (TLE), ictal ECoG discharges with an amplitude as high as 1000 μV originating from the medial temporal lobe could not be recorded on EEG. In contrast, ictal EEG discharges were recorded after ictal ECoG discharges propagated to the lateral temporal lobe. Here, we report a case of TLE in which the ictal EEG discharges, corresponding to ictal ECoG discharges confined to the medial temporal lobe, were recorded. Case report: In the present case, ictal EEG discharges were hardly recognized when the amplitude of the ECoG discharges was less than 1500 μV. During the evolution and burst suppression phase, corresponding to highly synchronized ECoG discharges with amplitudes greater than 1500 to 2000 μV, rhythmic negative waves with the same frequency were clearly recorded both on the lateral temporal lobe and scalp. The amplitude of the lateral temporal ECoG was approximately one-tenth of that of the medial temporal ECoG. The amplitude of the scalp EEG was approximately one-tenth of that of the lateral temporal ECoG. Conclusions: Highly synchronized ictal ECoG discharges with high amplitude of greater than 1500 to 2000 μV in the medial temporal lobe could be recorded on the scalp as ictal EEG discharges via volume conduction.
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- 2023
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19. Ectopic dorsal root ganglion in cauda equina mimicking schwannoma in a child.
- Author
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Murakami N, Kurogi A, Suzuki SO, Akitake N, Shimogawa T, Mukae N, Yoshimoto K, and Morioka T
- Abstract
Background: A heterotopic dorsal root ganglion (DRG) is sometimes observed in the vicinity of dysplastic neural structures during surgery for open spinal dysraphism; however, it is rarely associated with closed spinal dysraphism. Distinguish from neoplasms by preoperative imaging study is difficult. Although the embryopathogenesis of a heterotopic DRG has been speculated to be migration disorder of neural crest cells from primary neural tube, its details remain unelucidated., Case Description: We report a pediatric case with an ectopic DRG in cauda equina associated with a fatty terminal filum and bifid sacrum. The DRG mimicked a schwannoma in the cauda equina on preoperative magnetic resonance imaging. Laminotomy at L3 revealed that the tumor was entangled in the nerve roots, and small parts of the tumor were resected for biopsy. Histopathologically, the tumor consisted of ganglion cells and peripheral nerve fibers. Ki-67 immunopositive cells were observed at the periphery of the ganglion cells. These findings indicate the tumor comprised DRG tissue., Conclusion: We report detailed neuroradiological, intraoperative and histological findings and discuss the embryopathogenesis of the ectopic DRG. One should be aware of the possibility of ectopic or heterotopic DRGs when cauda equina tumors are observed in pediatric patients with neurulation disorders., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
- Published
- 2023
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20. PDZD8-deficient mice manifest behavioral abnormalities related to emotion, cognition, and adaptation due to dyslipidemia in the brain.
- Author
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Kurihara Y, Mitsunari K, Mukae N, Shoji H, Miyakawa T, and Shirane M
- Subjects
- Animals, Mice, Cognition, Fear, Lipid Metabolism, Brain physiopathology, Dyslipidemias complications, Adaptor Proteins, Signal Transducing genetics
- Abstract
Although dyslipidemia in the brain has been implicated in neurodegenerative disorders, the molecular mechanisms underlying its pathogenesis have been largely unclear. PDZD8 is a lipid transfer protein and mice deficient in PDZD8 (PDZD8-KO mice) manifest abnormal accumulation of cholesteryl esters (CEs) in the brain due to impaired lipophagy, the degradation system of lipid droplets. Here we show the detailed mechanism of PDZD8-dependent lipophagy. PDZD8 transports cholesterol to lipid droplets (LDs), and eventually promotes fusion of LDs and lysosomes. In addition, PDZD8-KO mice exhibit growth retardation, hyperactivity, reduced anxiety and fear, increased sensorimotor gating, and impaired cued fear conditioned memory and working memory. These results indicate that abnormal CE accumulation in the brain caused by PDZD8 deficiency affects emotion, cognition and adaptive behavior, and that PDZD8 plays an important role in the maintenance of brain function through lipid metabolism., (© 2023. The Author(s).)
- Published
- 2023
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21. Histopathological presence of dermal elements in resected margins of neural structures obtained from initial repair surgery for myelomeningocele.
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Murakami N, Kurogi A, Suzuki SO, Shimogawa T, Mukae N, Yoshimoto K, and Morioka T
- Abstract
Background: Development of dermoid or epidermoid cysts in myelomeningocele (MMC) sites is generally thought to occur in a delayed fashion due to implantation of dermal elements during initial repair surgery. Another theory is that dermal and dermoid elements may already be present within dysplastic neural structures at birth., Methods: We experienced histopathological presence of dermal elements in resected tissues at initial repair surgery in four out of 18 cases with MMC who required resection of parts or margins of the neural structures to perform cord untethering. Since one of these cases has already been reported, we describe the clinicopathological findings for the remaining three cases., Results: In Case1, cryptic dermoid elements were discovered in the terminal filum-like structure (FT-LS) caudal to the open neural placode (NP). The FT-LS had histopathological characteristics similar to the retained medullary cord. In Case 2, dermoid elements were discovered in the caudal margin of the dysplastic conus medullaris. In Case 3, a thin squamous epithelial layer overlapped the rostral margin of the NP where the NP was located near the skin. Case 1 developed an epidermoid cyst at 1 year and 2 months of age, which was totally resected., Conclusion: Prenatally existing cryptic dermoid elements in the caudal portion of neural structures and remnants of dermal elements overlapping the rostral margin of the NP are associated with delayed occurrence of dermoid/ epidermoid cysts. Postoperative histopathological investigation of the resected specimens is recommended. Once dermal elements are revealed, repeated imaging examination and additional surgery should be considered., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Surgical Neurology International.)
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- 2023
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22. PDZD8-deficient mice accumulate cholesteryl esters in the brain as a result of impaired lipophagy.
- Author
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Morita K, Wada M, Nakatani K, Matsumoto Y, Hayashi N, Yamahata I, Mitsunari K, Mukae N, Takahashi M, Izumi Y, Bamba T, and Shirane M
- Abstract
Dyslipidemia including the accumulation of cholesteryl esters (CEs) in the brain is associated with neurological disorders, although the underlying mechanism has been unclear. PDZD8, a Rab7 effector protein, transfers lipids between endoplasmic reticulum (ER) and Rab7-positive organelles and thereby promotes endolysosome maturation and contributes to the maintenance of neuronal integrity. Here we show that CEs accumulate in the brain of PDZD8-deficient mice as a result of impaired lipophagy. This CE accumulation was not affected by diet, implicating a defect in intracellular lipid metabolism. Whereas cholesterol synthesis appeared normal, degradation of lipid droplets (LDs) was defective, in the brain of PDZD8-deficient mice. PDZD8 may mediate the exchange of cholesterol and phosphatidylserine between ER and Rab7-positive organelles to promote the fusion of CE-containing LDs with lysosomes for their degradation. Our results thus suggest that PDZD8 promotes clearance of CEs from the brain by lipophagy, with this role of PDZD8 likely contributing to brain function., Competing Interests: The authors declare no competing interests., (© 2022 The Authors.)
- Published
- 2022
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23. Good seizure outcome after focal resection surgery for super-refractory status epilepticus: Report of two cases.
- Author
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Yonamoto A, Mukae N, Shimogawa T, Uehara T, Shigeto H, Sakata A, Mizoguchi M, Yoshimoto K, and Morioka T
- Abstract
Background: There is scarce evidence regarding focal resection surgery for super-refractory status epilepticus (SRSE), which is resistant to general anesthetic treatment over 24 h. We report two patients with SRSE, in whom good seizure outcomes were obtained following focal resection surgery., Case Description: Patient 1: A 58-year-old man who underwent left anterior temporal lobectomy with hippocampectomy at the age of 38 years after being diagnosed left medial temporal lobe epilepsy. After 19 years of surgery with no epileptic attacks, the patient developed SRSE. Electroencephalogram (EEG) demonstrated persistence of lateralized periodic discharges in the left frontotemporal region. On the 20
th day after SRSE onset, resection of the frontal lobe and temporal lobe posterior to the resection cavity was performed. Patient 2: A 62-year-old man underwent craniotomy for anaplastic astrocytoma in the left frontal lobe at the age of 34 years. Since the age of 60 years, he developed SRSE 3 times over 1 and 1/12 years. On EEG, repeated ictal discharges were observed at the medial part of the left frontal region during the three SRSEs. Corresponding to the ictal EEG findings, high signals on diffusion-weighted magnetic resonance images and focal hypermetabolism on fluorodeoxyglucose-positron emission tomography were observed around the supplementary motor area, medial to the resection cavity. Resection surgery of the area was performed during the interictal period., Conclusion: Good seizure outcome was obtained in the two cases which provide additional support for the recent concept of focal resection surgery as an indication for SRSE., Competing Interests: There are no conflict of interest., (Copyright: © 2022 Surgical Neurology International.)- Published
- 2022
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24. Implications and limitations of magnetic resonance perfusion imaging with 1.5-Tesla pulsed arterial spin labeling in detecting ictal hyperperfusion during non-convulsive status epileptics.
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Goto K, Shimogawa T, Mukae N, Shono T, Fujiki F, Tanaka A, Sakata A, Shigeto H, Yoshimoto K, and Morioka T
- Abstract
Background: Recent our reports showed that 3-T pseudocontinuous arterial spin labeling (3-T pCASL) magnetic resonance perfusion imaging with dual post labeling delay (PLD) of 1.5 and 2.5 s clearly demonstrated the hemodynamics of ictal hyperperfusion associated with non-convulsive status epilepticus (NCSE). We aimed to examine the utility of 1.5-T pulsed arterial spin labeling (1.5-T PASL), which is more widely available for daily clinical use, for detecting ictal hyperperfusion., Methods: We retrospectively analyzed the findings of 1.5-T PASL with dual PLD of 1.5 s and 2.0 s in six patients and compared the findings with ictal electroencephalographic (EEG) findings., Results: In patients 1 and 2, we observed the repeated occurrence of ictal discharges (RID) on EEG. In patient 1, with PLDs of 1.5 s and 2.0 s, ictal ASL hyperperfusion was observed at the site that matched the RID localization. In patient 2, the RID amplitude was extremely low, with no ictal ASL hyperperfusion. In patient 3 with lateralized periodic discharges (LPD), we observed ictal ASL hyperperfusion at the site of maximal LPD amplitude, which was apparent at a PLD of 2.0 s but not 1.5 sec. Among three patients with rhythmic delta activity (RDA) of frequencies <2.5 Hz (Patients 4-6), we observed obvious and slight increases in ASL signals in patients 4 and 5 with NCSE, respectively. However, there was no apparent change in ASL signals in patient 6 with possible NCSE., Conclusion: The detection of ictal hyperperfusion on 1.5-T PASL might depend on the electrophysiological intensity of the epileptic ictus, which seemed to be more prominent on 1.5-T PASL than on 3-T pCASL. The 1.5-T PASL with dual PLDs showed the hemodynamics of ictal hyperperfusion in patients with RID and LPD. However, it may not be visualized in patients with extremely low amplitude RID or RDA (frequencies <2.5 Hz)., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Surgical Neurology International.)
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- 2022
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25. Combined neuroendoscopic cyst wall fenestration and cyst-peritoneal shunt in an infant with glioependymal cyst.
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Irie K, Shimogawa T, Mukae N, Kuga D, Iwaki T, Mizoguchi M, and Yoshimoto K
- Abstract
Background: Glioependymal cysts (GECs) are rare, benign congenital intracranial cysts that account for 1% of all intracranial cysts. Surgical interventions are required for patients with symptomatic GECs. However, the optimal treatment remains controversial, especially in infants. Here, we report a male infant case of GECs that successfully underwent minimally invasive combined neuroendoscopic cyst wall fenestration and cyst-peritoneal (CP) shunt., Case Description: The boy was delivered transvaginally at 38 weeks and 6 days of gestation with no neurological deficits. Magnetic resonance imaging (MRI) at birth revealed multiple cysts with smooth and rounded borders and a non-enhancing wall in the right parieto-occipital region. The size of the cyst had increased rapidly compared to that of the prenatal MRI, which was performed at 37 weeks and 2 days. On the day of birth, Ommaya cerebrospinal fluid (CSF) reservoir was placed into the largest outer cyst. The patient underwent intermittent CSF drainage; however, he experienced occasional vomiting. At 2 months, he underwent combined neuroendoscopic cyst wall fenestration and CP shunt through a small hole. The patient's postoperative course was uneventful and there was no recurrence of the cyst. The pathological diagnosis was GEC., Conclusion: Combined neuroendoscopic cyst wall fenestration and CP shunt are a minimally invasive and effective treatment for infants with GECs., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Surgical Neurology International.)
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- 2022
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26. Impaired visual acuity as an only symptom of shunt malfunction, long time after initial cyst-peritoneal shunting for arachnoid cyst: A case report.
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Ono K, Mukae N, Nishimura A, Arimura K, Mizoguchi M, Yoshimoto K, and Iihara K
- Abstract
Background: Long-term outcomes after surgical treatment of arachnoid cysts (ACs) have not been reported adequately. Impaired visual acuity is not a common symptom of shunt dependency syndrome due to cyst-peritoneal (CP) shunt malfunction for ACs. We report a case of CP shunt malfunction, who presented only impaired visual acuity as a symptom, long after the initial surgical treatment., Case Description: A 16-year-old boy was surgically treated for the left frontal AC with CP shunting at 2 years of age. Extension of the peritoneal shunt catheter was performed at 15 years of age. A year later, he started experiencing impairment of visual acuity without headaches, which worsened to bilateral light perception. The presence of bilateral optic atrophy was confirmed. The AC in the left frontal lobe had enlarged very slightly, with shortening of the intracystic catheter, and the cerebrospinal fluid pressure was elevated to 30 cmH2O. He was treated with lumboperitoneal shunting. The visual acuity showed limited improvement., Conclusion: The possibility of CP shunt malfunction and shunt dependency syndrome should be considered, even if the patient presented only impaired visual acuity and no significant changes in the size of the ACs are observed., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Surgical Neurology International.)
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- 2022
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27. Intramedullary abscess at thoracolumbar region transmitted from infected dermal sinus and dermoid through retained medullary cord.
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Matsubara Y, Murakami N, Kurogi A, Lee S, Mukae N, Shimogawa T, Shono T, Suzuki SO, Yoshimoto K, and Morioka T
- Abstract
Background: A retained medullary cord (RMC) is a relatively newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during secondary neurulation. A congenital dermal sinus (CDS) may provide a pathway for intraspinal infections such as repeated meningitis. Intramedullary abscesses are the rarest but most serious complication of a CDS., Case Description: We treated a female infant with an intramedullary abscess in the thoracolumbar region, which was caused by infection of the CDS. Surgery revealed that the cord-like structure (C-LS) started from the cord with the intramedullary abscess, extended to the dural cul-de-sac, and further continued to the CDS tract and skin dimple. The boundary between the functional cord and the non-functional CL-S was electrophysiologically identified, and the entire length of the C-LS (the RMC) with an infected dermoid cyst was resected. As a result, the abscess cavity was opened and thorough irrigation and drainage of the pus could be performed. Histopathological examination of the C-LS revealed an infected dermoid cyst and abscess cavity with keratin debris in the fibrocollagenous tissue. The abscess cavity had a central canal-like ependymal lined lumen (CCLELL), with surrounding glial fibrillary acidic protein (GFAP)-immunopositive neuroglial tissues., Conclusion: We demonstrated that the transmission of an infection through the RMC was involved in the development of the intramedullary abscess. A good postoperative outcome was obtained because a terminal ventriculostomy for pus drainage could be achieved by excising the nonfunctional RMC., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Surgical Neurology International.)
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- 2022
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28. Retained Medullary Cord Associated with Terminal Myelocystocele and Intramedullary Arachnoid Cyst.
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Kurogi A, Murakami N, Mukae N, Shimogawa T, Shono T, Suzuki SO, and Morioka T
- Subjects
- Female, Humans, Infant, Magnetic Resonance Imaging, Spinal Cord diagnostic imaging, Spinal Cord surgery, Spine pathology, Arachnoid Cysts complications, Arachnoid Cysts diagnostic imaging, Arachnoid Cysts surgery, Meningocele surgery, Meningomyelocele complications, Meningomyelocele diagnostic imaging, Meningomyelocele surgery, Spina Bifida Occulta complications, Spinal Dysraphism complications
- Abstract
Introduction: The retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during the last phase of secondary neurulation. The terminal myelocystocele (TMC) is an unusual type of closed spinal dysraphism, characterized by localized cystic dilatation of the terminal part of the central canal that then herniates through a posterior spinal bifida. The co-occurrence of RMC and TMC is extremely rare., Case Presentation: We treated a baby girl with a huge sacrococcygeal meningocele-like sac with two components. Untethering surgery and repair surgery for the sac revealed that RMC, associated with intramedullary arachnoid cyst (IMAC), was terminated at the bottom of the rostral cyst, forming the septum of the two cystic components, and the caudal cyst was TMC derived from the central canal-like ependymal lining lumen (CC-LELL) of the RMC at the septum. IMAC within the RMC communicated with TMC, and both contained xanthochromic fluid with the same properties., Conclusion: We speculated that the mass effect of the coexistent IMAC impeded the flow of cerebrospinal fluid in the CC-LELL within the RMC and eventually formed a huge TMC. In surgical strategies for such complex pathologies, it is important to identify the electrophysiological border between the functional cord and nonfunctional RMC and the severe RMC to untether the cord, as with a typical or simple RMC., (© 2022 S. Karger AG, Basel.)
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- 2022
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29. Simultaneous Electroencephalographic and Electocorticographic Recordings of Lateralized Periodic Discharges in Temporal Lobe Epilepsy.
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Sakata A, Mukae N, Morioka T, Tanaka S, Shimogawa T, Shigeto H, Hotta T, Kang D, and Mizoguchi M
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- Electrocorticography, Electroencephalography, Humans, Retrospective Studies, Epilepsy, Temporal Lobe diagnosis
- Abstract
Objective: Lateralized periodic discharges (LPDs), which constitute an abnormal electroencephalographic (EEG) pattern, are most often observed in critically ill patients with acute pathological conditions, and are less frequently observed in chronic conditions such as focal epilepsies, including temporal lobe epilepsy (TLE). Here we aim to explore the pathophysiological mechanism of LPD in TLE., Methods: We retrospectively selected 3 patients with drug-resistant TLE who simultaneously underwent EEG and electrocorticography (ECoG) and demonstrated LPDs. We analyzed the correlation between the EEG and ECoG findings., Results: In patients 1 and 2, LPDs were recorded in the temporal region of the scalp during the interictal periods, when repeated spikes followed by slow waves (spike-and-wave complexes; SWs) and periodic discharges (PDs) with amplitudes of >600 to 800 µV appeared in the lateral temporal lobe over a cortical area of >10 cm
2 . In patient 3, when the ictal discharges persisted and were confined to the medial temporal lobe, repeated SWs were provoked on the lateral temporal lobe. When repeated SWs with amplitudes of >800 µV appeared in an area of the lateral temporal lobe of >10 cm2 , the corresponding EEG discharges appeared on the temporal scalp., Conclusions: LPDs in patients with TLE originate from repeated SWs and PDs of the lateral temporal lobe, which might represent a highly irritable state of the lateral temporal cortex during both interictal and ictal periods.- Published
- 2022
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30. Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex.
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Murakami N, Kurogi A, Kawakami Y, Noguchi Y, Hayashida M, Suzuki SO, Mukae N, Shimogawa T, Yoshimoto K, and Morioka T
- Abstract
Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended., Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage., Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
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- 2021
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31. Paroxysmal sympathetic hyperactivity and the later development of epilepsy in a chemotherapy-associated brain damage.
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Taira R, Yamamura K, Maeda T, Sakata A, Watanabe E, Shimogawa T, Mukae N, Ikeda C, Migita M, Watanabe O, Koga Y, Sakai Y, and Ohga S
- Subjects
- Child, Female, Humans, Antineoplastic Agents adverse effects, Autonomic Nervous System Diseases chemically induced, Drug Resistant Epilepsy chemically induced, Leukemia, Myeloid, Acute drug therapy, Neurotoxicity Syndromes etiology
- Abstract
Background: Chemotherapy in childhood leukemia potentially induces brain lesions and neurological sequelae. Paroxysmal sympathetic hyperactivity (PSH) is known as a treatment-associated complication; however, the full clinical spectra of PSH remain to be elusive., Case Report: A 5-year-old girl was diagnosed of acute myeloid leukemia (AML) M5. After the intensification therapy, she developed recurrent symptoms of episodic tachycardia, hypertension and perspiration lasting for several hours per day. The low-frequency-high-frequency ratio on Holter electrocardiography was rapidly increased from 0.84 to 2.24 at the onset of the paroxysmal event, whereas the video-monitoring electroencephalography (EEG) never identified ictal patterns of epileptiform discharges during the episodes. Thus, the diagnosis of PSH was given at 7 years of age. Myoclonic and generalized tonic-clonic seizures frequently appeared from 10 years of age, which poorly responded to anticonvulsants. EEG showed diffuse slow-wave bursts with multifocal spikes. Serial head magnetic resonance imaging (MRI) revealed diffuse cerebral and hippocampal atrophy, but not inflammatory lesions in the limbic system., Conclusion: We first demonstrate a pediatric case with PSH who developed drug-resistant epilepsy 3 years after the onset of PSH. Our data suggest the pathophysiological link of persistent PSH with chemotherapy-associated brain damage., (Copyright © 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
- Published
- 2021
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32. Saccular Limited Dorsal Myeloschisis with Spinal Cord Deviation out of the Spinal Canal to the Sac.
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Kurogi A, Morioka T, Murakami N, Shimogawa T, Mukae N, Matsuo Y, Imamoto N, Tateishi Y, and Suzuki SO
- Abstract
Saccular limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the saccular skin lesion to the underlying spinal cord. Since untethering surgery during the early postnatal period is often indicated to prevent sac rupture, saccular LDM should be distinguished from myelomeningocele (MMC) during the perinatal period. We treated two patients with the spinal cord deviation from the spinal canal to the sac, which mimicked a prolapse of the neural placode into the MMC sac. In patient 1, pre- and postnatal magnetic resonance imaging (MRI) revealed that the spinal cord was strongly tethered to the thick stalk. During surgery, the dorsally bent cord and stalk were united, and the border between these two was determined with intraoperative neurophysiological mapping (IONM). In patient 2, the spinal cord was tethered to two slender stalks close to each other, which was visible with the combined use of sagittal and axial postnatal three-dimensional heavily T2-weighted imaging (3D-hT2WI). The preoperative MRI hallmark of saccular LDM is the visualization of a stalk that links the bending cord and sac. Complete untethering surgery to return the cord into the spinal canal and correct its dorsal bending is recommended., Competing Interests: Conflicts of Interest Disclosure The authors declare that they have no conflicts of interest., (© 2021 The Japan Neurosurgical Society.)
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- 2021
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33. Spinal cord deformity with aggravation of tethering in saccular limited dorsal myeloschisis during the first 2 months of life.
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Shimogawa T, Mukae N, Kanata A, Tsukamoto H, Murakami N, Kurogi A, Shono T, Suzuki SO, and Morioka T
- Abstract
Background: Although the optimal timing of prophylactic untethering surgery for limited dorsal myeloschisis (LDM) with intact or subtle neurological findings diagnosed at birth remains undetermined, intentional delayed surgery is commonly used for flat and tail-like LDM. Conversely, for saccular LDM, early surgery is indicated during the postnatal period because it prevents rupture of the sac. We treated a saccular LDM patient, in whom intentional delayed surgery was selected because the sac was thickly covered with normal skin. We describe the clinical course of the case and discuss the optimal timing of the surgery., Case Description: The patient had a dorsal midline sac in the upper lumbar region. Initial magnetic resonance imaging (MRI) after birth revealed a tethering tract that began at the dome of the sac and joined the lumbar cord. Dorsal bending of the cord at the stalk-cord union and invagination of the cord into the sac were noted. At 2 months, he was neurologically normal; however, the second MRI examination revealed that the cord tethering was aggravated. The cord was markedly displaced dorsally and to the left, with deviation of the cord to the sac out of the spinal canal. Following untethering surgery, the spinal cord deformity markedly improved., Conclusion: Early surgery may be recommended for saccular LDM when tethering is present, including dorsal bending of the cord at the stalk-cord union and invagination of the cord into the sac observed on detailed MRI examination, even if the sac has no risk of rupture., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
- Published
- 2021
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34. Rapidly spreading seizures arise from large-scale functional brain networks in focal epilepsy.
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Uehara T, Shigeto H, Mukaino T, Yokoyama J, Okadome T, Yamasaki R, Ogata K, Mukae N, Sakata A, Tobimatsu S, and Kira JI
- Subjects
- Adolescent, Adult, Drug Resistant Epilepsy, Electrocorticography, Female, Humans, Magnetic Resonance Imaging, Male, Retrospective Studies, Young Adult, Connectome, Epilepsies, Partial physiopathology, Nerve Net physiopathology, Seizures physiopathology
- Abstract
It remains unclear whether epileptogenic networks in focal epilepsy develop on physiological networks. This work aimed to explore the association between the rapid spread of ictal fast activity (IFA), a proposed biomarker for epileptogenic networks, and the functional connectivity or networks of healthy subjects. We reviewed 45 patients with focal epilepsy who underwent electrocorticographic (ECoG) recordings to identify the patients showing the rapid spread of IFA. IFA power was quantified as normalized beta-gamma band power. Using published resting-state functional magnetic resonance imaging databases, we estimated resting-state functional connectivity of healthy subjects (RSFC-HS) and resting-state networks of healthy subjects (RSNs-HS) at the locations corresponding to the patients' electrodes. We predicted the IFA power of each electrode based on RSFC-HS between electrode locations (RSFC-HS-based prediction) using a recently developed method, termed activity flow mapping. RSNs-HS were identified using seed-based and atlas-based methods. We compared IFA power with RSFC-HS-based prediction or RSNs-HS using non-parametric correlation coefficients. RSFC and seed-based RSNs of each patient (RSFC-PT and seed-based RSNs-PT) were also estimated using interictal ECoG data and compared with IFA power in the same way as RSFC-HS and seed-based RSNs-HS. Spatial autocorrelation-preserving randomization tests were performed for significance testing. Nine patients met the inclusion criteria. None of the patients had reflex seizures. Six patients showed pathological evidence of a structural etiology. In total, we analyzed 49 seizures (2-13 seizures per patient). We observed significant correlations between IFA power and RSFC-HS-based prediction, seed-based RSNs-HS, or atlas-based RSNs-HS in 28 (57.1%), 21 (42.9%), and 28 (57.1%) seizures, respectively. Thirty-two (65.3%) seizures showed a significant correlation with either seed-based or atlas-based RSNs-HS, but this ratio varied across patients: 27 (93.1%) of 29 seizures in six patients correlated with either of them. Among atlas-based RSNs-HS, correlated RSNs-HS with IFA power included the default mode, control, dorsal attention, somatomotor, and temporal-parietal networks. We could not obtain RSFC-PT and RSNs-PT in one patient due to frequent interictal epileptiform discharges. In the remaining eight patients, most of the seizures showed significant correlations between IFA power and RSFC-PT-based prediction or seed-based RSNs-PT. Our study provides evidence that the rapid spread of IFA in focal epilepsy can arise from physiological RSNs. This finding suggests an overlap between epileptogenic and functional networks, which may explain why functional networks in patients with focal epilepsy frequently disrupt., Competing Interests: Declaration of Competing Interest T.U. has received honoraria from Eisai, Daiichi Sankyo, and Novartis Pharma. H.S. has received honoraria from UCB, Eisai, and Daiichi Sankyo. R.Y. received honoraria from Teijin Pharma, Ono Pharmaceutical, Takeda Pharmaceutical, Eisai, Novartis, Nihon Pharmaceutical, and CSL Behring. J.K. has received honoraria from Biogen Japan, Bayer Healthcare, Novartis Pharma, Mitsubishi Tanabe Pharma, Eisai, Sanofi, Nobelpharma, Otsuka Pharmaceutical, Chugai Pharmaceutical Company, and Teijin Pharma. The other authors have no conflicts of interest to disclose., (Copyright © 2021. Published by Elsevier Inc.)
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- 2021
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35. Endonasal endoscopic surgery for temporal lobe epilepsy associated with sphenoidal encephalocele.
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Mukae N, Kuga D, Murakami D, Komune N, Miyamoto Y, Shimogawa T, Sakata A, Shigeto H, Iwaki T, Morioka T, and Mizoguchi M
- Abstract
Background: Temporal lobe epilepsy (TLE) associated with temporal lobe encephalocele is rare, and the precise epileptogenic mechanisms and surgical strategies for such cases are still unknown. Although the previous studies have reported good seizure outcomes following chronic subdural electrode recording through invasive craniotomy, only few studies have reported successful epilepsy surgery through endoscopic endonasal lesionectomy., Case Description: An 18-year-old man developed generalized convulsions at the age of 15 years. Despite treatment with optimal doses of antiepileptic drugs, episodes of speech and reading difficulties were observed 2-3 times per week. Long-term video electroencephalogram (EEG) revealed ictal activities starting from the left anterior temporal region. Magnetic resonance imaging revealed a temporal lobe encephalocele in the left lateral fossa of the sphenoidal sinus (sphenoidal encephalocele). Through the endoscopic endonasal approach, the tip of the encephalocele was exposed. A depth electrode was inserted into the encephalocele, which showed frequent spikes superimposed with high-frequency oscillations (HFOs) suggesting intrinsic epileptogenicity. The encephalocele was resected 8 mm from the tip. Twelve months postoperatively, the patient had no recurrence of seizures on tapering of the medication., Conclusion: TLE associated with sphenoidal encephalocele could be controlled with endoscopic endonasal lesionectomy, after confirming the high epileptogenicity with analysis of HFOs of intraoperative EEG recorded using an intralesional depth electrode., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
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- 2021
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36. Surgical histopathology of a filar anomaly as an additional tethering element associated with closed spinal dysraphism of primary neurulation failure.
- Author
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Morioka T, Murakami N, Suzuki SO, Mukae N, Shimogawa T, Kurogi A, Shono T, and Mizoguchi M
- Abstract
Background: Closed spinal dysraphism of primary neurulation failure could be associated with filar anomalies, such as filar lipoma or thickened and tight filum terminale (TFT), resulting from impaired secondary neurulation. Retained medullary cord (RMC) is a remnant of the cavitary medullary cord originating from the secondary neurulation failure. Some filar lipomas are known to contain a central canal-like ependyma-lined lumen with surrounding neuroglial tissues (E-LC w/NGT), that is, a characteristic histopathology of RMC. To clarify the embryological background of these filar anomalies, we evaluated the histopathological findings., Methods: Among 41 patients with lesions of primary neurulation failure who underwent initial untethering surgery, the filum including cord-like structure (C-LS) was additionally resected in 10 patients (five dorsal and transitional lipomas; five limited dorsal myeloschisis). We retrospectively analyzed the clinical, neuroradiological, intraoperative, and histopathological findings., Results: Among 10 patients, two patients were diagnosed with RMC based on morphological features and intraoperative neurophysiological monitoring. The diagnosis of filar lipoma was made in six patients, since various amounts of fibroadipose tissue were histopathologically noted in the filum. Two patients were diagnosed with TFT, since the filum was composed solely of fibrocollagenous tissue. E-LC w/NGT was noted not only in both C-LSs of RMCs but also in two out of six fila both with filar lipomas and fila with TFTs., Conclusion: These findings provide further evidence for the idea that entities, such as filar lipoma, TFT, and RMC, can be considered consequences of a continuum of regression failure occurring during late secondary neurulation., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
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- 2021
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37. CD206 Expression in Induced Microglia-Like Cells From Peripheral Blood as a Surrogate Biomarker for the Specific Immune Microenvironment of Neurosurgical Diseases Including Glioma.
- Author
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Tanaka S, Ohgidani M, Hata N, Inamine S, Sagata N, Shirouzu N, Mukae N, Suzuki SO, Hamasaki H, Hatae R, Sangatsuda Y, Fujioka Y, Takigawa K, Funakoshi Y, Iwaki T, Hosoi M, Iihara K, Mizoguchi M, and Kato TA
- Subjects
- Antigens, CD metabolism, Antigens, Differentiation, Myelomonocytic metabolism, Biomarkers, Tumor genetics, Brain Neoplasms immunology, Brain Neoplasms pathology, Brain Neoplasms surgery, Calcium-Binding Proteins metabolism, Cells, Cultured, Feasibility Studies, Female, Glioma immunology, Glioma pathology, Glioma surgery, Humans, Male, Membrane Glycoproteins genetics, Microfilament Proteins metabolism, Microglia immunology, Microglia pathology, Monitoring, Immunologic, Phenotype, Prognosis, Receptors, Immunologic genetics, Tumor Microenvironment, Biomarkers, Tumor metabolism, Brain Neoplasms blood, Glioma blood, Membrane Glycoproteins metabolism, Microglia metabolism, Receptors, Immunologic metabolism
- Abstract
Targeting the unique glioma immune microenvironment is a promising approach in developing breakthrough immunotherapy treatments. However, recent advances in immunotherapy, including the development of immune checkpoint inhibitors, have not improved the outcomes of patients with glioma. A way of monitoring biological activity of immune cells in neural tissues affected by glioma should be developed to address this lack of sensitivity to immunotherapy. Thus, in this study, we sought to examine the feasibility of non-invasive monitoring of glioma-associated microglia/macrophages (GAM) by utilizing our previously developed induced microglia-like (iMG) cells. Primary microglia (pMG) were isolated from surgically obtained brain tissues of 22 patients with neurological diseases. iMG cells were produced from monocytes extracted from the patients' peripheral blood. Quantitative reverse transcription-polymerase chain reaction (qRT-PCR) revealed a significant correlation of the expression levels of representative markers for M1 and M2 microglia phenotypes between pMG and the corresponding iMG cells in each patient (Spearman's correlation coefficient = 0.5225, P < 0.0001). Synchronous upregulation of CD206 expression levels was observed in most patients with glioma (6/9, 66.7%) and almost all patients with glioblastoma (4/5, 80%). Therefore, iMG cells can be used as a minimally invasive tool for monitoring the disease-related immunological state of GAM in various brain diseases, including glioma. CD206 upregulation detected in iMG cells can be used as a surrogate biomarker of glioma., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Tanaka, Ohgidani, Hata, Inamine, Sagata, Shirouzu, Mukae, Suzuki, Hamasaki, Hatae, Sangatsuda, Fujioka, Takigawa, Funakoshi, Iwaki, Hosoi, Iihara, Mizoguchi and Kato.)
- Published
- 2021
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38. A Pedicled Posterior Septal-Nasal Floor Flap and a Novel Rescue Flap for Skull Base Reconstruction.
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Murakami D, Kuga D, Miyamoto Y, Komune N, Mukae N, Saito Y, Iihara K, and Nakagawa T
- Subjects
- Adult, Aged, Child, Female, Humans, Male, Retrospective Studies, Treatment Outcome, Young Adult, Cerebrospinal Fluid Leak surgery, Nasal Septum surgery, Plastic Surgery Procedures methods, Skull Base surgery, Skull Base Neoplasms surgery, Surgical Flaps surgery
- Abstract
Objective: We devised a new surgical alternative to the conventional nasoseptal flap, a pedicled posterior septal-nasal floor flap that we named the Kegon flap. We evaluated the effectiveness of this flap for skull base reconstruction in patients with high-flow cerebrospinal fluid (CSF) leakage after sellar/parasellar tumor resection., Methods: The Kegon flap with a novel rescue flap was designed to preserve blood flow and mucosa anterior to the nasal septum and to avoid flap damage during surgery. We retrospectively evaluated postoperative flap perfusion with T1-weighted contrast-enhanced magnetic resonance imaging and characterized complications and wound healing in 5 patients who experienced high-flow CSF leakage after sellar/parasellar tumor resection requiring reconstruction., Results: Postoperative T1-weighted contrast-enhanced magnetic resonance imaging demonstrated good flap perfusion in all patients. The area reconstructed with the Kegon flap healed within the first month following surgery. No postoperative CSF leakage or nasal hemorrhage was observed. There was no perforation of the anterior nasal septum after surgery. The mucosal defect had completely epithelialized in all patients by 3 months after surgery. There were no instances of prolonged nasal crusting or any subjective decrease in olfactory function 3 months after surgery., Conclusions: The use of a Kegon flap with a novel rescue flap was effective and helped preserve nasal structure and function in patients undergoing skull base reconstruction after sellar/parasellar tumor resection associated with high-flow CSF leakage., (Copyright © 2021 Elsevier Inc. All rights reserved.)
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- 2021
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39. Corpus callosotomy for drug-resistant epilepsy in a pediatric patient with Waardenburg syndrome Type I.
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Shimogawa T, Mukae N, Morioka T, Sakata A, Sakai Y, Matsumoto N, and Mizoguchi M
- Abstract
Background: Waardenburg syndrome (WS) is caused by autosomal dominant mutations. Since the coexistence of epilepsy and WS type I is rare, the detailed clinical features and treatment of epilepsy, including surgery, have not been fully reported for these patients. We report the first case of an individual with WS type I, who underwent corpus callosotomy (CC) for drug-resistant epilepsy and obtained good seizure outcomes., Case Description: A boy was diagnosed as having WS type I and developmental delay based on characteristic symptoms and a family history of hearing loss. He underwent cochlear implantation at 18 months of age. At 4 years of age, he developed epileptic seizures with a semiology of drop attack. Electroencephalography (EEG) showed bilateral synchronous high-amplitude spikes and wave bursts, dominant in the right hemisphere. Based on the multimodality examinations, we considered that ictal discharges propagated from the entire right hemisphere to the left, resulting in synchronous discharge and a clinical drop attack; therefore, CC was indicated. At 9 years of age, he underwent a front 2/3
rd CC. At 1 year, the patient became seizure free, and interictal EEG showed less frequent and lower amplitude spike and wave bursts than before., Conclusion: When patients with WS Type I and cognitive impairment show drug-resistant epilepsy, clinicians should consider a presurgical evaluation., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)- Published
- 2021
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40. Diagnostic accuracy for the epileptogenic zone detection in focal epilepsy could be higher in FDG-PET/MRI than in FDG-PET/CT.
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Kikuchi K, Togao O, Yamashita K, Momosaka D, Nakayama T, Kitamura Y, Kikuchi Y, Baba S, Sagiyama K, Ishimatsu K, Kamei R, Mukae N, Iihara K, Suzuki SO, Iwaki T, and Hiwatashi A
- Subjects
- Female, Humans, Magnetic Resonance Imaging, Male, Positron Emission Tomography Computed Tomography, Positron-Emission Tomography, Prospective Studies, Radiopharmaceuticals, Sensitivity and Specificity, Epilepsies, Partial diagnostic imaging, Fluorodeoxyglucose F18
- Abstract
Objectives: To examine the utility of FDG-PET/MRI in patients with epilepsy by comparing the diagnostic accuracy of PET/MRI and PET/CT in epileptogenic zone (EZ) detection., Methods: This prospective study included 31 patients (17 males, 14 females) who underwent surgical resection for EZ. All patients were first scanned using FDG-PET/CT followed immediately with FDG-PET/MRI. Two series of PET plus standalone MR images were interpreted independently by five board-certified radiologists. A 4-point visual score was used to assess image quality. Sensitivities and visual scores from both PETs and standalone MRI were compared using the McNemar test with Bonferroni correction and Dunn's multiple comparisons test., Results: The EZs were confirmed histopathologically via resection as hippocampal sclerosis (n = 11, 35.5%), gliosis (n = 8, 25.8%), focal cortical dysplasia (n = 6, 19.4%), and brain tumours (n = 6, 19.4%) including cavernous haemangioma (n = 3), dysembryoplastic neuroepithelial tumour (n = 1), ganglioglioma (n = 1), and polymorphous low-grade neuroepithelial tumour of the young (n = 1). The sensitivity of FDG-PET/MRI was significantly higher than that of FDG-PET/CT and standalone MRI (FDG-PET/MRI vs. FDG-PET/CT vs. standalone MRI; 77.4-90.3% vs. 58.1-64.5% vs. 45.2-80.6%, p < 0.0001, respectively). The visual scores derived from FDG-PET/MRI were significantly higher than those of FDG-PET/CT, as well as standalone MRI (2.8 ± 1.2 vs. 2.0 ± 1.1 vs. 2.1 ± 1.2, p < 0.0001, respectively). Compared to FDG-PET/CT, FDG-PET/MRI increased the visual score (51.9%, increased visual scores of 2 and 3)., Conclusions: The diagnostic accuracy for the EZ detection in focal epilepsy could be higher in FDG-PET/MRI than in FDG-PET/CT., Key Points: • Sensitivity of FDG-PET/MRI was significantly higher than that of FDG-PET/CT and standalone MRI (FDG-PET/MRI vs. FDG-PET/CT vs. standalone MRI; 77.4-90.3% vs. 58.1-64.5% vs. 45.2-80.6%, p < 0.0001, respectively). • Visual scores derived from FDG-PET/MRI were significantly higher than those of FDG-PET/CT and standalone MRI (2.8 ± 1.2 vs. 2.0 ± 1.1 vs. 2.1 ± 1.2, p < 0.0001, respectively). • Compared to FDG-PET/CT, FDG-PET/MRI increased the visual score (51.9%, increased visual scores of 2 and 3).
- Published
- 2021
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41. Two cases of retained medullary cord running parallel to a terminal lipoma.
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Kurogi A, Murakami N, Morioka T, Mukae N, Shimogawa T, Kudo K, Suzuki SO, and Mizoguchi M
- Abstract
Background: Retained medullary cord (RMC) is a newly defined entity believed to originate from the late arrest of secondary neurulation. Some RMCs contain varying amounts of lipomatous tissues, which need to be differentiated from spinal lipomas, such as filar and caudal lipomas (terminal lipomas)., Case Description: We surgically treated two patients with a nonfunctional cord-like structure (C-LS) that was continuous from the cord and extended to the dural cul-de-sac, and ran parallel to the terminal lipoma. In both cases, untethering surgery was performed by resecting the C-LS with lipoma as a column, under intraoperative neurophysiological monitoring. Histopathological examination confirmed that the central canal-like ependyma-lined lumen with surrounding neuroglial and fibrocollagenous tissues, which is the central histopathological feature of an RMC, was located on the unilateral side of the resected column, while the fibroadipose tissues of the lipoma were located on the contralateral side., Conclusion: Our findings support the idea proposed by Pang et al . that entities such as RMC and terminal lipomas are members of a continuum of regression failure occurring during late secondary neurulation, and the coexistence of RMC and terminal lipoma is not a surprising finding. Therefore, it may be difficult in clinical practice to make a distinct diagnosis between these two entities., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
- Published
- 2021
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42. Periodic discharges with high frequency oscillations recorded from a cerebellar gangliocytoma in an epileptic infant.
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Mukae N, Morioka T, Torio M, Sakai Y, Shimogawa T, Sakata A, Suzuki SO, and Mizoguchi M
- Abstract
Background: Subcortical epilepsies associated with developmental tumors in the cerebellum are rarely experienced. As supportive evidence of the intrinsic epileptogenicity of cerebellar tumors, previous electroencephalogram (EEG) studies with intratumoral depth electrodes demonstrated epileptiform or ictal discharges. Recent studies have demonstrated that high frequency oscillations (HFOs) can be regarded as a new biomarker of epileptogenesis and ictogenesis; however, there are few evidence about HFOs in cases of epilepsy associated with cerebellar tumors., Case Description: A 6-month-old Japanese male infant presented to our hospital with drug resistant epilepsy. We underwent subtotal resection of a cerebellar gangliocytoma and obtained good seizure outcomes. Intraoperative EEG in the tumor depicted HFOs in the form of ripples, riding on periodic discharges., Conclusion: Our findings provide further supportive evidence for the intrinsic epileptogenicity of cerebellar tumors., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)
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- 2021
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43. Nonconvulsive status epilepticus associated with Alzheimer's disease mimicking symptomatic focal epilepsy following the resection of a frontal parasagittal meningioma.
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Abe K, Mukae N, Morioka T, Sangatsuda Y, Sakata A, Suzuki SO, and Mizoguchi M
- Abstract
Background: Epilepsies are frequent in patients with Alzheimer's disease (AD); however, epilepsies in AD can easily go unrecognized because they usually present as focal impaired awareness seizures or nonconvulsive status epilepticus (NCSE) and can overlap with other symptoms of AD., Case Description: We performed an epilepsy surgery in a 69-year-old woman with progressive cognitive impairment and consciousness disorder, who was diagnosed with focal NCSE related to the resected meningioma in the right frontal parasagittal region. Intraoperative electrocorticography revealed localized periodic paroxysmal discharges with beta and gamma activities in the neighboring cortex where the meningioma existed. The histopathological diagnosis of AD was first made from the resected epileptogenic cortex., Conclusion: Even when there is a suspected epileptogenic lesion that could cause focal NCSE, AD should be ruled out in elderly patients with progressive cognitive decline., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Surgical Neurology International.)
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- 2020
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44. A juvenile case of epilepsy-associated, isocitrate dehydrogenase wild-type/histone 3 wild-type diffuse glioma with a rare BRAF A598T mutation.
- Author
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Sadashima S, Suzuki SO, Haruyama H, Mukae N, Fujioka Y, Hata N, Mizoguchi M, Ishimatsu K, Hiwatashi A, and Iwaki T
- Subjects
- Adolescent, Brain Neoplasms complications, Brain Neoplasms pathology, Glioma complications, Glioma pathology, Histones, Humans, Isocitrate Dehydrogenase, Male, Mutation, Brain Neoplasms genetics, Epilepsy, Temporal Lobe complications, Glioma genetics, Proto-Oncogene Proteins B-raf genetics
- Abstract
Here, we report a juvenile (18-year-old male) case of epilepsy-associated, isocitrate dehydrogenase wild-type/histone 3 wild-type diffuse glioma with a rare BRAF mutation and a focal atypical feature resembling diffuse astrocytoma. The patient presented with refractory temporal lobe epilepsy. Subsequently, magnetic resonance imaging revealed a hyperintense lesion in the right temporal lobe on fluid attenuated inversion recovery images. The patient underwent right lateral temporal lobectomy and amygdalohippocampectomy. Histopathologically, the tumor showed isomorphic, diffuse, infiltrative proliferation of glial tumor cells and intense CD34 immunoreactivity. The tumor cells were immunonegative for isocitrate dehydrogenase 1 (IDH1) R132H and BRAF V600E. Notably, the tumor cells showed the lack of nuclear staining for α-thalassemia/mental retardation syndrome, X-linked (ATRX). In addition, the Ki-67 labeling index, using a monoclonal antibody MIB-1, was elevated focally at tumor cells with p53 immunoreactivity. Molecular analyses identified a BRAF
A598T mutation, the first case reported in a glioma. BRAFA598T is predicted to result in loss of kinase action; however, inactive mutants can stimulate mitogen-activated protein kinase kinase (MEK)-extracellular signal-regulated kinase (ERK) signaling through CRAF activation. Thus, according to the recent update of the consortium to inform molecular and practical approaches to central nervous system tumor taxonomy (cIMPACT-NOW update 4), our case is also compatible with diffuse glioma with the mitogen-activated protein kinase (MAPK) pathway alteration. Thorough immunohistochemical and molecular studies are necessary for diagnosis of epilepsy-associated, diffuse gliomas. Partial resemblance in histopathological and molecular genetic features to diffuse astrocytoma also calls for attention., (© 2020 Japanese Society of Neuropathology.)- Published
- 2020
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45. Two Cases of Large Filar Cyst Associated with Terminal Lipoma: Relationship with Retained Medullary Cord.
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Mukae N, Morioka T, Suzuki SO, Murakami N, Shimogawa T, Kanata A, Tsukamoto H, and Mizoguchi M
- Subjects
- Cauda Equina surgery, Cysts complications, Cysts surgery, Female, Humans, Infant, Lipoma complications, Lipoma surgery, Male, Neural Tube Defects complications, Neural Tube Defects surgery, Spinal Cord abnormalities, Spinal Cord diagnostic imaging, Spinal Cord surgery, Spinal Cord Neoplasms complications, Spinal Cord Neoplasms surgery, Cauda Equina diagnostic imaging, Cysts diagnostic imaging, Lipoma diagnostic imaging, Neural Tube Defects diagnostic imaging, Spinal Cord Neoplasms diagnostic imaging
- Abstract
Background: A small, incidental filar cyst associated with terminal lipoma is thought to be caused by failure of secondary neurulation; however, the precise embryologic background is not fully understood. Retained medullary cord (RMC) also originates from late arrest of secondary neurulation. The central feature of RMC histopathology is a central canal-like ependyma-lined lumen with surrounding neuroglial core., Case Description: We surgically treated 2 patients with a large cyst in the rostral part of the filum and lipoma in the caudal filum. At cord untethering surgery, the filum was severed at the caudal part of the cyst. Histopathologically, the filar cyst was the cystic dilatation of the central canal-like structure at the marginal part of the lipoma. The central canal-like structure was continuous caudally in the lipoma, and its size decreased toward the caudal side., Conclusions: The present findings support the idea raised by Pang et al that entities such as filar cyst, terminal lipomas, and RMC can all be considered consequences of a continuum of regression failure occurring during late secondary neurulation., (Copyright © 2020 Elsevier Inc. All rights reserved.)
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- 2020
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46. Comparison of pseudocontinuous arterial spin labeling perfusion MR images and time-of-flight MR angiography in the detection of periictal hyperperfusion.
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Shirozu N, Morioka T, Tokunaga S, Shimogawa T, Inoue D, Arihiro S, Sakata A, Mukae N, Haga S, and Iihara K
- Abstract
Background: Magnetic resonance imaging (MRI), including perfusion MRI with three-dimensional pseudocontinuous arterial spin labeling (ASL) and diffusion-weighted imaging (DWI), are applied in the periictal (including ictal and postictal) detection of circulatory and metabolic consequences associated with epilepsy. Our previous report revealed that periictal hyperperfusion can firstly be detected on ASL, and cortical hyperintensity of cytotoxic edema secondarily obtained on DWI from an epileptically activated cortex. Although magnetic resonance angiography (MRA) using three-dimensional time-of-flight is widely used to evaluate arterial circulation, few MRA studies have investigated the detection of periictal hyperperfusion., Methods: To compare the ability of ASL and MRA to detect the periictal hyperperfusion on visual inspection, we retrospectively selected 23 patients who underwent ASL and MRA examination on both periictal and interictal periods. Patients were divided into the following three groups according to periictal ASL/DWI findings: positive ASL and DWI findings ( n = 13, ASL+/DWI+ group), positive ASL and negative DWI findings ( n = 5, ASL+/DWI- group), and negative ASL and DWI findings (n = 5, ASL-/DWI- group)., Results: Periictal hyperperfusion on MRA was detected in 6 out of 13 patients (46.2%) in the ASL+/DWI+ group, but not in all patients in the ASL+/DWI- and ASL-/DWI- groups. Furthermore, in 5 out of these 6 patients, the diagnosis of periictal MRA hyperperfusion could not be made without referring to interictal MRA and/or periictal ASL findings, because the periictal MRA findings were so minute., Conclusion: The minimum requirement for the development of periictal MRA hyperperfusion is that its epileptic event is intense enough to induce the uncoupling between metabolism and circulation, with the induction of glutamate excitotoxity, and severe cytotoxic edema on DWI. ASL is vastly superior to MRA in the detection of periictal hyperperfusion., Competing Interests: None., (© 2020 The Authors. Published by Elsevier B.V.)
- Published
- 2020
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47. Neurosurgical Pathology and Management of Limited Dorsal Myeloschisis Associated with Congenital Dermal Sinus in Infancy.
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Morioka T, Murakami N, Suzuki SO, Takada A, Tajiri S, Shimogawa T, Mukae N, and Iihara K
- Subjects
- Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Sacrum diagnostic imaging, Sacrum surgery, Thoracic Vertebrae diagnostic imaging, Thoracic Vertebrae surgery, Dermoid Cyst diagnostic imaging, Dermoid Cyst surgery, Neural Tube Defects diagnostic imaging, Neural Tube Defects surgery, Spina Bifida Occulta diagnostic imaging, Spina Bifida Occulta surgery
- Abstract
Background: Because of the shared origin of limited dorsal myeloschisis (LDM) and congenital dermal sinus (CDS), CDS elements may be found within the fibroneural LDM stalk. When part of the CDS invested in the intradural stalk is left during untethering surgery, inclusion tumors such as dermoid cysts may develop. However, the most appropriate surgical strategy for LDM with CDS is still under debate., Methods: Of 19 patients with LDM, 3 (15.8%) had histologically verified CDS elements. We retrospectively analyzed the clinicopathological findings of these patients., Results: In patient 1, the entire stalk including a tiny dermoid cyst at the intradural stalk could be resected through two-level laminectomy during untethering at 6 months of age. In patients 2 and 3, the stalk appeared to be a typical LDM stalk during the initial surgery at 18 and 7 days, respectively; however, CDS was histologically diagnosed in the proximal severed end of the stalk. Postoperative three-dimensional heavily T2-weighted imaging demonstrated spherical enlargement of the remnant stalk, and the entire length of the remnant stalk including newly developed dermoid was resected during the second surgery at 3 years 11 months and 11 months, respectively. Histopathologically, glial fibrillary acidic protein-immunopositive neuroglial tissues and CDS elements were mainly located at the proximal and distal sites of the stalk, respectively, supporting the "dragging down and pulling up" theory. In patients 2 and 3, however, the proximal head of the dermoid cyst passed the distal head of the neuroglial tissues and located at the stalk-cord attachment., Conclusion: Surgeons should be aware of the approximately 10% possibility of the coexistence of CDS when managing infant LDM. However, the recommendation for excision of the entire length of the LDM stalk in all patients should be more carefully made because such a strategy may result in an unnecessary extent of laminotomy/laminectomy for most patients with pure LDM. However, once the postoperative histological examination reveals coexistence of CDS in the resected proximal part of the stalk, the entire length of the remnant stalk should be excised as soon as possible., (© 2020 S. Karger AG, Basel.)
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- 2020
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48. Correction to: Human tail-like cutaneous appendage with a contiguous stalk of limited dorsal myeloschisis.
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Sarukawa M, Morioka T, Murakami N, Shimogawa T, Mukae N, Kuga N, Suzuki SO, and Iihara K
- Abstract
The article was recently published, contained error. Author name "Nobutaka Mukai" should be "Nobutaka Mukae". Given in this article is the correct name.
- Published
- 2019
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49. Human tail-like cutaneous appendage with a contiguous stalk of limited dorsal myeloschisis.
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Sarukawa M, Morioka T, Murakami N, Shimogawa T, Mukae N, Kuga N, Suzuki SO, and Iihara K
- Subjects
- Humans, Infant, Retrospective Studies, Neural Tube Defects pathology, Skin pathology
- Abstract
Purpose: Limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the skin lesion to the underlying spinal cord. On account of the external skin lesion, all LDMs are either flat (nonsaccular) or saccular, and a human tail-like cutaneous appendage has not been reported., Methods: In our 14 LDM patients, 2 had tail-like appendages. We retrospectively analyzed the relationship between the appendage and the LDM tract from the clinicopathological findings of these 2 patients., Results: Preoperative magnetic resonance imaging including three-dimensional heavily T2-weighted images demonstrated an intradural tethering tract, but failed to reveal the precise communication with the appendage. However, surgery revealed the extradural and intradural slender stalk, starting at the base of appendage and running through the myofascial defect. Histological examination demonstrated that there was a tight anatomical relationship between the fibroadipose tissue of the appendage and the fibrocollagenous LDM stalk., Conclusion: When there is potential for an LDM stalk in patients with an appendage, a meticulous exploration of the stalk leading from an appendage is required. Clinicians should be aware of possible morphological variations of skin lesions associated with LDM.
- Published
- 2019
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50. Continuous ictal discharges with high frequency oscillations confined to the non-sclerotic hippocampus in an epileptic patient with radiation-induced cavernoma in the lateral temporal lobe.
- Author
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Mukae N, Morioka T, Torio M, Sakata A, Suzuki SO, and Iihara K
- Abstract
Background: Intraoperative electrocorticography (iECoG) recording is recommended for treating cavernoma related epilepsy. However, "interictal" paroxysmal activities are generally recordable but are not always identical to the epileptogenic zone., Case Description: We surgically treated a 15-year-old girl with drug-resistant epilepsy associated with radiation-induced cavernoma in the right lateral temporal lobe. iECoG revealed paroxysmal activities in the cortex around the cavernoma. Additionally, continuous subclinical "ictal" discharges with high-frequency oscillations (HFO), confined to the histologically non-sclerotic hippocampus, were recorded. Following additional hippocampectomy, a good seizure outcome was obtained., Conclusion: iECoG and HFO analysis revealed high epileptogenicity in the non-sclerotic hippocampus of this patient.
- Published
- 2019
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