88 results on '"Genjiro Hirose"'
Search Results
2. Vestibular symptoms in acute hemispheric strokes
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Genjiro Hirose, Miho Miaki, and Shuichiro Eguchi
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Adult ,Male ,medicine.medical_specialty ,Middle temporal gyrus ,Lateralization of brain function ,Temporal lobe ,Angular gyrus ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Gyrus ,Parietal Lobe ,Internal medicine ,otorhinolaryngologic diseases ,medicine ,Humans ,Prospective Studies ,030212 general & internal medicine ,Aged ,Retrospective Studies ,Aged, 80 and over ,Vestibular system ,business.industry ,Nausea ,Middle Aged ,Vestibular cortex ,Temporal Lobe ,Stroke ,medicine.anatomical_structure ,Vestibular Diseases ,nervous system ,Neurology ,Vertigo ,Cardiology ,Female ,Vestibule, Labyrinth ,Neurology (clinical) ,Nerve Net ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
A prospective study focused on whether vestibular symptoms are seen in acute hemispheric strokes, and if so, the frequency and lateralization of causative lesions on MRI. Among 668 patients with hemispheric infarction, we prospectively included those with chief complaints of acute vestibular symptoms, such as vertigo/dizziness, nausea/vomiting and gait instability, in the "VS" group. We also retrospectively reviewed MRI of all stroke patients, and included cases with the findings of parieto-insular vestibular cortex (PIVC) or temporo-periSylvian vestibular cortex (TPSVC) lesion by diffusion-weighted MRI, in the "PIVC" group. Eight patients were found to belong to the VS group, and six other patients to the PIVC group. In the VS group, six patients had the responsible lesion on the right hemisphere, in the middle cerebral artery (MCA) territory except one case and two on the left MCA territory, particularly in the insula, retro-insular region, superior/middle temporal gyrus, angular gyrus, supra-marginal gyrus, putamen and hippocampus/para-hippocampal gyrus. In contrast, none of the six other patients of the PIVC group had vestibular symptoms. One of them had a lesion in the right hemisphere and five in the left hemisphere. Four lesions were located in the insular area and two within the temporal lobe. In conclusion, cerebral hemispheric infarction limited to the PIVC or TPSVC does not necessarily cause vertigo. However, unilateral hemispheric infarctions, restricted to the areas belonging to the vestibular cortical network may cause vestibular symptoms. The lesions responsible for vestibular symptoms are located more often in the right hemisphere.
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- 2019
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3. Follow-up study of a patient with early onset cerebral amyloid angiopathy following childhood cadaveric dural graft
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Kenji Yoshiki, Kensaku Kasuga, Kazuhiko Kumahashi, Kazunori Ido, Kouichi Misaki, Yukihiko Kohda, Genjiro Hirose, and Akihiro Shioya
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Adult ,Male ,medicine.medical_specialty ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,Cerebrospinal fluid ,Postoperative Complications ,mental disorders ,medicine ,Cadaver ,Humans ,Brain Tissue Transplantation ,Neuroradiology ,Amyloid beta-Peptides ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,medicine.disease ,Magnetic Resonance Imaging ,Surgery ,Transplantation ,Cerebral Amyloid Angiopathy ,Neurology (clinical) ,Neurosurgery ,Cerebral amyloid angiopathy ,Dura Mater ,Cadaveric spasm ,business ,Complication ,030217 neurology & neurosurgery - Abstract
We retrospectively studied the T2 star (T2*)-weighted magnetic resonance imaging (MRI) of a 40-year-old patient diagnosed with symptomatic early-onset cerebral amyloid angiopathy (CAA), occurring 34 years following childhood neurosurgery using a cadaveric dural patch. Our findings revealed that CAA associated with cadaveric dural transplantation could progress rapidly, sometimes with bilateral bleeding. This microbleed evolution is suggestive of water-soluble amyloid-β transmission via cerebrospinal fluid alongside perivascular drainage pathways with deposition in the cerebral artery walls due to clearance disturbances. Multiple intracerebral hemorrhages associated with CAA with a childhood cadaveric dural graft should be considered a life-threatening medical complication.
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- 2021
4. The Barrés test and Mingazzini test —Importance of the original paper by Giovanni Mingazzini―
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Genjiro Hirose
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Leg ,medicine.medical_specialty ,Supine position ,business.industry ,Diagnostic Techniques, Neurological ,Eponym ,History, 19th Century ,History, 20th Century ,Test (assessment) ,Paresis ,Clinical Practice ,Hemiparesis ,Physical medicine and rehabilitation ,Arm ,Humans ,Medicine ,Neurology (clinical) ,Bibliographies as Topic ,medicine.symptom ,business - Abstract
In order to find a subtle hemiparesis of the arms and legs, so called "Barré's test" has been routinely used in clinical practice. This eponym has been questioned by several neurologists. To clarify this, I searched and found the original paper by Giovanni Mingazzini, reported in Revue Neurologique in 1913. He showed arm drift test with his original photo, as asking the patient to stretch his arms in front, hands in the same horizontal plane with the manner of swearing and the fingers spread. The eyes are closed. The examiner observes downward drift of the hand after one half to a minute. He described a similar test for the legs in this article. The patient in supine position raises the legs in a 45 degree angle from the bed. If the leg drops downward too early, an organic hemiparesis could be present. Barré described a new leg drift test in 1919 with a patient lying on the abdomen. He also presented the Mingazzini's arm and leg tests with photos as carried by his patient-models in his article of 1937. He did not quote the original article of Mingazzini as a reference. These brought us incorrect information to consider the presence of Barré's arm test.
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- 2015
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5. [Cerebellar Control of Ocular Movements: Application to the Topographical Diagnosis of Cerebellar Lesions]
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Genjiro, Hirose
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Eye Movements ,Cerebellar Diseases ,Cerebellum ,Humans - Abstract
Over the last decade, substantial information on cerebellar oculomotor control has been provided by the use of sophisticated neuroanatomical, neurophysiological, and imaging techniques. We now know that an intact cerebellum is a prerequisite for normal oculomotor performance. This review clarifies the current knowledge on structure-function correlations of the cerebellum in relation to ocular movements and allows them to be applied to topographical diagnosis of cerebellar lesions. The cerebellar regions most closely related to oculomotor function are: (1) the flocculus/paraflocculus for VOR suppression, cancellation, smooth pursuit eye movement and gaze-holding, (2) the nodulus/ventral uvula for velocity storage and low frequency prolonged vestibular response, and (3) the dorsal oculomotor vermis (declive VI, folium VII) and the posterior portion of the fastigial nucleus (fastigial oculomotor region) for saccades and smooth pursuit initiation. Symptomatically, defects in the flocculus/parflocculus cause saccadic pursuit, downbeat nystagmus, and impairments to visual suppression of the VOR. Lesions of the nodulus/uvula reveal as periodic alternating nystagmus. Lesions of the oculomotor vermis and the fastigial nucleus can induce saccadic dysmetria, while fastigial nucleus lesions may also cause ocular flutter/opsoclonus. A detailed knowledge of cerebellar anatomy and the physiology of eye movements enables localization of lesions to specific areas of the cerebellum.
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- 2016
6. Serious Adverse Effects of Gamma Knife Radiosurgery for Mesial Temporal Lobe Epilepsy
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Hiroaki Onishi, Tetsuro Kawamura, Genjiro Hirose, and Yukihiko Kohda
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Adult ,Male ,Reoperation ,medicine.medical_specialty ,medicine.medical_treatment ,Radiosurgery ,Temporal lobe ,Epilepsy ,Postoperative Complications ,Recurrence ,Image Interpretation, Computer-Assisted ,medicine ,Humans ,Cyst ,Adverse effect ,Aged ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Middle Aged ,Anterior Temporal Lobectomy ,medicine.disease ,Magnetic Resonance Imaging ,Contrast medium ,Hemiparesis ,Epilepsy, Temporal Lobe ,Brain Damage, Chronic ,Female ,Surgery ,Neurology (clinical) ,Radiology ,medicine.symptom ,business ,Follow-Up Studies - Abstract
Gamma knife radiosurgery (GKRS) for mesial temporal lobe epilepsy (MTLE) has been proposed as an alternative to surgical resection. We report serious adverse effects of the treatment after follow-up periods over 9 years in 11 patients treated with GKRS between 1997 and 2000. The target volume of the entorhinoamygdalohippocampectomy area was 4.8-17.1 ml. Marginal dose of 20-25 Gy to the 50% isodose was delivered. One patient was drowned after suffering seizure 7 months after GKRS. Two patients did not show any reduction in seizure frequency over 9 and 18 months. Both patients requested open surgery and became seizure-free postoperatively. Four of the other eight patients were classified as Engel's class I within 4 years after GKRS. One of the four patients experienced symptomatic radiation-induced cerebral edema transiently, one developed radiation necrosis and required surgery 5 years after GKRS, and one developed cognitive impairment with hemiparesis 10 years after GKRS. Magnetic resonance (MR) imaging showed a large cyst in the irradiated temporal lobe. This patient recovered fully after the cyst excision. Only one patient became seizure-free and antiepileptic drug-free without symptomatic radiation-induced complications. However, MR imaging revealed abnormal enhancement, cyst formation, and diffuse white matter change in the irradiated temporal lobe 9 years after GKRS. GKRS for MTLE causes adverse effects of delayed seizure remission and symptomatic radiation-induced complications. Therefore, GKRS cannot be considered as an ideal alternative to surgery for MTLE. Long-term follow-up studies including MR imaging with contrast medium are required for the patients even after successful control of seizures.
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- 2012
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7. Moyamoya Disease Presenting with an Acute Confusional State in an Elderly Patient
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Muichi Kaito, Hisashi Takada, Yuichiro Gondo, Megumi Nakanishi, Makoto Matsui, and Genjiro Hirose
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Male ,medicine.medical_specialty ,Pediatrics ,Hemiplegia ,Anastomosis ,Level of consciousness ,Occlusion ,medicine ,Humans ,Moyamoya disease ,Confusion ,Aged ,medicine.diagnostic_test ,business.industry ,Rehabilitation ,Brain ,Emergency department ,medicine.disease ,Magnetic Resonance Imaging ,Blood Cell Count ,Cerebral Angiography ,Surgery ,Stenosis ,Carotid Arteries ,Neurology (clinical) ,Moyamoya Disease ,Differential diagnosis ,Cardiology and Cardiovascular Medicine ,business ,Blood Chemical Analysis ,Cerebral angiography - Abstract
Moyamoya disease is the angiographic diagnosis of a clinical syndrome showing bilateral stenosis or occlusion of the distal internal carotid arteries and their major branches with extensive parenchymal, leptomeningeal, or transdural anastomoses. The clinical features normally present as reversible ischemic neurologic deficits, sensory-motor attacks with acute hemiplegia, and motor convulsion. An acute confusional state (ACS) among hospitalized patients is a frequent and serious problem. It is characterized by an acute neurologic deficit with a fluctuating course of impaired attention span, unorganized thinking, and altered levels of consciousness. We report a case of 66-year-old woman who presented with an ACS in the emergency department. The subsequent workups including a neuroradiological examination revealed a rare case of moyamoya disease with bifrontal ischemic infarction. The recognition of an ACS as a manifestation of moyamoya disease should therefore be included in the differential diagnosis of elderly patients who present with an acutely altered neuropsychiatric state. A prompt diagnosis may help to select the most appropriate therapy for this rare disorder especially in elderly patients.
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- 2010
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8. Preface
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Genjiro Hirose, MD, Ph. D. and Kunio Tashiro, MD, Ph. D.
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- 2004
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9. [Ring (20) chromosome epileptic syndrome]
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Genjiro, Hirose
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Diagnosis, Differential ,Epilepsy ,Humans ,Electroencephalography ,Ring Chromosomes ,Prognosis - Abstract
Ring (20) chromosome epilepsy syndrome is characterized by highly refractory epilepsy that is often associated with non-pathognomonic, electroencephalographic (EEG) changes. Seizures typically begin during the stage of childhood around the age of 6 years. Nonconvulsive status epilepticus (NCSE) is the most common seizure types and is distinguished by a long-lasting, confusional state that is often associated with EEG patterns in the form of prolonged, high-voltage slow waves with occasional spike/sharp components. Patients with this syndrome suffer from intractable seizures with cognitive decline and frequent epileptic episodes. Accompanying features of this rare disorder, such as superficial minor dysmorphic abnormalities if any, mental retardation and behavioral changes are quite variable. Because of the variability in clinical presentation, in particular the lack of clear dysmorphic features, the clinical diagnosis of this disorder can be delayed before being diagnosed genetically. Most patients with this syndrome have chromosomal changes in the form of a mosaic. High levels of mosaicism correlate well with a lower age of onset and severe cognitive impairment. Here, we emphasize the importance of early G-banding chromosomal analysis when patients present with unexplainable severe seizures and repetitive NCSE, even in the absence of any dysmorphic features suggestive of a chromosomal disorder.
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- 2015
10. Cardiac 123I-MIBG scintigraphy can assess the disease severity and phenotype of PD
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Muichi Kaito, Misuzu Saiki, Ariyuki Hori, Koichiro Sakai, Suzuka Taki, Shinji Saiki, Kotaro Higashi, Miho Miaki, Kazuo Kakeshita, Susumu Fujino, Genjiro Hirose, and Satoshi Kataoka
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Adult ,Gait Ataxia ,Lewy Body Disease ,Male ,medicine.medical_specialty ,Pathology ,Parkinson's disease ,Unified Parkinson's disease rating scale ,Scintigraphy ,Severity of Illness Index ,Gastroenterology ,Statistics, Nonparametric ,Iodine Radioisotopes ,Central nervous system disease ,Atrophy ,Internal medicine ,Tremor ,mental disorders ,Severity of illness ,Humans ,Medicine ,Age of Onset ,Enzyme Inhibitors ,Radionuclide Imaging ,Aged ,Aged, 80 and over ,Neurologic Examination ,Analysis of Variance ,medicine.diagnostic_test ,business.industry ,Dementia with Lewy bodies ,Heart ,Parkinson Disease ,Middle Aged ,Multiple System Atrophy ,medicine.disease ,nervous system diseases ,3-Iodobenzylguanidine ,Phenotype ,Neurology ,Case-Control Studies ,Regression Analysis ,Female ,Neurology (clinical) ,Age of onset ,business - Abstract
Objective : Cardiac 123 I-metaiodobenzylguanidine (MIBG) scintigraphy studies of patients with idiopathic Parkinson's disease (PD) found decreased uptake. Whether this decrease is associated with clinical severity as assessed by the Unified Parkinson's Disease Rating Scale (UPDRS) and the phenotypes of PD has not been determined. Methods : Cardiac MIBG scintigraphy was performed on 34 patients with PD, 7 with multiple system atrophy (MSA), 4 with dementia with Lewy bodies (DLB), and 11 normal controls (NCs). Early and delayed MIBG heart/mediastinum (H/M) ratios were evaluated. PD severity was assessed by the Hoehn and Yahr (H–Y) stage and UPDRS. Patients were grouped in two phenotypes, tremor and postural instability gait difficulty (PIGD)-dominant groups based on UPDRS components. Associations between MIBG uptake and age at onset, UPDRS, and disease phenotype were analyzed in each group. Results : The early H/M ratio was significantly lower in patients with PD (1.45±0.207) than in the NCs (2.08±0.231), and in those with MSA (1.99±0.284), but not in those with DLB (1.29±0.0435). The delayed H/M ratio for PD (1.33±0.276) also was significantly decreased as compared to the ratios for NCs (2.17±0.286) and MSA (2.16±0.414) but not DLB (1.16±0.0949). The early H/M ratio was significantly correlated with both UPDRS score and age at onset, whereas the delayed H/M ratio only was significantly correlated with age at onset. The PIGD-dominant group had significantly higher UPDRS scores and lower H/M ratios than the tremor-dominant group. Conclusion : Cardiac MIBG scintigraphy can be used to differentiate PD from MSA and NC, and to determine the disease severity and phenotypes of PD.
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- 2004
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11. Chorea-acanthocytosis associated with tourettism
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Kohei Shimazaki, Ichiro Matsunari, Ariyuki Hori, Misuzu Saiki, Shinji Saiki, Koichiro Sakai, Kotaro Higashi, Genjiro Hirose, and Satoshi Kataoka
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medicine.medical_specialty ,Pediatrics ,Chorea ,Neurological disorder ,Disease ,Sister ,medicine.disease ,Acanthocytosis ,Central nervous system disease ,Tourettism ,Neurology ,medicine ,Neurology (clinical) ,medicine.symptom ,Psychology ,Psychiatry ,Chorea acanthocytosis - Abstract
We report on a case of Chorea-acanthocytosis (ChAc) in association with Tourettism that consisted of motor and vocal tics, attention deficit-hyperactivity disorder, and obsessive-compulsive disorder in addition to the typical symptoms of ChAc. The subject was compared with his elder sister who had the same disease but milder clinical profile and neuroradiological findings. The [(18)F]-2-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) findings did not explain the differences in symptomatology between the patient and his sister, although they may have correlated with severity.
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- 2004
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12. Interaction of a Paraneoplastic Cerebellar Degeneration-Associated Neuronal Protein with the Nuclear Helix-Loop-Helix Leucine Zipper Protein MRG X
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Koichiro Sakai, Yingyi Li, Tomoyasu Shirakawa, Genjiro Hirose, and Yoko Kitagawa
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Leucine zipper ,Recombinant Fusion Proteins ,Molecular Sequence Data ,Purkinje cell ,Nerve Tissue Proteins ,Biology ,Paraneoplastic Cerebellar Degeneration ,Purkinje Cells ,Cellular and Molecular Neuroscience ,Genes, Reporter ,Sequence Homology, Nucleic Acid ,Two-Hybrid System Techniques ,Tumor Cells, Cultured ,medicine ,Humans ,Genes, Tumor Suppressor ,Molecular Biology ,Peptide sequence ,Cell Nucleus ,Leucine Zippers ,ATF3 ,Basic helix-loop-helix ,Tumor Suppressor Proteins ,Helix-Loop-Helix Motifs ,Cell Biology ,Transfection ,Cell cycle ,Molecular biology ,Recombinant Proteins ,Protein Structure, Tertiary ,Gene Expression Regulation, Neoplastic ,medicine.anatomical_structure ,Cytoplasm ,Mutation ,Carrier Proteins ,Fatty Acid-Binding Protein 7 ,Protein Binding - Abstract
Paraneoplastic cerebellar degeneration-associated antigen PCD17/cdr2 is a neuronal protein expressed predominantly in the cytoplasm of cerebellar Purkinje cells. The biological activities of this protein are not known; however, the presence of a leucine zipper motif in its amino acid sequence suggests that this protein might interact with other proteins harboring a leucine zipper motif. In this study we found by means of a yeast two-hybrid system, ligand overlay assay, and co-immunoprecipitation assay that PCD17 interacts with a nuclear helix-loop-helix leucine zipper protein, MRG X. Overexpression of MRG X in T98G glioblastoma cells by transfection caused abnormal morphological changes in the nucleus and induced cell death. On the other hand, coexpression of PCD17 with MRG X prevented nuclear morphological changes and cell death in T98G cells. MRG X, which is thought to be functionally related to the cell cycle and cell growth, may be regulated by PCD17/cdr2 in Purkinje cells.
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- 2002
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13. Induction of Cytotoxic T Lymphocytes Specific for Paraneoplastic Cerebellar Degeneration-associated Antigen by DNA Immunization
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Yoko Kitagawa, Yiying Li, Genjiro Hirose, Koichiro Sakai, and Tomoyasu Shirakawa
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Cytotoxicity, Immunologic ,Immunology ,Nerve Tissue Proteins ,chemical and pharmacologic phenomena ,Biology ,medicine.disease_cause ,Paraneoplastic Cerebellar Degeneration ,Autoimmunity ,Mice ,Purkinje Cells ,Immune system ,Antigen ,medicine ,Cerebellar Degeneration ,Animals ,Humans ,Immunology and Allergy ,Cytotoxic T cell ,Autoantibodies ,Mice, Inbred BALB C ,DNA ,Paraneoplastic cerebellar degeneration ,medicine.disease ,Recombinant Proteins ,Naked DNA ,biology.protein ,Female ,Immunization ,Antibody ,T-Lymphocytes, Cytotoxic - Abstract
Paraneoplastic cerebellar degeneration associated with gynecological and breast malignancies (PCD) is known to develop autoantibodies and autoreactive cytotoxic T lymphocytes (CTLs) specific for a cytoplasmic protein of Purkinje cells PCD17/cdr2, in the blood of patients. The functional roles of these antibodies and CTLs in the pathogenesis of PCD are unknown. Induction of immune response to this antigen in experimental animals should be useful to clarify the immune mechanisms in PCD patients. We immunized Balb/c mice with naked DNA, which is encoded human PCD17 neural protein in an eukaryotic expression plasmid under control of CMV promoter, and explored whether or noth humoral and cell-mediated immune responses against PCD17 could be induced in vivo . We show that DNA immunization with nake pcd17 cDNA could induce autoantibodies against the cytoplasmic protein of Purkinje cells and CTLs could lyse syngenic myeloma cells pulsed with H-2K-restricted PCD17 peptide. In spite of the generation of anti-Purkinje cell antibodies and PCD17-specific CTLs in vivo , neither clinical nor pathological changes consistent with significant cerebellar degeneration have been detected.
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- 2001
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14. Induction of major histocompatibility complex class I molecules on human neuroblastoma line cells by a flavoid antioxidant
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Yingyi Li, Genjiro Hirose, Koichiro Sakai, Yoko Kitagawa, and Tomoyasu Shirakawa
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Transcription, Genetic ,Flavonoid ,Gene Expression ,E-box ,Biology ,Major histocompatibility complex ,Antioxidants ,Neuroblastoma ,Genes, Reporter ,MHC class I ,Tumor Cells, Cultured ,medicine ,Humans ,Enhancer ,Transcription factor ,Neurons ,chemistry.chemical_classification ,General Neuroscience ,Histocompatibility Antigens Class I ,Flow Cytometry ,medicine.disease ,Molecular biology ,chemistry ,Cell culture ,biology.protein ,Silymarin - Abstract
Human major histocompatibility complex (MHC) class I expression is usually suppressed in neuronal cells and neuroblastoma cells. In the present study, we analyzed the effect of a flavonoid antioxidant, silymarin, on the induction of MHC class I molecules in human neuroblastoma line cells. Treatment of neuroblastoma cells with silymarin resulted in the expression of MHC class I molecules. Silymarin treatment enhanced the transcriptional activity of the reporter construct containing MHC class I promoter truncated within -428 bp of transcription initiation, but not the construct containing the promoter truncated within -284 bp. Because an E-box element is located between -428 and -285 bp of the transcription initiation, results suggest that silymarin acts on the enhancer activity of the E-box in the MHC class I promoter. Our findings indicate that silymarin induces the transcriptional factors to enhance the MHC class I promoter through the class I E-box element.
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- 2001
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15. Amyotrophic Lateral Sclerosis Associated with Sarcoidosis
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Yoko Yamaya, Shinji Saiki, Akira Yoshioka, and Genjiro Hirose
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Pathology ,medicine.medical_specialty ,Sarcoidosis ,business.industry ,Paratracheal lymph nodes ,Amyotrophic Lateral Sclerosis ,Autopsy ,General Medicine ,Middle Aged ,Spinal cord ,medicine.disease ,Fatal Outcome ,medicine.anatomical_structure ,Atrophy ,Internal Medicine ,medicine ,Humans ,Female ,Amyotrophic lateral sclerosis ,medicine.symptom ,Lateral funiculus ,business ,Bulbar palsy - Abstract
We report a rare association of amyotrophic lateral sclerosis (ALS) with incidental pulmonary and muscle sarcoidosis. A 63-year-old woman presented with slowly progressive weakness and atrophy of the extremities starting from the left leg. The biopsy of a small mass in the left gastrocnemius revealed a typical sarcoid nodule. She was treated with corticosteroid for possible sarcoid neuromyopathy. In spite of the treatment, her clinical course was relentlessly progressive and she died of bulbar palsy. Autopsy revealed a loss of motor neurons in the anterior horn, vacuolar degeneration of the lateral funiculus, and noncaseating granulomas in paratracheal lymph nodes and lungs. No granulomatous lesion or cellular infiltration was found in the spinal cord.
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- 2001
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16. Non-N-methyl-d-aspartate glutamate receptors mediate oxygen–glucose deprivation-induced oligodendroglial injury
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Yoko Yamaya, David E Pleasure, Akira Yoshioka, Shinji Saiki, Masumi Kanemoto, Jacqueline S. Beesley, and Genjiro Hirose
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medicine.medical_specialty ,Necrosis ,Glutamic Acid ,Biology ,DNA laddering ,Cell Line ,chemistry.chemical_compound ,Internal medicine ,Lactate dehydrogenase ,medicine ,Animals ,Hypoxia ,Molecular Biology ,Cell damage ,TUNEL assay ,General Neuroscience ,Osmolar Concentration ,Glutamate receptor ,medicine.disease ,Molecular biology ,Culture Media ,Rats ,Oligodendroglia ,Glucose ,Endocrinology ,Receptors, Glutamate ,Terminal deoxynucleotidyl transferase ,chemistry ,Cell culture ,Calcium ,Neurology (clinical) ,medicine.symptom ,Excitatory Amino Acid Antagonists ,Developmental Biology - Abstract
Cells of oligodendroglial lineage are susceptible to oxygen and glucose deprivation. When oligodendrocyte-like cells differentiated from CG-4-immortalized rat O-2A progenitor cells were exposed to hypoxia alone or glucose deprivation alone for 48 h, release of lactate dehydrogenase (LDH) into the culture medium did not increase. However, when cells were deprived of both oxygen and glucose for 6 or 12 h preceding reoxygenation for 2 h, LDH release increased. Adding glucose to the medium protected against cell death and increased lactate production in a concentration-dependent manner. Cell damage induced by deprivation of oxygen and glucose was prevented by calcium-free medium or by non-N-methyl-D-aspartate glutamate receptor (GluR) antagonists, such as 6-cyano-7-nitroquinoxaline-2,3-dione or LY293558, but not by the voltage-dependent calcium channel blocker, nimodipine, or by the N-methyl-D-aspartate GluR antagonist, MK-801. The glutamate concentration in the medium from cells exposed to oxygen-glucose deprivation for 12 h was 49.70+/-3.04 microM/l, which is sufficient to activate GluRs during deprivation of oxygen and glucose. Apoptotic cells detected by terminal deoxynucleotidyl transferase (TdT)-mediated dUTP-biotin nick end-labeling (TUNEL) or Hoechst 33258 staining did not increase in cells exposed to oxygen-glucose deprivation for 12 h and subsequent reoxygenation for 2 h. No DNA laddering was detected by agarose gel electrophoresis from cells exposed to deprivation of oxygen and glucose. Neither acetyl-YVAD-CHO, an inhibitor of caspase-1-like proteases, nor acetyl-DEVD-CHO, an inhibitor of caspase-3-like proteases, prevented oxygen-glucose deprivation-induced injury. Thus, oxygen and glucose deprivation causes calcium-influx-induced necrotic cell damage in cells of oligodendroglial lineage via non-N-methyl-D-aspartate GluR channels.
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- 2000
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17. Analysis of the RNA Recognition Motifs of Human Neuronal ELAV-like Proteins in Binding to a Cytokine mRNA
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Yoko Kitagawa, Genjiro Hirose, and Koichiro Sakai
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Untranslated region ,Molecular Sequence Data ,Biophysics ,ELAV-Like Protein 2 ,Nerve Tissue Proteins ,Phenylalanine ,Peptide ,Biology ,Biochemistry ,Protein Structure, Secondary ,Aspartic acid ,Humans ,Amino Acid Sequence ,RNA, Messenger ,3' Untranslated Regions ,Molecular Biology ,Sequence Deletion ,chemistry.chemical_classification ,Binding Sites ,RNA-Binding Proteins ,RNA ,Cell Biology ,Glutamic acid ,Molecular biology ,Peptide Fragments ,Amino Acid Substitution ,ELAV Proteins ,chemistry ,Interleukin-3 ,Cytokine mrna ,Leucine ,Sequence Alignment - Abstract
Human neuronal Elav-like proteins contain three RNP-type RNA recognition motifs (RRMs). Previous reports demonstrated that a single RRM of the proteins is not sufficient to bind to the uridine-rich stretch in the 3′ untranslated region of mRNAs and that the bi-RRM peptide consisting of the first two RRMs is necessary for the binding. The present study was designed to examine the potential contributions of the first two RRMs when binding to a cytokine mRNA. Deletions of the internal or terminal amino acid residues of the first RRM (RRM1) of the HuC/ple21 ELAV-like protein completely abolished RNA binding. However, removal of any region of the second RRM (RRM2) except for the eight amino acid residues, which correspond to the potent fourth β-sheet structure of RRM2, did not affect RNA binding. Conjugation of the eight amino acid residues to RRM1 enhanced the RNA binding as well as the entire RRM2, indicating that the octapeptide of RRM2 can be compensated for by the binding function of RRM2. The present study also showed that the substitutions of glutamic acid at 42 for aspartic acid and leucine at 44 for phenylalanine in the first potent α-helix structure of RRM1, as were seen in another ELAV-like protein Hel-N1, markedly affected the RNA binding.
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- 1999
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18. Vertigo due to Cerebrovascular Diseases. The Pathophysiology and Imaging Diagnosis
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Genjiro Hirose
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Vestibular system ,medicine.medical_specialty ,biology ,business.industry ,Cerebrovascular disorder ,Ischemia ,medicine.disease ,biology.organism_classification ,Surgery ,Otorhinolaryngology ,Neuroimaging ,Vertigo ,Internal medicine ,otorhinolaryngologic diseases ,Etiology ,Cardiology ,Medicine ,sense organs ,Neurology (clinical) ,Brainstem ,Vertebrobasilar insufficiency ,business - Abstract
Vertigo is a subtype of dizziness, which can result from lesions in diverse locations such as the inner ear, neck paravertebral stretch receptors, visual and vestibular interaction centers in the brainstem and cerebellum, and in the integrative balance center of the thalamus or cortex. This study focused on vertigo due to various cerebrovascular diseases secondary to vertebrobasilar system diseases including extracranial causes as well as intracranial etiologies. The most common cerebrovascular disorder to cause vertigo, oscillating vision and impulsion was vertebrobasilar insufficiency secondary to atherosclerosis of the subclavian, vertebral and basilar arteries. However, it is not always clear which structures exhibit ischemia. When vertigo is associated with other symptoms of the brainstem, we can assume that vertigo is caused by ischemia in the exact site of the brainstem. Therefore, meticulous clinical neurological examinations are mandatory including bedside neuro-otological examinations before obtaining neuroimaging studies are performed. Knowledge of the mechanisms that underlie vestibular disorders is essential to correctly diagnose and effectively treat patients with vertigo secondary to cerbrovascular diseases.
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- 1999
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19. Primary position upbeat nystagmus due to unilateral medial medullary infarction
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Halmagyi Gm, Tomoyasu Shirakawa, Junya Kawada, Tomoko Ogasawara, Akira Yoshioka, Genjiro Hirose, and Satoshi Kataoka
- Subjects
Male ,Infarction ,Nystagmus ,Lesion ,boats ,Nystagmus, Physiologic ,Vestibular nuclei ,boats.ship_class ,medicine ,Humans ,Medulla Oblongata ,Intercalated nucleus ,business.industry ,Cerebral Infarction ,Anatomy ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Electrooculography ,Neurology ,Vertical nystagmus ,Neurology (clinical) ,Upbeat nystagmus ,medicine.symptom ,business ,Perihypoglossal nuclei - Abstract
We report on a patient who developed primary position upbeat nystagmus (ppUBN) due to a unilateral medial medullary infarction. On oculography, the slow phases of the nystagmus sometimes had an exponentially decreasing velocity waveform, indicating that the nystagmus was due to impairment of the vertical position-to-velocity neural integrator. On magnetic resonance imaging, the lesion was caudal to the vestibular nuclei and to the most rostral of the perihypoglossal nuclei, the nucleus intercalatus, a structure that was also involved in a previously reported case of ppUBN due to a unilateral medullary lesion. On the basis of these imaging and oculographic observations, we propose that a unilateral lesion of the nucleus intercalatus is sufficient to cause ppUBN and that the nucleus intercalatus is a part of the vertical position-to-velocity neural integrator in the human ocular-motor system.
- Published
- 1998
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20. Drug induced parkinsonism
- Author
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Genjiro Hirose
- Subjects
Cinnarizine ,medicine.drug_class ,Parkinsonism ,Amantadine ,Atypical antipsychotic ,Pharmacology ,medicine.disease ,Neurology ,Dopamine ,Dopamine receptor D2 ,Anticholinergic ,medicine ,Neurology (clinical) ,Psychology ,Flunarizine ,medicine.drug - Abstract
The use of neuroleptics as psychotherapeutic agents has resulted in extrapyramidal syndromes including parkinsonism. This specific drug-induced parkinsonism (DIP) mimics idiopathic Parkinson’s disease with the typical parkinsonian triad but the symptomatology of this disorder is a rather akinetic rigid syndrome with lesser incidence of resting tremor. This disorder is usually dose-dependent and related to individual susceptibility to neuroleptics. Recent PET techniques with selective radioactive ligands enable us to study extrapyramidal side effects and dopamine D2 receptor occupancy. Now we know that more than 80% D2-receptor occupancy is consistent with the appearance of DIP, whereas a low occupancy between 40–70% induces no DIP. Conventional neuroleptics in regular doses usually cause more than 80% D2 occupancy with resultant parkinsonian symptoms, whereas regular doses of atypical neuroleptics cause only less than 40–70% D2 occupancy without parkinsonism. The other mechanism to explain the lower incidence of DIP in patients with atypical neuroleptics is the “fast-off” hypothesis. D2 receptor occupancy by atypical antipsychotic drugs is rather loose and transient, so they easily dissociate to allow normal dopamine transmission. Newer calcium entry blocking agents such as flunarizine and cinnarizine are known to cause similar DIP. The pathomechanism of this disorder was studied by SPECT and the dose dependent dopamine D2-receptor occupancy by these drugs was noted to explain DIP. The first management for this disorder is to withdraw the offending drugs if possible or switch from conventional neuroleptics to atypical agents. If it is impossible to change the drugs, it is better to add anticholinergics. For the elderly, it is recommended to use amantadine to prevent anticholinergic side effects.
- Published
- 2006
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- View/download PDF
21. Varicose veins associated with CADASIL result from a novel mutation in the Notch3 gene
- Author
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Misuzu Saiki, T. Umemori, Kitagawa Y, K. Murata, Koichiro Sakai, Genjiro Hirose, Shinji Saiki, and M. Matsui
- Subjects
Adult ,Male ,Heterozygote ,Pathology ,medicine.medical_specialty ,DNA Mutational Analysis ,CADASIL ,medicine.disease_cause ,Risk Assessment ,Varicose Veins ,Leukoencephalopathy ,Exon ,Japan ,Risk Factors ,Varicose veins ,Prevalence ,medicine ,Humans ,Genetic Predisposition to Disease ,splice ,Receptor, Notch3 ,Aged, 80 and over ,Mutation ,Receptors, Notch ,business.industry ,Intron ,Middle Aged ,CADASIL Syndrome ,medicine.disease ,Pedigree ,Female ,Neurology (clinical) ,medicine.symptom ,business - Abstract
No genetically diagnosed cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) pedigrees with venous insufficiency have been described. In a CADASIL pedigree with varicose veins, the authors have identified a novel heterozygous mutation in the 3' splice acceptor site of intron 15 of the Notch3 gene. This, based on mRNA analysis, resulted in skipping of exon 16 including eight cysteine residues of EGF-like repeats.
- Published
- 2006
- Full Text
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22. [Current topics of epilepsy]
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Mitsuru, Kawamura and Genjiro, Hirose
- Subjects
Aged, 80 and over ,Automobile Driving ,Epilepsy ,Humans ,Anticonvulsants ,Illusions ,Aged - Published
- 2013
23. [An overview of epilepsy: its history, classification, pathophysiology and management]
- Author
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Genjiro, Hirose
- Subjects
Neurons ,Epilepsy ,Animals ,Humans ,Anticonvulsants ,Brain Waves - Abstract
Epilepsy, a common chronic set of neurological disorders characterized by seizures, affects more than 50 million people worldwide. In fact, it is estimated that the annual incidence of new onset epilepsy in the general population is more than 80 per 100,000, occurring mostly in children and the elderly. Epilepsy is not a single specific disease, or even a single syndrome, but rather a broad category of symptom complexes arising from any number of disordered brain functions. The history of epilepsy dates back to a time when it was associated with religious experiences and even demonic possession; textbooks from the Babylonian Era (718-612 BC) emphasize the supernatural nature of epilepsy, while in ancient Greece, Hippocrates described it as the "Sacred Disease". Our modern understanding of epilepsy as a neurological disorder associated with seizures only originated in the mid-19th century through the research of John Hughlings-Jackson. Classification of epilepsies, epileptic syndromes, and related seizure disorders first appeared 1981 and later in 1989, as described by the International League Against Epilepsy (ILAE). Newer classifications have since been proposed by the same organization; however, these are still rather controversial and have not yet been accepted worldwide. The pathophysiology of epilepsy, including the pharmacological and neurophysiological aspects, has been studied extensively. Epileptogenicity is induced by abnormal cellular excitability that arises from depolarization and hyperpolarization events, as well as from aberrant neuronal networks that develop abnormal synchronization. These events can be studied using mutant epileptogenic animals, such as the GAERS rat model of absence epilepsy. The past 15 years has seen the development of many new drugs for the treatment of epilepsy, thus providing a diverse choice for epileptologists and their patients. However, a better understanding of these drugs is required to improve the therapeutic management of patients suffering with the chronic burden of epilepsy.
- Published
- 2013
24. Altered Serum Lipoproteins and Apolipoproteins in Carotid Atherosclerosis
- Author
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Ariyuki Hori, Tsukasa Saigan, Genjiro Hirose, Satoshi Kataoka, and Hidenobu Michishita
- Subjects
Carotid atherosclerosis ,medicine.medical_specialty ,Apolipoprotein B ,biology ,business.industry ,Neurology ,Internal medicine ,cardiovascular system ,Cardiology ,medicine ,biology.protein ,lipids (amino acids, peptides, and proteins) ,In patient ,Neurology (clinical) ,Cardiology and Cardiovascular Medicine ,business ,Lipoprotein - Abstract
We evaluated the serum levels of lipoproteins and apolipoproteins in patients with angiographically proven extracranial and/or intracranial carotid atherosclerosis (ECA and/or ICA). The prevalence of
- Published
- 1995
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25. A case of cerebral peduncular infarction presenting pure motor hemiparesis
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Genjiro Hirose, Satoshi Kataoka, Hidenobu Michishita, Tsukasa Saigan, and Tomoko Ogasawara
- Subjects
medicine.medical_specialty ,Hemiparesis ,business.industry ,Internal medicine ,medicine ,Cardiology ,Physical therapy ,Infarction ,medicine.symptom ,medicine.disease ,business - Abstract
症例は59歳男性, 高血圧と糖尿病の既往があり, 歩行時の動揺感と左側の筋力低下を生じ来院した.神経症候学的には意識清明, 構音障害は認めず, 片麻痺様歩行を呈した.瞳孔は左右差なく, 眼球運動正常で, 軟口蓋挙上に左右差なかったが, 軽度左中枢性顔面神経麻痺, 左不全片麻痺を認めた.知覚系は正常, 左側深部腱反射は充進し, 左Babinski徴候陽性, 小脳徴候は陰性であった.発症3日後の頭部CT検査では中脳の右大脳脚に低吸収域を認めた.頭部MRI検査ではT2強調画像で右大脳脚の外側2/3に限局した高信号域を認めた.脳血管撮影では両側椎骨動脈及び脳底動脈には異常なく, 右後大脳動脈にも狭窄, 閉塞性病変は認められなかった.本例はpure motor hemiparesisを呈した大脳脚の1acunarinfactionと考えられた.
- Published
- 1995
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26. [Overview: diagnosis of vestibular syndromes]
- Author
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Genjiro Hirose
- Subjects
Vestibular system ,Adult ,medicine.medical_specialty ,genetic structures ,biology ,business.industry ,Nystagmus ,Audiology ,Vestibular Nerve ,Vestibular nerve ,biology.organism_classification ,eye diseases ,Downbeat nystagmus ,Vertigo ,Saccade ,otorhinolaryngologic diseases ,Vestibulocochlear Nerve Diseases ,Medicine ,Vertical nystagmus ,Humans ,sense organs ,Neurology (clinical) ,Upbeat nystagmus ,medicine.symptom ,business - Abstract
Vestibular syndromes are one of the commonest paroxysmal disorders in our clinical practice. These consist of vertigo, oculomotor abnormalities (nystagmus), postural changes and nausea/vomiting. Vertigo can be classified as real vertigo and dizziness, based upon the presence of clinical rotatory perception. In order to diagnose a responsible lesion for various central and peripheral vestibular syndromes, we have to carefully observe nystagmus in patients with acute vertigo. Gaze-evoked nystagmus is the most important nystagmus in patients with the central vestibular syndromes. The finding is easily found at the bed side examination. In order to keep a velocity-position neural signal such as gaze holding, the neural structure to hold and maintain the neural command for a saccade is hypothesized and this has been called as the brainstem neural integrator, which sends tonic-step commands for eccentric gaze. If this fails then the integrator becomes leaky and the eyes drift back to the central position. This movement necessitates corrective saccades, hence gaze-evoked nystagmus will ensue. Vertical nystagmus such as primary position upbeat or downbeat nystagmus is also seen only in the central vestibular syndromes. The detection and diagnosis of these characteristic nystagmus are essential for primary clinicians who care patients with acute vertigo.
- Published
- 2012
27. Analysis of autoantibody binding to 52-kd paraneoplastic cerebellar degeneration-associated antigen expressed in recombinant proteins
- Author
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John E. Greenlee, Genjiro Hirose, Tomoko Ogasawara, Kurt A. Jaeckle, and Koichiro Sakai
- Subjects
Cerebellum ,Leucine zipper ,Paraneoplastic Syndromes ,Immunoblotting ,Molecular Sequence Data ,Enzyme-Linked Immunosorbent Assay ,Biology ,Epitope ,law.invention ,Antigen ,Cerebellar Diseases ,law ,medicine ,Humans ,Amino Acid Sequence ,Antigens ,Autoantibodies ,Autoantibody ,Paraneoplastic cerebellar degeneration ,medicine.disease ,Molecular biology ,Peptide Fragments ,Recombinant Proteins ,Molecular Weight ,medicine.anatomical_structure ,Neurology ,Immunology ,Recombinant DNA ,biology.protein ,Neurology (clinical) ,Antibody - Abstract
A 52-kd neural antigen was reported to be recognized by anti-Purkinje cell antibodies in serum of a patient with paraneoplastic cerebellar degeneration associated with uterine carcinoma. In this study, we demonstrate that this neural antigen is recognized by antibodies known as anti-Purkinje cell antibody type I (PCAb Type I) and anti-YO. The latter's antigen is reported to be specific for the 62- to 64-kd antigen CDR62. Assuming that the 52-kd and 62- to 64-kd antigens share a common epitope(s) recognized by all of these antibodies, we examined the antigenic region on the 52-kd protein by immunoblots with deletion fragment proteins of the recombinant 52-kd protein. A major epitope was localized in the region of amino acid residues 94 to 133 of the 52-kd protein, which is the site of a leucine zipper motif. The potential pathogenicity of PCAb Type I is discussed.
- Published
- 1993
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28. [Anti-neuronal nuclear antibodies, type 1 and type 2]
- Author
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Genjiro, Hirose
- Subjects
Neurons ,Blotting, Western ,RNA-Binding Proteins ,Nerve Tissue Proteins ,Immunohistochemistry ,ELAV Proteins ,Antigens, Neoplasm ,Reference Values ,Antibodies, Antinuclear ,Neuro-Oncological Ventral Antigen ,Animals ,Humans ,Biomarkers ,Paraneoplastic Syndromes, Nervous System - Published
- 2010
29. A neuropathological study of autosomal-dominant chorea-acanthocytosis with a mutation of VPS13A
- Author
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Shinji Saiki, Masahito Yamada, Chiho Ishida, Takao Makifuchi, and Genjiro Hirose
- Subjects
Adult ,Male ,Pathology ,medicine.medical_specialty ,Caudate nucleus ,Vesicular Transport Proteins ,Anterior commissure ,Biology ,Pathology and Forensic Medicine ,Cellular and Molecular Neuroscience ,Atrophy ,Fatal Outcome ,Huntington's disease ,medicine ,Humans ,Gliosis ,Chorea acanthocytosis ,Genes, Dominant ,Neurons ,Putamen ,Neurodegeneration ,Brain ,Anatomy ,medicine.disease ,Immunohistochemistry ,Globus pallidus ,nervous system ,Mutation ,Neurology (clinical) ,Autopsy ,Neuroacanthocytosis ,Enkephalin, Leucine - Abstract
We report the first autopsy case of genetically confirmed, autosomal-dominant chorea-acanthocytosis (AD-ChAc), showing a heterozygous mutation (G–A) at nucleotide position 8,295 in exon 57 of VPS13A. The patient was a 36-year-old Japanese man and the duration of his illness was 11 years. Neuropathologically, the patient showed marked atrophy and neuronal loss, particularly small and medium-sized neurons, with astrocytic gliosis in the caudate nucleus, putamen and globus pallidus. These findings were similar to previous autopsy reports of autosomal-recessive ChAc (AR-ChAc) with mutations of VPS13A. The broad distribution of atrophic neurons and astrocytosis throughout the whole brain was unique in our AD-ChAc patient and has not been described in AR-ChAc. The neuronal density of the dorsal caudate nucleus was lower than that of the ventral side in this patient as well as in three Huntington’s disease (HD) patients. The neuronal densities in both the rostral and caudal sides were lower than that in the middle region at the anterior commissure level, while in the three HD patients, the neuronal densities of the caudate nucleus were more decreased in the caudal side. This ChAc patient showed faint immunoreactivity in the caudate nucleus and globus pallidus with antibodies against the striatal neurotransmitters, methionine–enkephalin, leucine–enkephalin and substance P. The difference in patterns of neuronal vulnerability could reflect those in the mechanisms of neurodegeneration between ChAc and HD.
- Published
- 2008
30. Primary skeletal muscle involvement in chorea-acanthocytosis
- Author
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Shinji Saiki, Muichi Kaito, Yuichiro Gondo, Yoko Kitagawa, Toshihide Kumamoto, Megumi Nakanishi, Nobutaka Hattori, Koichiro Sakai, Ken-Ya Murata, Misuzu Saiki, Genjiro Hirose, and Makoto Matsui
- Subjects
medicine.medical_specialty ,Vesicular Transport Proteins ,Central nervous system disease ,Trinucleotide Repeats ,Chorea ,Internal medicine ,Neuroacanthocytosis ,Medicine ,Humans ,McLeod syndrome ,RNA, Messenger ,Muscle, Skeletal ,Chorea acanthocytosis ,Sarcolemma ,business.industry ,Skeletal muscle ,Spectrin ,DNA ,medicine.disease ,Amyotrophy ,Endocrinology ,medicine.anatomical_structure ,Neurology ,Mutation ,Neurology (clinical) ,medicine.symptom ,business - Abstract
Chorea-acanthocytosis (ChAc) is a hereditary disease characterized by involuntary movements and amyotrophy with elevation of serum creatine kinase. Although skeletal muscle involvement in ChAc has been suggested, the mechanism remains unclear. To investigate chorein abnormalities of the skeletal muscles of ChAc patients with an apparently heterozygous VPS13A mutation compared with those of other hereditary choreic diseases, we performed histological and immunohistochemical studies of the skeletal muscles from 3 ChAc, 1 Huntington's disease (HD), 1 McLeod syndrome (MLS), and 1 normal control (NC) with 2 originally generated anti-chorein antibodies. Chorein immunoreactivities in HD, MLS, and NC were found linearly along the sarcolemma and appeared as speckles in the sarcoplasma, but those in ChAc were uneven and discontinuous along the sarcolemmas and increased in the sarcoplasma especially in type I fibers. This histological observation suggests chorein abnormalities of skeletal muscles might be associated with primary involvement of skeletal muscles in this disorder. © 2007 Movement Disorder Society
- Published
- 2007
31. [Newly published Neurological Clinical Pathological Conference]
- Author
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Genjiro, Hirose
- Subjects
Publishing ,Pathology, Clinical ,Neurology ,Humans ,Congresses as Topic - Published
- 2006
32. [Diagnostic tests: Anti-neuronal nuclear antibodies, type 1 and type 2]
- Author
-
Genjiro, Hirose
- Subjects
ELAV Proteins ,Staining and Labeling ,Antigens, Neoplasm ,Antibodies, Antinuclear ,Neuro-Oncological Ventral Antigen ,Humans ,RNA-Binding Proteins ,Nerve Tissue Proteins ,Blotting, Far-Western ,Immunohistochemistry ,Biomarkers ,Paraneoplastic Syndromes, Nervous System - Published
- 2005
33. [Ictal alteration of 99mTc ECD SPECT imaging in a patient with secondary paroxysmal kinesigenic dyskinesia caused by hyperglycemia]
- Author
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Misuzu, Saiki, Shinji, Saiki, Yuichiro, Gondo, Ken-ya, Murata, Koichiro, Sakai, and Genjiro, Hirose
- Subjects
Male ,Tomography, Emission-Computed, Single-Photon ,Electroencephalography ,Organotechnetium Compounds ,Middle Aged ,Magnetic Resonance Imaging ,Basal Ganglia ,Diabetes Complications ,Chorea ,Cerebrovascular Circulation ,Hyperglycemia ,Humans ,Cysteine ,Radiopharmaceuticals - Abstract
We described a 61-year-old man with diabetes mellitus who presented with hyperglycemia related paroxysmal kinesigenic dyskinesia (PKD) with sudden development of paroxysmal unilateral involuntary movements (IMs) of his neck and the left extremities. Ictal 99mTc-ethylcysteinate dimer SPECT (ECD-SPECT) revealed a hyperperfusion over the contralateral frontal cortex and a hypoperfusion over the contralateral basal ganglia. Immediate correction of hyperglycemia after admission resulted in a marked improvement of IMs and a return to normal cerebral blood flow on interictal ECD-SPECT imaging. These findings suggest that dysfunction of the indirect pathway through the basal ganglia lead to an imbalance of the cortico-striato-thalamo-cortical circuit and may have contributed to the cause of PKD in this case.
- Published
- 2005
34. Induction of humoral responses specific for paraneoplastic cerebellar degeneration-associated antigen by whole recombinant yeast immunization
- Author
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Yoko Kitagawa, Koichiro Sakai, Shinji Saiki, Misuzu Saiki, Megumi Nakanishi, and Genjiro Hirose
- Subjects
Immunogen ,Immunology ,Nerve Tissue Proteins ,Saccharomyces cerevisiae ,Biology ,medicine.disease_cause ,Autoantigens ,Paraneoplastic Cerebellar Degeneration ,Epitope ,law.invention ,Autoimmunity ,Autoimmune Diseases ,Mice ,Purkinje Cells ,Antigen ,law ,otorhinolaryngologic diseases ,medicine ,Immunology and Allergy ,Animals ,Paraneoplastic cerebellar degeneration ,medicine.disease ,Virology ,Disease Models, Animal ,Antigens, Surface ,Recombinant DNA ,biology.protein ,Somatic antigen ,Immunization ,Antibody - Abstract
Paraneoplastic cerebellar degeneration (PCD) is a potent autoimmune disorder in which antigen-driven responses toward the onconeural antigen are assumed to occur in patients. Yeast cell wall has adjuvant capacity and provides immunostimulatory effects of the antigen expressing in viable cells. The recombinant yeast expressing the PCD-associated antigen may become an immunogen for inducing PCD-associated autoimmunity in mice. We attempted to induce autoimmune responses with whole recombinant yeast expressing PCD-associated antigen. SJL/J strain of mouse is found to be a responder to the major epitope on the antigen for anti-Purkinje cell antibodies, and whole recombinant yeast could induce cellular and humoral autoimmune responses in vivo ion SJL/J mice. The immunization technique based on the recombinant yeast expressing a PCD-associated antigen provides a new tool for analyzing the underlying immunological pathomechanisms of PCD.
- Published
- 2004
35. Chorea-acanthocytosis associated with Tourettism
- Author
-
Shinji, Saiki, Genjiro, Hirose, Koichiro, Sakai, Ichiro, Matsunari, Kotaro, Higashi, Misuzu, Saiki, Satoshi, Kataoka, Ariyuki, Hori, and Kohei, Shimazaki
- Subjects
Adult ,Male ,Neuropsychological Tests ,Magnetic Resonance Imaging ,Corpus Striatum ,Speech Disorders ,Attention Deficit Disorder with Hyperactivity ,Chorea ,Fluorodeoxyglucose F18 ,Humans ,Dementia ,Female ,Radiopharmaceuticals ,Tomography, Emission-Computed ,Tourette Syndrome - Abstract
We report on a case of Chorea-acanthocytosis (ChAc) in association with Tourettism that consisted of motor and vocal tics, attention deficit-hyperactivity disorder, and obsessive-compulsive disorder in addition to the typical symptoms of ChAc. The subject was compared with his elder sister who had the same disease but milder clinical profile and neuroradiological findings. The [(18)F]-2-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) findings did not explain the differences in symptomatology between the patient and his sister, although they may have correlated with severity.
- Published
- 2004
36. Valproic acid-induced hearing loss and tinnitus
- Author
-
Genjiro Hirose, Akiko Horiguchi, Ariyuki Hori, Satoshi Kataoka, Koichi Tomoda, Masayuki Takashima, Koichiro Sakai, and Noriko Iwasaki
- Subjects
Adult ,Valproic Acid ,medicine.medical_specialty ,Hearing loss ,business.industry ,General Medicine ,Audiology ,Tinnitus ,Audiometry ,Anesthesia ,Internal Medicine ,medicine ,Humans ,Anticonvulsants ,Female ,medicine.symptom ,business ,Child ,Hearing Loss ,medicine.drug - Published
- 2003
37. Anti-Ma2 antibody related paraneoplastic limbic/brain stem encephalitis associated with breast cancer expressing Ma1, Ma2, and Ma3 mRNAs
- Author
-
Genjiro Hirose, Koichiro Sakai, Kentaro Sahashi, and K Mano
- Subjects
Pathology ,medicine.medical_specialty ,Paraneoplastic Syndromes ,Short Report ,Breast Neoplasms ,Nerve Tissue Proteins ,Breast cancer ,Antigen ,Antigens, Neoplasm ,medicine ,Limbic System ,Humans ,RNA, Messenger ,Antigens ,Aged ,biology ,business.industry ,Atypical Medullary Breast Carcinoma ,Cancer ,Proteins ,medicine.disease ,Psychiatry and Mental health ,Medullary carcinoma ,Carcinoma, Medullary ,Antibody Formation ,biology.protein ,Encephalitis ,Surgery ,Female ,Neurology (clinical) ,Antibody ,Breast carcinoma ,business ,Brain Stem - Abstract
A 69 year old woman presented with cognitive impairment and supranuclear gaze palsy caused by paraneoplastic limbic/brain stem encephalitis associated with atypical medullary breast carcinoma. The cerebrospinal fluid from the patient harboured an anti-neuronal cell antibody against Ma2 antigen, but not against Ma1 or Ma3 antigen. Despite the antibody being restricted to the Ma2 antigen, the patient's cancer tissue expressed Ma1, Ma2, and Ma3 mRNAs. These results, and the expression of Ma2 mRNA in an atypical medullar breast carcinoma in another patient without paraneoplastic encephalitis, indicate that the induction of anti-Ma2 antibody depends on host immunoreponsiveness and not on the presence of the antigen itself in the cancer.
- Published
- 2003
38. Rostral lateral pontine infarction: neurological/topographical correlations
- Author
-
Ariyuki Hori, Shinji Saiki, Miho Miaki, Y. Yamaya, Misuzu Saiki, Genjiro Hirose, and Satoshi Kataoka
- Subjects
Male ,Functional Laterality ,Lesion ,Central nervous system disease ,Disability Evaluation ,Imaging, Three-Dimensional ,Pons ,medicine ,Vertebrobasilar Insufficiency ,Humans ,Stroke ,Paresis ,Aged ,Aged, 80 and over ,Neurologic Examination ,Pontine Base ,Brain Mapping ,Palsy ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Anatomy ,Cerebral Infarction ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Hemiparesis ,Basilar Artery ,Female ,Neurology (clinical) ,medicine.symptom ,business ,Magnetic Resonance Angiography - Abstract
The authors correlated neurologic features of rostral lateral pontine infarct (rLPI) with lesion location on MRI. rLPI is a motor-sensory stroke presenting as crural monoparesis or crural dominant hemiparesis and segmental superficial or deep sensory disturbances. The dorsolateral pontine base causes crural paresis without supranuclear facial palsy.
- Published
- 2003
39. [A questionnaire survey of the neurology training program in Japan]
- Author
-
Genjiro, Hirose
- Subjects
Japan ,Neurology ,Education, Medical, Graduate ,Surveys and Questionnaires - Abstract
A questionnaire survey was performed in order to see the current trends of the neurology training program in Japan. A questionnaire was sent out to 81 neurology program directors of the medical schools and large hospitals. 72 program directors answered the questionnaires. According to the summed results, each program had an average of 37 inpatient beds, 7 teaching staffs with the neurology board certification. The program had an average of 4 residents annually, and they served as junior neurology residents for 1.8 years, and as chief residents for 1 year with 6 months of hospital consultation. 1.4 years training in the internal medicine was prerequisite for the neurology program. The training of clinical neurophysiology was done mainly by the own faculty staff in each program, but the training of neuroradiology and neuropathology varied. A quarter of the training programs had their own teaching staffs of neuroradiology and the rest of three quarters asked for training to the neuroradiology department. 32 of 72 programs had their own teaching neuropathologists and 26 programs asked the training in the pathology department and 14 programs did not have any teaching staffs of neuropathology. It seems that these numerical data are quite similar to those of the American standard of Accreditation Councils. We must still improve the real contents of the neurology training program with more capable teaching staffs.
- Published
- 2003
40. Binding of the ELAV-like protein in murine autoimmune T-cells to the nonameric AU-rich element in the 3' untranslated region of CD154 mRNA
- Author
-
Shinji Saiki, Koichiro Sakai, Genjiro Hirose, Misuzu Saiki, and Yoko Kitagawa
- Subjects
Untranslated region ,DNA, Complementary ,Encephalomyelitis, Autoimmune, Experimental ,Translational efficiency ,Immunology ,CD40 Ligand ,chemical and pharmacologic phenomena ,Regulatory Sequences, Nucleic Acid ,Transfection ,Binding, Competitive ,Autoimmune Diseases ,ELAV-Like Protein 1 ,Mice ,immune system diseases ,T-Lymphocyte Subsets ,Complementary DNA ,Animals ,Luciferase ,Molecular Biology ,3' Untranslated Regions ,AU-rich element ,Messenger RNA ,biology ,Three prime untranslated region ,Tumor Necrosis Factor-alpha ,RNA-Binding Proteins ,Myelin Basic Protein ,Molecular biology ,Peptide Fragments ,Myelin basic protein ,Clone Cells ,Rats ,ELAV Proteins ,Gene Expression Regulation ,Antigens, Surface ,biology.protein - Abstract
The CD154 molecule is important for experimental allergic encephalomyelitis (EAE) which is mediated by autoimmune CD4(+) T-cells. Post-transcriptional instabilization/stabilization of mRNAs, which contain an adenylate uridylate rich element (ARE) in their 3' untranslated region (3'UTR), is regulated in part by binding of ARE-binding proteins to the element. We have investigated the protein which binds to the nonameric ARE in the 3'UTR of CD154 mRNA. A protein which binds to the CD154 ARE was found to exist in a extract prepared from murine autoimmune T-cells activated with myelin basic protein (MBP), and turned out to be mHuR which is a ubiquitous ELAV-like protein. It was found that mHuR was upregulated upon stimulation of the T-cells with a MBP antigen. The CD154 ARE and the ARE in the 3'UTR of tumor necrosis factor-alpha (TNF-alpha) mRNA were competed in binding to mHuR, indicating that both AREs bind to the same site on mHuR. The presence of the CD154 ARE downstream of the luciferase cDNA in a reporter plasmid decreased the translational efficiency, and co-expression of the mHuR slightly increased the translation. These results suggest the possibility that the ELAV-like protein participates in the regulation of the expression of CD154 on the autoimmune T-cells. Modification of the expression of CD154 on autoimmune T-cells by regulating the ELAV-like protein may provide effective therapy for EAE and human multiple sclerosis.
- Published
- 2003
41. Effect of a paraneoplastic cerebellar degeneration-associated neural protein on B-myb promoter activity
- Author
-
Koichiro Sakai, Genjiro Hirose, Misuzu Saiki, Yoko Kitagawa, and Shinji Saiki
- Subjects
animal structures ,Chromosomal Proteins, Non-Histone ,Purkinje cell ,Cell ,Cell Cycle Proteins ,Nerve Tissue Proteins ,Biology ,Paraneoplastic Cerebellar Degeneration ,lcsh:RC321-571 ,Purkinje Cells ,Antigen ,Two-Hybrid System Techniques ,Chlorocebus aethiops ,medicine ,Cerebellar Degeneration ,Animals ,Promoter Regions, Genetic ,lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry ,Psychological repression ,Derepression ,COS cells ,B-myb ,fungi ,Paraneoplastic cerebellar degeneration ,medicine.disease ,Molecular biology ,E2F Transcription Factors ,Neoplasm Proteins ,DNA-Binding Proteins ,medicine.anatomical_structure ,Cerebellar degeneration ,Neurology ,Immunoglobulin G ,COS Cells ,Nerve Degeneration ,biology.protein ,Trans-Activators ,Antibody ,Transcription Factors - Abstract
In this study, we have shown that a paraneoplastic cerebellar degeneration (PCD)-associated antigen, pcd17, binds to a cell cycle-related protein, MRG15. MRG15 derepresses the E2F-responsive B-myb promoter. The pcd17 antigen inhibits the derepression of the B-myb transcriptional activity by MRG15, and, as a result, pcd17 represses the promoter. Delivery of anti-Purkinje cell antibodies (anti-Yo) into the cells inhibits the repression of B-myb promoter activity by pcd17. Because derepression of the B-myb promoter has been implicated in neuronal death, the results suggest the possible role of the antibodies in the pathogenesis of PCD.
- Published
- 2003
42. [A case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI]
- Author
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Natsuko, Yuki, Akira, Yoshioka, Yoko, Yamaya, Misuzu, Saiki, and Genjiro, Hirose
- Subjects
Portal Vein ,Hepatic Encephalopathy ,Humans ,Female ,Middle Aged ,Magnetic Resonance Imaging - Abstract
A 49-year-old woman, without any past history of liver diseases and blood transfusion, was admitted to our service because of somnolence, and flapping tremor. Neurologically, she was drowsy and disoriented. She had bilateral pyramidal tract signs and flapping tremor. Although the laboratory examination showed marked hyperammonemia (217 micrograms/dl), neither abdominal CT nor liver biopsy showed any evidence of liver cirrhosis. An abdominal angiography showed portal vein hypoplasia associated with the portal-systemic shunt. A T2-weighted MRI showed the high intensity areas in the bilateral deep cerebral white matter, and the posterior limbs of the bilateral internal capsules. This is a rare case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI.
- Published
- 2003
43. Type-2 astrocyte-like cells are more resistant than oligodendrocyte-like cells against non-N-methyl-D-aspartate glutamate receptor-mediated excitotoxicity
- Author
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Natsuko Yuki, Akira Yoshioka, Genjiro Hirose, Yoko Yamaya, David E Pleasure, and Shinji Saiki
- Subjects
Blotting, Western ,Neurotoxins ,Excitotoxicity ,Drug Resistance ,Kainate receptor ,medicine.disease_cause ,Cell Line ,Cellular and Molecular Neuroscience ,medicine ,Animals ,RNA, Messenger ,Receptors, AMPA ,Glial fibrillary acidic protein ,biology ,Chemistry ,Stem Cells ,Glutamate receptor ,Cell Differentiation ,Molecular biology ,Immunohistochemistry ,Oligodendrocyte ,Rats ,Oligodendroglia ,medicine.anatomical_structure ,Biochemistry ,Receptors, Glutamate ,Astrocytes ,biology.protein ,NMDA receptor ,Cyclothiazide ,Calcium ,Astrocyte ,medicine.drug - Abstract
Glutamate causes excitotoxicity via non-N-methyl-D-aspartate (NMDA) glutamate receptors (GluR) in oligodendrocytes. Because both oligodendrocytes and type 2 astrocytes are differentiated from oligodendrocyte-type 2 astrocyte (O-2A) progenitor cells, we investigated whether astrocytes are also vulnerable to non-NMDA GluR-mediated excitotoxicity. For these studies, oligodendrocyte-like cells (OLC) and type 2 astrocyte-like cells (2ALC) were derived from CG-4 cells, an immortalized rat O-2A progenitor cell line. About 50% of 2ALC were positive for glial fibrillary acidic protein and 90% were positive for A2B5, verifying that these cells have an type 2 astrocytic phenotype. A 24-hr exposure of OLC to 2 mM kainate, an activator of non-NMDA GluR, caused cell damage as shown by the release of lactate dehydrogenase. The extent of kainate-induced OLC damage was increased by cyclothiazide. In contrast, exposure of 2ALC to 2 mM kainate alone did not induce injury, though mild 2ALC injury was elicited by exposure to 2 mM kainate plus 100 microM cyclothiazide. Furthermore, we found that the kainate induced Ca(2+) uptake by 2ALC was 27.5% of that induced by kainate in OLC. Finally, both OLC and 2ALC expressed non-NMDA GluR subunit mRNAs, including GluR2, GluR3, GluR4, GluR6, GluR7, KA1, and KA2, but quantitative Western blot analysis revealed higher immunodetectable GluR2 and lower immunodetectable GluR3 and GluR4 in 2ALC than in OLC. Together, these results suggest that astrocytes are relatively resistant to non-NMDA GluR-mediated excitotoxicity because they have a higher expression of GluR2 and lower expression of GluR3 and GluR4.
- Published
- 2002
44. A novel murine myotonia congenita without molecular defects in the ClC-1 and the SCN4A
- Author
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Koichiro Sakai, Ariyuki Hori, Tomoyasu Shirakawa, Genjiro Hirose, and Y. Kitagawa
- Subjects
musculoskeletal diseases ,congenital, hereditary, and neonatal diseases and abnormalities ,Myotonia Congenita ,Transgene ,Molecular Sequence Data ,Mutation, Missense ,Mice, Transgenic ,Biology ,Genetic determinism ,Sodium Channels ,Mice ,Chloride Channels ,Genotype ,medicine ,Animals ,Humans ,NAV1.4 Voltage-Gated Sodium Channel ,Muscle, Skeletal ,Gene ,Genetics ,CLCN1 ,Base Sequence ,Myotonia congenita ,Myotonia ,medicine.disease ,Phenotype ,Mice, Inbred C57BL ,Amino Acid Substitution ,biology.protein ,Neurology (clinical) - Abstract
The authors report a new murine model for myotonia congenita designated as B6MT. This line spontaneously arose from breeding of transgenic C57BL/6CrSlc mice, irrelevant of the transgene. The B6MT mouse showed moderate to severe action myotonia, and electromyography revealed myotonic discharge. The phenotype was transmitted with autosomal recessive inheritance. Molecular genetic study of the ClC-1 and the SCN4A genes revealed polymorphism with no functional consequences.
- Published
- 2002
45. Suppression of the transcriptional activity and DNA binding of nuclear factor-kappa B by a paraneoplastic cerebellar degeneration-associated antigen
- Author
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Yoko Kitagawa, Genjiro Hirose, Koichiro Sakai, Yingyi Li, and Tomoyasu Shirakawa
- Subjects
Transcription, Genetic ,Paraneoplastic Syndromes ,Immunology ,Purkinje cell ,Repressor ,Nerve Tissue Proteins ,Antigen ,Transcription (biology) ,Cerebellar Diseases ,medicine ,Tumor Cells, Cultured ,Immunology and Allergy ,Animals ,COS cells ,biology ,Tumor Necrosis Factor-alpha ,NF-kappa B ,DNA ,Paraneoplastic cerebellar degeneration ,medicine.disease ,Molecular biology ,Recombinant Proteins ,Cell biology ,medicine.anatomical_structure ,Neurology ,COS Cells ,Nerve Degeneration ,biology.protein ,Neurology (clinical) ,Antibody ,Nucleus - Abstract
Paraneoplastic cerebellar degeneration (PCD) associated with gynecological malignancies is a disorder in which an autoimmune mechanism has been suggested, and both antibody- and cell-mediated immune responses exist against pcd17/cdr2, a neural protein expressed in cerebellar Purkinje neurons and brainstem neurons. In this report, we describe that pcd17 can suppress the basal or activated NF-kappaB-dependent transcriptional activity in a co-transfection study. The DNA binding of constitutive NF-kappaB complexes decreased in the nucleus of TNF-alpha-stimulated neuroblastoma cells, though pcd17 does not bind to classical NF-kappaB consensus site. These data indicate that pcd17 is a potential repressor for NF-kappaB-dependent gene transcription in neurons.
- Published
- 2001
46. Activation of thrombosis and fibrinolysis following brain infarction
- Author
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Tsukasa Saigan, Genjiro Hirose, Satoshi Kataoka, Ariyuki Hori, and Tomoyasu Shirakawa
- Subjects
Brain Infarction ,Male ,medicine.medical_specialty ,Pathology ,medicine.medical_treatment ,Antithrombin III ,Fibrinogen ,Gastroenterology ,Fibrin ,Central nervous system disease ,Fibrin Fibrinogen Degradation Products ,Risk Factors ,Internal medicine ,Antifibrinolytic agent ,Fibrinolysis ,medicine ,Humans ,Fibrinolysin ,Aged ,Fibrinopeptide A ,alpha-2-Antiplasmin ,biology ,Cerebral infarction ,business.industry ,Antithrombin ,Thrombin ,Thrombosis ,medicine.disease ,Antifibrinolytic Agents ,Neurology ,biology.protein ,Female ,Neurology (clinical) ,business ,medicine.drug - Abstract
To clarify the sequence of alterations in the thrombotic and fibrinolytic systems after acute brain infarction, we prospectively examined sequential changes in coagulatory markers in 38 patients suffering from cardioembolic infarcts (CEI), 41 patients with atherothrombotic infarcts (ATI), 58 patients with lacunar infarcts (LI), and 32 age-matched controls. The plasma level of thrombin–antithrombin III complex (TAT), fibrinopeptide A (FpA), D-dimer, fibrin degradation products-E (FDP-E), fibrinogen, α2-plasmin inhibitor–plasmin complex (PIC), and percent activity of antithrombin III (AT-III) were measured within 48 h, at 1 week, and at 3 weeks after the stroke onset. Significantly elevated levels of TAT and FpA, which are both markers of thrombin formation, were observed in CEI patients, and these elevated levels were associated with increasing D-dimer levels for 3 weeks (P
- Published
- 2000
47. Cyclic GMP/cyclic GMP-dependent protein kinase system prevents excitotoxicity in an immortalized oligodendroglial cell line
- Author
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Masumi Kanemoto, Genjiro Hirose, Shinji Saiki, Akira Yoshioka, Yoko Yamaya, and David E Pleasure
- Subjects
IBMX ,Transcription, Genetic ,Neurotoxins ,Pharmacology ,Biology ,Biochemistry ,Propentofylline ,Cellular and Molecular Neuroscience ,chemistry.chemical_compound ,medicine ,Cyclic GMP-Dependent Protein Kinases ,Excitatory Amino Acid Agonists ,Phosphoprotein Phosphatases ,Animals ,RNA, Messenger ,Phosphorylation ,Protein kinase A ,Cyclic GMP ,Rolipram ,Cell Line, Transformed ,Forskolin ,Kainic Acid ,Activator (genetics) ,Phosphodiesterase ,Dibutyryl Cyclic GMP ,Molecular biology ,Rats ,Isoenzymes ,Oligodendroglia ,chemistry ,cardiovascular system ,Calcium ,medicine.drug - Abstract
Previously, we have demonstrated that excitotoxicity of oligodendrocyte-like cells (OLC), differentiated from immortalized rat O-2A progenitor cells (CG-4 cells), is prevented by cyclic AMP-elevating agents. We now report that some agents that elevate cyclic GMP prevent OLC excitotoxicity. Kainate-induced injury was prevented by cyclic GMP analogues (8-bromo-cyclic GMP and dibutyryl cyclic GMP), a guanylate cyclase activator [atrial natriuretic peptide (ANP)], and phosphodiesterase inhibitors [3-isobutyl-1-methylxanthine (IBMX), ibudilast, propentofylline, and rolipram]. When both forskolin and 8-bromo-cyclic GMP were added, kainate-induced injury was additively prevented. There was a strong positive correlation between suppression of kainate-induced Ca2+ influx and prevention of injury by these chemicals. The measurement of intracellular cyclic AMP and cyclic GMP by radioimmunoassay demonstrated the following: an increase of cyclic GMP with treatment with 8-bromo-cyclic GMP, dibutyryl cyclic GMP, and ANP; an increase of cyclic AMP with treatment with ibudilast and rolipram; and an increase of both cyclic AMP and cyclic GMP with treatment with IBMX and propentofylline. Kainate-induced Ca2+ influx was decreased by 8-(4-chlorophenylthiol)-guanosine-3',5'-monophosphate, an activator of cyclic GMP-dependent protein kinase (PKG), or okadaic acid, an inhibitor of protein phosphatases 1 and 2A. RT-PCR and westem blotting of OLC demonstrated transcription of PKG II gene and translation of PKG Ibeta mRNA, but no translation of PKG Ialpha mRNA. Therefore, we concluded that the cyclic GMP/PKG system prevents OLC excitotoxicity.
- Published
- 2000
48. A case of the cheiro-oral syndrome secondary to the unilateral pontine tegmental hemorrhage
- Author
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Hidenobu Michishita, Sugianto, Genjiro Hirose, Satoshi Kataoka, and Tetsuhiko Arai
- Subjects
Cheiro-oral syndrome ,medicine.medical_specialty ,business.industry ,Medicine ,business ,Dermatology - Abstract
51歳男性, 突然の両側口周囲, 左手掌のしびれ感を主訴として入院.神経学的には両側口周囲, 左手掌, 特に手指先端部に触覚と痛覚の低下が認められた.頭部CTでは, 右橋被蓋部に微小な高吸収域を認め, 核磁気共鳴画像でもT1, T2強調画像で右同部に高信号域を認めた.本症では限局性の右橋被蓋出血により右側の内側毛帯, 右側へ交叉後の三叉神経毛帯腹側路と非交叉性背側三叉神経視床路が障害されて両側口周囲におよぶ手掌, 口症候群が発症したと考えられる.両側口周囲及び対側手掌のcheiro-oral syndromeは一側橋被蓋病変により生じることから脳幹の局在診断に重要な徴候と考えられた.
- Published
- 1991
- Full Text
- View/download PDF
49. Antibody recognition and RNA binding of a neuronal nuclear autoantigen associated with paraneoplastic neurological syndromes and small cell lung carcinoma
- Author
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Genjiro Hirose, Koichiro Sakai, Tomohiro Kumagai, and Yoko Kitagawa
- Subjects
Electrophoresis ,DNA, Complementary ,Lung Neoplasms ,Paraneoplastic Syndromes ,Immunology ,Immunoblotting ,RNA-binding protein ,ELAV-Like Protein 3 ,Enzyme-Linked Immunosorbent Assay ,Nerve Tissue Proteins ,Biology ,Autoantigens ,Binding, Competitive ,Hippocampus ,Epitope ,Proto-Oncogene Proteins c-myc ,Antigen ,Antibody Specificity ,Immunology and Allergy ,Humans ,RNA, Messenger ,Carcinoma, Small Cell ,3' Untranslated Regions ,chemistry.chemical_classification ,Neurons ,RNA recognition motif ,Autoantibody ,RNA ,Nuclear Proteins ,RNA-Binding Proteins ,Antigens, Nuclear ,Molecular biology ,Recombinant Proteins ,Amino acid ,Gene Expression Regulation, Neoplastic ,Neurology ,chemistry ,ELAV Proteins ,biology.protein ,Neurology (clinical) ,Antibody ,Oligonucleotide Probes ,Epitope Mapping - Abstract
The PLE21/HuC neural protein is an autoantigen for anti-neuronal nuclear autoantibodies (ANNA-1/anti-Hu/Type IIa antibodies) from a patient with paraneoplastic limbic encephalomyelitis and small cell lung carcinoma. This antigen belongs to the Hu/ELAV-like protein family, contains three RNA recognition motifs (RRMs) and has RNA binding capacity. In many autoimmune diseases mediated by autoantibodies, antibodies often interfere with the biological functions of their target antigens. To investigate the influences of the autoantibodies on the biological function of the antigen, we mapped the regions which were required for the antibody recognition and for the RNA binding. Deletion analysis of the antigen revealed that the epitopes for the antibodies were localized in the regions of 12 residues, amino acids 161–172, and eight residues, amino acids 29–38, of the first and second RRMs. It was also shown that the eight residues, amino acids 29–38, and the 10 residues, amino acids 187–194, were required for the RNA binding. Although amino acids 29–38 were necessary for both the antibody recognition and the RNA bindings, pre-incubation of the PLE21 antigen with the antibodies did not inhibit the formation of the complex of PLE21, the antibodies and RNA. Thus, the regions required for the antibody recognition are not identical with those for the RNA binding, and it seems unlikely that the autoantibodies interfere with RNA binding of the antigen.
- Published
- 1999
50. Cyclic AMP-elevating agents prevent oligodendroglial excitotoxicity
- Author
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David E Pleasure, Akira Yoshioka, Hiroshi Kitasato, Yuko Shimizu, and Genjiro Hirose
- Subjects
Pyridines ,Excitotoxicity ,Kainate receptor ,AMPA receptor ,Biology ,medicine.disease_cause ,Biochemistry ,Cell Line ,Cellular and Molecular Neuroscience ,chemistry.chemical_compound ,medicine ,Cyclic AMP ,Excitatory Amino Acid Agonists ,Phosphoprotein Phosphatases ,Animals ,Cycloheximide ,Enzyme Inhibitors ,Protein kinase A ,Protein Synthesis Inhibitors ,Forskolin ,Kainic Acid ,Cell Death ,L-Lactate Dehydrogenase ,Colforsin ,Glutamate receptor ,Phosphodiesterase ,Molecular biology ,Cyclic AMP-Dependent Protein Kinases ,Rats ,Oligodendroglia ,medicine.anatomical_structure ,chemistry ,Bucladesine ,Xanthines ,Calcium ,Astrocyte - Abstract
Previously, we have demonstrated that cells of the oligodendroglial lineage express non-NMDA glutamate receptor genes and are damaged by kainate-induced Ca2+ influx via non-NMDA glutamate receptor channels, representing oligodendroglial excitotoxicity. We find in the present study that agents that elevate intracellular cyclic AMP prevent oligodendroglial excitotoxicity. After oligodendrocyte-like cells, differentiated from the CG-4 cell line established from rat oligodendrocyte type-2 astrocyte progenitor cells, were exposed to 2 mM kainate for 24 h, cell death was evaluated by measuring activity of lactate dehydrogenase released into the culture medium. Released lactate dehydrogenase increased about threefold when exposed to 2 mM kainate. Kainate-induced cell death was prevented by one of the following agents: adenylate cyclase activator (forskolin), cyclic AMP analogues (dibutyryl cyclic AMP and 8-bromo-cyclic AMP), and cyclic AMP phosphodiesterase inhibitors (3-isobutyl-1-methylxanthine, pentoxifylline, propentofylline, and ibudilast). Simultaneous addition of both forskolin and phosphodiesterase inhibitors prevented the kainate-induced cell death in an additive manner. A remarkable increase in Ca2+ influx (approximately 5.5-fold) also was induced by kainate. The cyclic AMP-elevating agents caused a partial suppression of the kainate-induced increase in Ca2+ influx, leading to a less prominent response of intracellular Ca2+ concentration to kainate. The suppressing effect of forskolin on the kainate-induced Ca2+ influx was partially reversed by H-89, an inhibitor of cyclic AMP-dependent protein kinase. In contrast to this, okadaic acid, an inhibitor of protein phosphatases 1 and 2A, brought about a decrease in the kainate-induced Ca2+ influx. We therefore concluded that cyclic AMP-elevating agents prevented oligodendroglial excitotoxicity by cyclic AMP-dependent protein kinase-dependent protein phosphorylation, resulting in decreased kainate-induced Ca2+ influx.
- Published
- 1998
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