研究者詳細 - 小野寺　理

論文 - 小野寺　理

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Expanded polyglutamine stretches interact with TAF(II)130, interfering with CREB-dependent transcription 査読

T Shimohata, T Nakajima, M Yamada, C Uchida, O Onodera, S Naruse, T Kimura, R Koide, K Nozaki, Y Sano, H Ishiguro, K Sakoe, T Ooshima, A Sato, T Ikeuchi, M Oyake, T Sato, Y Aoyagi, Hozumi, I, T Nagatsu, Y Takiyama, M Nishizawa, J Goto, Kanazawa, I, Davidson, I, N Tanese, H Takahashi, S Tsuji

NATURE GENETICS 26 ( 1 ) 29 - 36 2000年9月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：NATURE PUBLISHING GROUP

At least eight inherited neurodegenerative diseases are caused by expanded CAG repeats encoding polyglutamine (polyQ) stretches. Although cytotoxicities of expanded polyQ stretches are implicated, the molecular mechanisms of neurodegeneration remain unclear. We found that expanded! polyQ stretches preferentially bind to TAF(II)130, a coactivator involved in cAMP-responsive element binding protein (CREB)-dependent transcriptional activation, and strongly suppress CREB-dependent transcriptional activation. The suppression of CREB-dependent transcription and the cell death induced by polyQ stretches were restored by the co-expression of TAF(II)130. Our results indicate that interference of transcription by the binding of TAF(II)130 with expanded polyQ stretches is involved in the pathogenetic mechanisms underlying neurodegeneration.

DOI： 10.1038/79139

Mutational Analysis of X-Linked Adrenoleukodystrophy Gene 査読

Hiroki Takano, Ryoko Koike, Osamu Onodera, Shoji Tsuji

Cell Biochemistry and Biophysics 32 177 - 185 2000年

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Humana Press

X-linked adrenoleukodystrophy (ALD) is an inherited peroxisomal disorder characterized by progressive neurological dysfunction, occasionally associated with adrenal insufficiency. The clinical phenotypes of ALD are quite variable, and include childhood ALD, adult-onset ALD, adrenomyeloneuropathy, and Addison's disease only. Although the causative gene for ALD has been identified, the physiological role of the gene product remains to be clarified. Despite many mutations having been identified in patients with these clinical phenotypes, the genotype-phenotype correlations have not been clarified. The authors investigated genotype-phenotype correlatons in ALD by analyses on 29 unrelated Japanese patients with ALD and by a review of the literature. All the phenotypes were associated with mutations leading to protein truncation, as well as those resulting in subtle amino acid changes. Furthermore, there were no differences in phenotypic expression among the natures of the subtle amino acid changes. All these data indicate that no obvious correlations exist between the phenotypes of ALD patients and their geneotypes, suggesting that other genetic or environmental factors may also be involved in determining phenotypic expression in ALD.

DOI： 10.1385/CBB:32:1-3:177

Characteristics of an annular vertical diaphragmless shock tube

Hosseini, S.H.R., Onodera, O., Takayama, K.

Shock Waves 10 ( 3 ) 2000年

No mutation in the entire coding region of the alpha-synuclein gene in pathologically confirmed cases of multiple system atrophy 査読

T Ozawa, H Takano, O Onodera, H Kobayashi, T Ikeuchi, R Koide, K Okuizumi, T Shimohata, K Wakabayashi, H Takahashi, S Tsuji

NEUROSCIENCE LETTERS 270 ( 2 ) 110 - 112 1999年7月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：ELSEVIER SCI IRELAND LTD

To determine whether mutations in the coding region of the alpha-synuclein gene are relevant in cases of multiple system atrophy (MSA), detailed nucleotide sequence analysis of the alpha-synuclein gene was performed using total RNA obtained from autopsied brain specimens of 11 pathologically confirmed cases of MSA. The brain specimens used in this study contained both gray and white matter, which were dissected from the frontal, temporal or occipital lobe. No nucleotide alterations were found in the entire coding region of the alpha-synuclein gene in any of the cases. While mutations In the regulatory or intronic regions of the gene were not ruled out, our results suggest that mutations in the coding region of the alpha-synuclein gene are unlikely to contribute to the pathogenesis of MSA. (C) 1999 Elsevier Science ireland ltd. All rights reserved.

DOI： 10.1016/S0304-3940(99)00475-9

Mutational analysis and genotype-phenotype correlation of 29 unrelated Japanese patients with X-linked adrenoleukodystrophy 査読

H Takano, R Koike, O Onodera, R Sasaki, S Tsuji

ARCHIVES OF NEUROLOGY 56 ( 3 ) 295 - 300 1999年3月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：AMER MEDICAL ASSOC

Background: X-linked adrenoleukodystrophy (ALD) is an inherited disease characterized by progressive neurologic dysfunction, occasionally associated with adrenal insufficiency. The classic form of AID usually has onset in childhood (childhood cerebral ALD), with rapid neurologic deterioration leading to a vegetative state. Adult-onset cerebral ALD also presents with rapidly progressive neurologic dysfunction. Milder phenotypes such as adrenomyeloneuropathy and Addison disease only also have been recognized. Despite discovery of the causative gene, a molecular basis for the diverse clinical presentations remains to be elucidated.
Objectives: To conduct mutational analyses in 29 Japanese patients with ALD from 29 unrelated families, to obtain knowledge of the spectrum of mutations in this gene, and to study genotype-phenotype correlations in Japanese patients.
Methods: The 29 patients comprised 13 patients with childhood cerebral ALD, 11 patients with adult-onset cerebral ALD, and 5 patients with adrenomyeloneuropathy. We conducted detailed mutational analyses of 29 unrelated Japanese patients with ALD by genomic Southern blot analysis and direct nucleotide sequence analysis of reverse transcriptase-polymerase chain reaction products derived from total RNA that was extracted from cultured skin fibroblasts, lymphoblastoid cells, or peripheral blood leukocytes.
Results: Three patients with adult-onset cerebral ALD were identified as having large genomic rearrangements. The remaining 26 patients were identified as having 21 independent mutations, including 12 novel mutations resulting in small nucleotide alterations in the ALD gene, Eighteen (69%) of 26 mutations were missense mutations. Most missense mutations involved amino acids conserved in homologous gene products, including PMF70, mALDRP, and Pxa1p. The AG dinucleotide deletion at position 1081-1082, which has been reported previously to be the most common mutation in white patients (12-17%), was also identified as the most common mutation in Japanese patients (12%). All phenotypes were associated with mutations resulting in protein truncation or subtle amino acid changes. There were no differences in phenotypic expressions between missense mutations involving conserved amino acids and those involving nonconserved amino acids.
Conclusion: There are no obvious correlations be tween the phenotypes of patients with ALD and their genotypes, suggesting that other genetic or environmental factors modify the phenotypic expressions of ALD.

Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network 査読

Y Nagai, O Onodera, J Chun, WJ Strittmatter, Burke, JR

EXPERIMENTAL NEUROLOGY 155 ( 2 ) 195 - 203 1999年2月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：ACADEMIC PRESS INC

Eight inherited neurodegenerative diseases are caused by genes with expanded CAG; repeats coding for polyglutamine domains in the disease-producing proteins. The mechanism by which this expanded polyglutamine domain causes neurodegenerative disease is unknown, but nuclear and cytoplasmic polyglutamine protein aggregation is a common feature. In transfected COS7 cells, expanded polyglutamine proteins aggregate and disrupt the vimentin intermediate filament network. Since neurons have an intermediate filament network composed of neurofilament (NF) and NF abnormalities occur in neurodegenerative diseases, we examined whether pathologic-length polyglutamine domain proteins also interact with NF. We expressed varying lengths polyglutamine-green fluorescent protein fusion proteins in a neuroblast cell line, TR1. Pathologic-length polyglutamine-GFP fusion proteins formed large cytoplasmic aggregates surrounded by neurofilament. Immunoisolation of pathologic-length polyglutamine proteins coisolated 68-kDa NF protein demonstrating molecular interaction. These observations suggest that polyglutamine interaction with NF is important in the pathogenesis of the polyglutamine repeat diseases. (C) 1999 Academic Press.

DOI： 10.1006/exnr.1998.6991

Generation of neuronal intranuclear inclusions by polyglutamine-GFP: Analysis of inclusion clearance and toxicity as a function of polyglutamine length 査読

KL Moulder, O Onodera, Burke, JR, WJ Strittmatter, EM Johnson

JOURNAL OF NEUROSCIENCE 19 ( 2 ) 705 - 715 1999年1月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：SOC NEUROSCIENCE

Recent evidence suggests that, in huntingtin and many other proteins, polyglutamine repeats are a toxic stimulus in neurodegenerative diseases. To investigate the mechanism by which these repeats may be toxic, we transfected primary rat cerebellar granule neurons with polyglutamine-green fluorescent protein (GFP) fusion constructs containing 19 (Q19-GFP), 35 (Q35-GFP), 56 (Q56-GFP), or 80 (Q80-GFP) glutamine residues. All constructs, except Q19-GFP, aggregated within the nuclei of transfected cells in a length- and time-dependent manner. Although Q35-GFP expression led to the development of several small aggregates per cell, these aggregates were cleared or degraded, and the cells remained viable. In contrast, Q80-GFP expression resulted in one or two large aggregates and induced cell death. Caspase activation was observed after Q80-GFP aggregation, but inhibition of caspases with Boc-aspartyl-(OMe)-fluoromethylketone (BAF) only served to delay, not prevent, toxicity. In addition, aggregation and toxicity were not affected by other modulators of neuronal cell death such as genetic deletion of the proapoptotic bcl-2 family member bax or addition of the protein synthesis inhibitor cycloheximide. Lastly, nuclear condensation did not occur as part of the toxicity. These data suggest that polyglutamine-GFP expression is toxic to primary neurons but that the death is distinct from classical apoptosis.

Polyglutamine domain proteins with expanded repeats bind neurotilament, altering the neurofilament network 査読

Y Nagai, O Onodera, WJ Strittmatter, Burke, JR

OXIDATIVE/ENERGY METABOLISM IN NEURODEGENERATIVE DISORDERS 893 192 - 202 1999年

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：NEW YORK ACAD SCIENCES

Proteins with expanded polyglutamine (polyQ) repeats cause eight inherited neurodegenerative diseases, Nuclear and cytoplasmic polyQ protein is a common feature of these diseases, but its role in cell death remains debatable. Since the neuronal intermediate filament network is composed of neurofilament (NF) and NF abnormalities occur in neurodegenerative diseases, we examined whether pathologic-length polyQ domain proteins interact with NF. me expressed polyQ-green fluorescent fusion proteins (GFP) in a neuroblast cell line, TR1. Pathologic-length polyQ-GFP fusion proteins form large cytoplasmic aggregates surrounded by neurofilament, Immunoisolation of pathologic-length polyQ proteins co-isolated 68 kD NF protein demonstrating molecular interaction. These observations suggest that polyQ interaction with NF is important in the pathogenesis of the polyglutamine repeat disease.

DOI： 10.1111/j.1749-6632.1999.tb07826.x

Transgenic mice harboring a full-length human mutant DRPLA gene exhibit age-dependent intergenerational and somatic instabilities of CAG repeats comparable with those in DRPLA patients 査読

Toshiya Sato, Mutsuo Oyake, Kenji Nakamura, Kazuki Nakao, Yoshimitsu Fukusima, Osamu Onodera, Shuichi Igarashi, Hiroki Takano, Koki Kikugawa, Yoshinori Ishida, Takayoshi Shimohata, Reiji Koide, Takeshi Ikeuchi, Hajime Tanaka, Naonobu Futamura, Ryusuke Matsumura, Tetsuya Takayanagi, Fumiaki Tanaka, Gen Sobue, Osamu Komure, Mie Takahashi, Akira Sano, Yaeko Ichikawa, Jun Goto, Ichiro Kanazawa, Motoya Katsuki, Shoji Tsuji

Human Molecular Genetics 8 ( 1 ) 99 - 106 1999年

掲載種別：研究論文（学術雑誌）

Dentatorubral-pallidoluysian atrophy (DRPLA) is one among an increasing number of hereditary neurodegenerative diseases determined as being caused by unstable expansion of CAG repeats coding for polyglutamine stretches. To investigate the molecular mechanisms underlying CAG repeat instability, we established three transgenic lines each harboring a single copy of a full-length human mutant DRPLA gene carrying a CAG repeat expansion. These transgenic mice exhibited an age-dependent increase (+0.31 per year) in male transmission and an age-dependent contraction (-1.21 per year) in female transmission. Similar tendencies in intergenerational instabilities were also observed in human DRPLA parent-offspring pairs. The intergenerational instabilities of the CAG repeats may be interpreted as being derived from the instability occurring during continuous cell division of spermatogonia in the male, and that occurring during the period of meiotic arrest in the female. The transgenic mice also exhibited an age-dependent increase in the degree of somatic mosaicism which occurred in a cell lineage-dependent manner, with the size range of CAG repeats being smaller in the cerebellum than in other tissues including the cerebrum, consistent with observations in autopsied tissues of DRPLA patients. Thus, the transgenic mice described in this study exhibited age-dependent intergenerational as well as somatic instabilities of expanded CAG repeats comparable with those observed in human DRPLA patients, and are therefore expected to serve as good models for investigating the molecular mechanisms of instabilities of CAG repeats.

DOI： 10.1093/hmg/8.1.99

Application of shock wave research to geophysics

Takayama, K., Onodera, O., Saito, T.

Journal of Materials Processing Technology 85 ( 1-3 ) 1999年

Evolution of shock waves and the primary vortex loop discharged from a square cross-sectional tube

Jiang, Z., Onodera, O., Takayama, K.

Shock Waves 9 ( 1 ) 1999年

Stability of converging cylindrical shock waves in a vertical annular co-axial diaphragmless shock tube

Hosseini, S.H.R., Onodera, O., Takayama, K.

Transactions of the Japan Society for Aeronautical and Space Sciences 42 ( 135 ) 1999年

Efficacy of early plasmapheresis in Bickerstaff's encephalitis 査読

T Ozawa, O Onodera, T Inuzuka, Y Soma, S Tsuji

INTERNAL MEDICINE 37 ( 11 ) 986 - 989 1998年11月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：JAPAN SOC INTERNAL MEDICINE

Double filtration plasmapheresis (DFPP) was performed in a patient with Bickerstaff's brainstem encephalitis (BBE) in its early phase. He was a 27-year-old male patient suffering from diplopia, facial palsy and drowsiness following upper respiratory tract infection, and had high titers of immunoglobulin G (IgG) antibodies against ganglioside NeuAc alpha 2-8NeuAc alpha 2-3Gal beta 1-3GalNAc beta 1-4(NeuAc alpha 2-8NeuAc alpha 2-3)Gal beta 1-4Glc beta 1-1'Cer (GQ1b) in the serum, DFPP effected immediate improvement of his drowsiness, supporting the diagnosis of BBE, Our observations suggest that DFPP during the early phase of BBE efficiently prevents the progression of consciousness disturbances.

DOI： 10.2169/internalmedicine.37.986

A Japanese family with adrenoleukodystrophy with a codon 291 deletion: A clinical, biochemical, pathological, and genetic report 査読

S Kano, M Watanabe, M Kanai, R Koike, O Onodera, S Tsuji, K Okamoto, M Shoji

JOURNAL OF THE NEUROLOGICAL SCIENCES 158 ( 2 ) 187 - 192 1998年6月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：ELSEVIER SCIENCE BV

We report a Japanese family with adrenoleukodystrophy (ALD) with a three base pair deletion (delGAG291) in the ALD gene. A variety of phenotypes were observed within this family. While the proband (patient 1) was classified as having a rare intermediate type of adult cerebral and cerebello-brain stem forms, his younger brother (patient 2) and nephew (patient 3) had a childhood ALD type. Another nephew (patient 4) of patient 1 was classified as having an adolescent form. The tau level in the cerebrospinal fluid (CSF) in patient 1 was as high as that of patients with Alzheimer's disease (AD). His brain magnetic resonance image (MRI) showed abnormalities in the bilateral cerebellar hemispheres and brain stem, but not in the cerebral white matter, where marked reductions of the cerebral blood flow and oxygen metabolism were clearly demonstrated by positron emission tomography (PET). In patients 2 and 3, the autopsy findings showed massive demyelination of the cerebral white matter with sparing of the U-fibers, compatible with the findings of childhood ALD. Oleic and erucic acids (Lorenzo's Oil) were administered to patients 1 and 4, but sufficient effectiveness was not obtained. The findings in this family suggest that delGAG291 is part of the cause of Japanese ALD with phenotypic variations. Moreover, although the scale of the study is limited, there is a possibility that PET can detect an insidious lesion which is undetectable by computed tomogram (CT) or MRI analysis, and that the higher level of tau reflects the process of neuronal degeneration in ALD. Lorenzo's Oil should be given in the early stage. (C) 1998 Elsevier Science B.V. All rights reserved.

DOI： 10.1016/S0022-510X(98)00120-8

Molecular cloning, structural organization, sequence, chromosomal assignment, and expression of the mouse alpha-N-acetylgalactosaminidase gene 査読

T Herrmann, D Schindler, H Tabe, O Onodera, S Igarashi, A Polack, D Zehnpfennig, S Tsuji

GENE 211 ( 2 ) 205 - 214 1998年5月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：ELSEVIER SCIENCE BV

Alpha-N-acetylgalactosaminidase (2-acetamido-2-deoxy-alpha-D-galactoside acetamidodeoxy-galactohydrolase, NAGA; EC 3.2.1.49) deficiency is a recently recognized autosomal recessive lysosomal disease. As a prerequisite for the generation of an animal model, the mouse NAGA gene was cloned and characterized. The NAGA gene was assigned to mouse chromosome 15 band E3, syntenic to the region encompassing the human gene, and NAGA-deficient mutant human cells transfected with the cosmid clone containing the mouse NAGA gene expressed NAGA activity. Comparison of the mouse NAGA nucleotide sequence with the human NAGA sequence predicted that the mouse NAGA gene contains an open reading frame of 1245 bp, comprising nine coding exons and spanning a genomic region of 8258 bp, and a 3' untranslated region of 0.5 kb. The 5' untranslated region was determined in primer extension studies to be 235 bp in length. Nucleotide identity between the human and mouse NAGA exons ranged from 67.4 to 89.5%, with better matches for exons 1-7 than for 8 and 9. The overall amino acid identity between the mouse and human deduced NAGA polypeptides was 82.0%, between those of mouse and chicken 72.9%. Homology was found to only one other mouse gene, i.e. the a-galactosidase A (GALA; EC 3.2.1.22) gene. The amino acid identity ranged from 51.6 to 62.1% in the polypeptide regions corresponding to NAGA exons 2-7 and GALA exons 1-6, but little, if any, in the remainder. These analyses gave emphasis to the strong conservation of the NAGA gene and its origin from an ancestor common with the GALA gene, with NAGA exons 8 and 9 and GALA exon 7 being the most divergent regions in the evolution of the two genes. (C) 1998 Elsevier Science B.V. All rights reserved.

DOI： 10.1016/S0378-1119(98)00103-6

Apolipoprotein E epsilon 4 allele and progression of cortical Lewy body pathology in Parkinson's disease 査読

K Wakabayashi, A Kakita, S Hayashi, K Okuizumi, O Onodera, H Tanaka, A Ishikawa, S Tsuji, H Takahashi

ACTA NEUROPATHOLOGICA 95 ( 5 ) 450 - 454 1998年5月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：SPRINGER VERLAG

To elucidate whether the apolipoprotein E epsilon 4 allele (APOE4) affects cortical neuropathology in Parkinson's disease (PD), we determined APOE genotypes and quantified the densities of cortical Lewy bodies (LBs), amyloid plaques and neurofibrillary tangles in 22 autopsy-proven PD cases (12 with dementia; 10 without dementia) that were not accompanied by Alzheimer's disease. The APOE4 frequency in the demented patient group was 0.21, which was significantly higher than that in Japanese controls (P < 0.04). LB densities in demented PD patients were significantly higher than those in non-demented PD patients, despite the shorter disease duration in the former. Moreover, plaque density in the temporal cortex and LB density in the cingulate cortex were significantly higher in the group with APOE4 than in that without the allele. There was no difference in tangle density between these two groups. These results suggest that APOE4 may influence the increase in the number of cortical LBs and amyloid plaques in PD. It is possible that when PD occurs in individuals with APOE4 concomitantly evolving cortical LB pathology in a proportion of cases results in limbic (transitional) or neocortical-type LB disease.

A case of chronic enteroviral meningitis and hydrocephalus associated with Bruton type agammaglobulinemia 査読

Tetsutaro Ozawa, Osamu Onodera, Osamu Iizuka, Yoshinori Tanno, Ikuyo Eguchi, Yoshiaki Soma, Shoji Tsuji

Brain and Nerve 50 ( 2 ) 191 - 196 1998年2月

記述言語：日本語掲載種別：研究論文（学術雑誌）

We report a 10-year-old boy with chronic enteroviral meningitis associated with agammaglobulinemia (CEMA) and hydrocephalus. He was treated with a low-dose intravenous administration (100 mg/kg/4 weeks) of gammaglobulin (γ-gl) since he was diagnosed as having Bruton type agammaglobulinemia at 1 year of age. At this admission, neurological examination revealed meningeal signs, Babinski sign, frontal signs, urinary incontinence, and mental retardation (IQ=48) which was considered to be a sequela of the enteroviral encephalitis which had occurred in his first year of life. T1-weighted MR imaging of the brain following gadolinium administration revealed a marked dilatation of the lateral ventricles and dense enhancement of the meninges. Enterovirus was detected in the cerebrospinal fluid (CSF) using tissue culture. Histological examination of a biopsied leptomeningeal specimen revealed inflammatory thickening, which was a likely cause of the obstruction to the flow of CSF. The hydrocephalus in this patient was treated with external drainage of CSF from the lateral ventricle. The CEMA was brought into remission by means of the intraventricular administration of γ-gl, at a dose of 125-250 mg/week (total dose: 1.5 g/8 weeks), in addition to the high dose intravenous administration (400 mg/kg/4 weeks) of γ-gl. Because of the poor prognosis of patients with CEMA, the intraventricular administration of γ-gl should be initiated immediately following a diagnosis of enteroviral meningitis.

Pick's disease: selective occurrence of apolipoprotein E-immunoreactive Pick bodies in the limbic system 査読

S Hayashi, K Wakabayashi, K Iwanaga, A Kakita, K Seki, M Tanaka, K Okuizumi, O Onodera, H Tanaka, S Tsuji, H Takahashi

ACTA NEUROPATHOLOGICA 95 ( 1 ) 1 - 4 1998年1月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：SPRINGER VERLAG

We carried out immunohistochemical examination of apolipoprotein E (apoE) in brains from two patients with Pick's disease. In these cases 1 and 2, the APOE genotypes were epsilon 3/4 and epsilon 3/3, respectively. In both cases, numerous argyrophilic globular intraneuronal inclusions, Pick bodies (PBs), were distributed widely throughout the brain, and immunohistochemically were occasionally positive for apoE. Interestingly, such apoE-immunoreactive PBs were virtually restricted to neurons in the limbic system; in the dentate gyrus, the proportion of apoE-immunoreactive PBs relative to the total number of argyrophilic PBs was 5.0% in case 1 and 2.7% in case 2, whereas in the frontal and temporal neocortices it was less than 0.1% in both cases. Diffuse cytoplasmic immunoreactivity for apoE was found in only a few limbic system neurons without PBs in both cases. In conclusion, it is considered that apoE may not be positively involved in the process of PB formation and that the preferential distribution of apoE-immunoreactive PBs in the limbic system may reflect the presence of certain regional factors associated with the synthesis or metabolism of apoE in this particular system.

DOI： 10.1007/s004010050759

Inhibition of α-ketoglutarate-and pyruvate dehydrogenase complexes in E. coli by a glutathione S-transferase containing a pathological length poly-Q domain: A possible role of energy deficit in neurological diseases associated with poly-Q expansions? 査読

Cooper AJ, Sheu KF, Burke JR, Onodera O, Strittmatter WJ, Roses AD, Blass JP

Age 21 ( 1 ) 25 - 30 1998年1月

Prevertebral abscesses with a protracted insidious clinical course and subsequent lethal, acute pyogenic meningitis and septic shock 査読

Tetsutaro Ozawa, Osamu Onodera, Akiyoshi Kakita, Kenju Aoki, Keiko Tanaka, Yoshiaki Soma, Hitoshi Takahashi, Shoji Tsuji

Brain and Nerve 50 ( 1 ) 75 - 79 1998年1月

記述言語：日本語掲載種別：研究論文（学術雑誌）

This report concerns a 66-year-old man suffering from prevertebral abscesses with a protracted insidious clinical course and subsequent lethal and acute pyogenic meningitis. The patient had a three-month history of mild neck pain, and died as a result of septic shock due to staphylococcus aureus (methicillin-susceptible) infection two days after admission to the hospital. At autopsy, abscesses encapsulated by fibrous connective tissue were found on the ventral surfaces of the cervical and thoracic regions of the spine. The prevertebral abscess on the upper cervical region was organized with dense fibrous tissue and contained a small number of inflammatory cells. On the other hand, the prevertebral abscess on the thoracic region was purulent and contained numerous inflammatory cells, macrophages and gram-positive cocci. Pyogenic spondylitis and discitis accompanying the prevertebral abscesses were multiple and widespread. These features suggested that the abscesses developed initially on the cervical region, extended caudally through the prevertebral space, directly involving the corpus vertebrae and discs, and ultimately caused sepsis. It is important to note that prevertebral abscesses can exhibit a protracted clinical course with only mild symptom such as minor neck pain and then manifest abruptly as acute meningitis and sepsis.

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