Language：Japanese   Publisher：Surgical Neurology International  

Background: Duplicate origin of the middle cerebral artery (MCA) is a rare variation of MCA, often mislabeled as the fenestration of the M1 segment of MCA. Case Description: The authors treated an unruptured aneurysm, 8 mm in diameter, associated with a duplicate origin of MCA in a 42-year-old woman who underwent magnetic resonance imaging for transient vertigo. Clipping surgery was inapplicable due to the lack of space to insert clip blades between the neck and two origins of MCA. Under stent-assisted maneuver, the aneurysm sac was successfully obliterated using three coils, resulting in Raymond-Roy class 1 occlusion status. Digital subtraction angiography performed 3 months after the embolization showed complete obliteration of the aneurysm. So far, only 11 patients with aneurysms associated with duplicate origin of MCA have been reported. We performed a literature review of this very rare combination. The size of aneurysms ranged from 2 to 8 mm, with a mean of 5.2 mm. The neck of the aneurysm is mainly located at the corner between the inferior limb and the internal carotid artery. Ours is the youngest and has the largest aneurysm. Conclusion: Aneurysm can arise from duplicate origin of MCA, for which stent-assisted coiling may be an appropriate treatment modality.

DOI： 10.25259/SNI_219_2024

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PubMed

Language：English   Publisher：日本脳腫瘍病理学会  

Language：English  

DOI： 10.7759/cureus.56228

PubMed

Language：English   Publisher：The Japanese Society for Neuroendovascular Therapy  

<p><b>Objective:</b> Catastrophic antiphospholipid syndrome (CAPS) is a disease characterized by a poor prognosis and a high mortality rate, leading to systemic thrombosis. Approximately two-thirds of CAPS cases are associated with conditions such as infections, malignancies, surgical interventions, and events linked to the disease activity of systemic lupus erythematosus (SLE). Herein, we present a case of CAPS with multiorgan ischemia following ischemic stroke.</p><p><b>Case Presentation:</b> In this case report, a 33-year-old woman with a history of SLE and prolonged steroid use manifested impaired consciousness. Detection of the right internal carotid artery (ICA) occlusion led to successful ICA recanalization through endovascular thrombectomy. Postoperatively, she experienced pulmonary embolism and renal infarction. Although antiphospholipid syndrome (APS) was suspected, APS-related antibodies were negative. Anticoagulation therapy was initiated, presuming corticosteroid-induced thrombosis. However, she developed multiorgan thrombosis, culminating in multiple organ failure. Based on her clinical course, a diagnosis of CAPS was established. Intensive care and plasma exchange therapy were instrumental in her recovery, and she was discharged with a modified Rankin Scale score of 4.</p><p><b>Conclusion:</b> When encountering multiorgan ischemia following ischemic stroke in a young adult patient with an autoimmune disease, the consideration of CAPS as a differential diagnosis is crucial, even if APS-related antibodies test negative.</p>

DOI： 10.5797/jnet.cr.2024-0023

PubMed

Language：Japanese   Publisher：Journal of Neuro-Oncology  

Purpose: To investigate the impact of early and continuous postoperative inpatient rehabilitation during chemoradiotherapy on functional outcomes and overall survival (OS) in patients with glioblastoma (GBM), particularly in different age groups. Methods: This retrospective cohort study at a university hospital (2011–2016) included 75 of 119 consecutive patients newly diagnosed with GBM who underwent standardized treatment and postoperative rehabilitation. Patients were divided into older (≥ 65 years, n = 45) and younger (< 65 years, n = 30) groups, engaging in a 50-day rehabilitation program. We assessed rehabilitation progress, Barthel Index (BI), Brunnstrom Recovery Stage (BRS), adverse events, and OS. BI at discharge and survival were analyzed using multivariate and Cox regression models, respectively. Results: The mean age was 72.5 ± 6.3 and 52.4 ± 7.8 years in the older and younger groups, respectively. Both groups demonstrated significant improvements in BI and BRS. Despite more adverse events in the older group, no significant difference existed in median OS (older group: 18.7 months vs. younger group: 18.3 months, p = 0.87). Early walking training, reduced fatigue during chemoradiotherapy, and high Karnofsky Performance Status at admission significantly impacted the BI at discharge. Cox regression analysis identified the BI at discharge as a significant predictor of survival (hazard ratio [HR] 0.98, 95% confidence interval [CI] 0.97–0.99, p = 0.008). Conclusion: Integrated rehabilitation improves functional outcomes, and enhanced ADL at discharge is associated with improved survival outcomes in patients with GBM, regardless of age. This highlights the need for personalized rehabilitation in treatment protocols. Further prospective studies are warranted to confirm these findings.

DOI： 10.1007/s11060-024-04785-1

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PubMed

Language：English   Publisher：(一社)日本脳神経外科学会  

症例は14歳男児。バスケットボール中に頭部を負傷した後、頭痛、めまい、複視を呈した。CTで左下小脳小帯に小さな血腫を認めた。MRIによる経過観察では、血腫からの軽度の出血と軽度の拡張を繰り返されたが、神経学的障害は認められなかった。しかし、4年後に悪心、嘔吐、左外転麻痺を発症した。MRIにより腫瘤が認められ、mulberry-shaped腫瘍が示唆された。病変は外科的切除された。病変の病理組織学的検査の結果、毛様細胞性星細胞腫が認められた。次世代シーケンス解析の結果、毛様細胞性星細胞腫はARIDIA遺伝子、ATM遺伝子、POLE遺伝子変異を有していたが、BRAJ遺伝子には変異は認められなかった。

Language：Japanese   Publisher：(一社)日本脊髄外科学会  

症例は80歳女性で、急な頭痛と嘔気のため救急搬送された。頭部CTで後頭蓋にくも膜下出血を認め、3D-CTAでは頭蓋内に動脈瘤や動脈解離の所見を認めなかった。脊髄血管造影ではC7椎間孔から脊髄へ向かうradiculomedullary arteryを認め、前脊髄動脈から左側で動静脈シャントを形成し、同部位から逆行性に頭側へ向かうdrainerを認め、feeder上に動脈瘤が形成されていた。左C8椎間孔レベルのradicular arteryは同定できず、頸椎perimedullary動静脈瘻(AVF)もしくはradicular AVFと診断した。day29に直達術で前方アプローチにてシャント離断を行い、術中所見からradicular AVFと診断した。動脈瘤を凝固し、その近位部を離断した時点でシャントが消失したため、シャントポイントには操作を加えずfeeder occlusionの形で硬膜内操作を終えた。術後37日目にリハビリテーション病院へ転院し、その後自宅退院した。術後1年目でシャントや動脈瘤再発を認めていない。

Language：English   Publisher：(一社)日本脳神経外科学会  

症例は94歳女性で、先天性頭蓋顔面異形成であった。1日前から頭痛および嘔吐を呈していた。重度の難聴および視覚障害があり、コミュニケーションは困難であった。CTにて、くも膜下出血、両側頬骨、下顎骨、外耳道の異形成が認められた。CT angiographyにて、頸動脈低形成および頭蓋内動脈に多発性動脈瘤性変化を認めた。前下小脳動脈瘤破裂と診断し、コイル塞栓術を施行した。患者の特異的顔貌はTreacher Collins症候群と一致していた。術前に誤嚥性肺炎を発症しており、術後6日目まで呼吸管理を行った後、抜管した。その後、意識状態の改善は認められず、下顎骨および鼻腔異形成による気道閉塞のため呼吸状態は悪化した。患者の家族は侵襲的蘇生を望まず、術後14日目に死亡した。

Language：English   Publisher：鹿児島大学医学会  

ロゼット形成性グリア神経細胞腫瘍は稀な腫瘍で,緩徐に発育し(WHO grade 1),後頭蓋窩に好発する.我々は,亜全摘出後11年以上再発なく経過する,視床を起源とした稀なロゼット形成性グリア神経細胞腫瘍を経験したため報告する.患者は20歳代前半の女性で,6ヵ月前からの記憶障害,頭痛,霧視を自覚し当科を受診した.Magnetic resonance imaging(MRI)では閉塞性水頭症,18mmの右視床から生じた非嚢胞性第三脳室腫瘍を認めた.病変は,T1強調像で等信号,T2強調像では高信号を呈し,ガドリニウムによる造影効果は認めなかった.右側からのtrans-ventricular subchoroidal approachにより,視床後壁にごく少量の遺残を残して,柔らかい腫瘍を亜全摘出した.組織学的には,周囲に好酸球性神経細胞性コアや小血管コアを伴うロゼットを含めたalveolar component,および毛様細胞性星細胞腫に類似した充実性成分で構成されていた.ロゼットを構成する細胞は,Olig-2,MAP,synaptophysinが陽性で,Ki-67 indexは1%であった.術後,症状は消失した.術後11年3ヵ月後のMRIでは再発なく経過していた.本症例は,ロゼット形成性グリア神経細胞腫瘍の発生部位としては稀な視床から発生し,亜全摘出後11年経過しても再発なく経過している点において,極めて稀な症例である.(著者抄録)

Language：Japanese   Publisher：株式会社医学書院  

DOI： 10.11477/mf.1436204834

PubMed

Language：Japanese   Publisher：(一社)日本小児神経外科学会  

症例は0生日女児.妊娠27週に前頭骨の変形と後頸部腫瘤を指摘された.32週の胎児Magnetic Resonance Imaging(MRI)で後頸部脳瘤を認め,38週に予定帝王切開で出生した.後頸部に長径4cmの腫瘤を認め,MRIで腫瘤内に頭蓋内から逸脱した組織を認めたことから後頭部脳瘤と診断した.1生日に脳瘤修復術,9生日に髄液漏修復術を施行した.さらに術後3ヵ月後に水頭症に対して脳室-腹腔シャント術を施行した.同胞の姉も脳瘤にて手術歴がある.同胞間脳瘤発症例は少なく,多彩な奇形を呈する本例はまれである.今回,同胞間発症と水頭症併発の2点に着目して考察を加える.(著者抄録)

Language：Japanese   Publisher：Journal of Neuro-Oncology  

Purpose: Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare, accounting for less than 1% of all central nervous system tumors. This rarity and novelty complicate the diagnosis and treatments of PPTID. We therefore aimed to evaluate the clinicopathological significance of this tumor. Methods: At 11 institutions participating in the Kyushu Neuro-Oncology Study Group, data for patients diagnosed with PPTID were collected. Central pathology review and KBTBD4 mutation analysis were applied to attain the diagnostically accurate cohort. Results: PPTID was officially diagnosed in 28 patients: 11 (39%) with WHO grade 2 and 17 (61%) with WHO grade 3 tumors. Median age was 49 years, and the male:female ratio was 1:2.1. Surgery was attempted in all 28 patients, and gross total resection (GTR) was achieved in 46% (13/28). Adjuvant radiotherapy and chemotherapy were administered to, respectively, 82% (23/28) and 46% (13/28). The 5-year progression-free survival (PFS) and overall survival rates were 64.9% and 70.4% respectively. Female sex (p = 0.018) and GTR (p < 0.01) were found to be independent prognostic factors for PFS and female sex (p = 0.019) was that for OS. Initial and second recurrences were most often leptomeningeal (67% and 100% respectively). 80% (20/25) of patients harbored a KBTBD4 mutation. Conclusions: Female sex and GTR were independent prognostic factors in our patients with PPTID. Leptomeningeal recurrence was observed to be particularly characteristic of this tumor. The rate of KBTBD4 mutation observed in our cohort was acceptable and this could prove the accuracy of our PPTID cohort.

DOI： 10.1007/s11060-023-04310-w

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PubMed

Language：Japanese   Publisher：JAPAN EPILEPSY SOCIETY  

<p>Corpus callosotomy is considered the most effective procedure for preventing tonic, atonic, and other seizures causing a fall. This study reports a case of a 6-year-old female, diagnosed with trisomy 21 following birth, wherein corpus callosotomy was effective for the treatment of atypical absence status epilepticus. She developed West syndrome at eight months of age. At 5 years, she experienced multiple seizure types, including tonic, myoclonic, and atypical absence seizures. She was hospitalized at 6 years 0 months, because of her inability to walk and difficulty in eating with the increase in atypical absence seizures to more than 100 times a day. The condition was intractable and corpus callosotomy was performed at 6 years and 1 month. The postoperative electroencephalogram revealed generalized spike-wave complexes localized to the right frontal area. Two years have passed since, and her seizures have disappeared. There have been several reports regarding the effects of corpus callosotomy on atypical absence seizures. This case suggests that corpus callosotomy may be a prominent treatment option for intractable atypical absence seizures.</p>

DOI： 10.3805/jjes.40.541

Scopus

Language：Japanese   Publisher：株式会社医学書院  

DOI： 10.11477/mf.1436204720

PubMed

Language：Japanese   Publisher：(一社)日本てんかん学会  

脳梁離断術はてんかん性スパズム、強直発作、脱力発作などの転倒する発作で最も有効とされる。今回、非定型欠神発作重積に脳梁離断術が有効だった1例を経験したので報告する。症例は6歳女児。生後Down症候群と診断された。8ヵ月時にWest症候群を発症した。5歳時から強直発作、ミオクロニー発作、非定型欠神発作などの多彩な発作を認めるようになり、Lennox-Gastaut syndrome spectrumと診断した。6歳0ヵ月時には最大で1日100回以上の非定型欠神発作が出現して、脳波検査では発作時、発作間欠時脳波ともに全般性変化を呈した。難治に経過し、薬剤治療による欠神発作重積の抑制は困難と判断して6歳1ヵ月時に脳梁離断術を行った。手術直後から各種発作は消失した。術後の脳波では全般性棘徐波が右前頭部に局在化した。脳梁を介した神経興奮の拡がりが脳梁離断術によって抑制されたことによって、全般発作である非定型欠神発作が抑えられた可能性を考えた。本症例の経験から、特に欠神発作重積が薬剤抵抗性で退行を呈するなどの緊急性を要する症例では、脳梁離断術の適応を積極的に検討すべきと考えた。(著者抄録)

Language：Japanese   Publisher：The Japanese Society for Pediatric Neurosurgery  

<p>Congenital anomalies such as neural tube defects (NTD), microcephaly, Chiari malformation, premature skull fusion, and syringomyelia are associated with 15-20% of occipital encephaloceles. NTDs are thought to develop as a result of environmental or genetic factors, or a combination of the two, and the onset of encephaloceles in siblings is uncommon. We present a case study of occipital encephalocele with hydrocephalus and syringomyelia in a patient with a sibling-onset encephalocele. A newborn girl with an occipital encephalocele is the patient. Her sister was born with an occipital encephalocele as well. Prenatal ultrasound confirmed both frontal bone depression and posterior cervical mass at 27 weeks pregnant. At 32 weeks pregnant, fetal magnetic resonance imaging revealed a posterior cervical encephalocele. The neural tissue had escaped from the cranium. It also revealed the herniation of the hindbrain. Syringomyelia was discovered between the lower cervical cord and the thoraco-lumbar spinal cord junction. At 38 weeks pregnant, the baby was delivered via scheduled cesarean section. A 4 cm diameter mass was discovered in the midline suboccipital to upper cervical vertebrae. She was diagnosed with syringomyelia and an occipital encephalocele. On the first day of her life, the child underwent encephalocele repair surgery. On the ninth day, however, the patient required another repair surgery for a cerebrospinal fluid leak. Following that, the encephalocele vanished and the syringomyelia improved, but the child required a VP shunt for progressive hydrocephalus at 3 months of age. The mechanisms of progressive hydrocephalus in a child with occipital encephalocele will be discussed. Hyperthermia, genetic background, maternal nutritional deficiency, aflatoxin, and other environmental factors are all risk factors for encephalocele. Concerning genetic factors, the incidence of NTDs among siblings is only about 2-5%, and there are still many unknowns in the encephalocele of genetic factors. Hydrocephalus has been reported in 60-90% of patients with occipital encephalocele, with 63% developing hydrocephalus after surgery. The causes of hydrocephalus include stenosis or torsion of the cerebrospinal fluid tract and venous sinus obstruction. After the encephalocele was repaired, subcutaneous fluid retention, which was thought to be CSF leak, was also observed. In this case, we can say that the changes in cerebrospinal fluid circulation and infection caused the hydrocephalus. In contrast to children with an encephalocele that did not have hydrocephalus or brain tissue, the presence of hydrocephalus resulted in a worse prognosis. We will keep a close eye on her future development.</p>

DOI： 10.34544/jspn.48.3_290

Language：Japanese   Publisher：The Japanese Society of Spinal Surgery  

<p>  We report a case of spinal radicular arteriovenous fistula (SRAVF) at the lower cervical region in an 80-year-old woman who presented with a sudden onset of headache and nausea due to subarachnoid hemorrhage (SAH). Initial computed tomography (CT) showed an SAH mainly in the posterior cranial fossa. 3D-CT angiography (CTA) showed no intracranial aneurysm or dissection. The angiogram showed that the left C7 radiculomedullary artery (RMA) joined the anterior spinal artery (ASA), which supplied a feeding artery toward the AV shunt point at the left C8 level and a draining vein toward the cranial side from the shunt point. The feeding artery had an aneurysm, and there was no left C8 RMA. The preoperative diagnosis was SAH due to spinal perimedullary AVF, prompting a direct anterior approach on day 29. </p><p>  Intraoperative findings showed that the feeding artery from the ASA ran to the left side. An aneurysm was located in front of the spinal cord, and the AV shunt was found on the C8 nerve root, leading to the diagnosis of renal arteriovenous fistula (RAVF). To preserve the function of the left C8 nerve root, we did not cauterize the shunt on the nerve root but performed trapping of the aneurysm as proximal occlusion of the AVF. Intraoperative angiography confirmed the disappearance of the AV shunt. The postoperative course was uneventful; CTA one year after surgery showed no recurrence of the AVF.</p><p>  RAVF of the mid or lower cervical spine is rare, and its standard treatment has not been established. Detailed preoperative examination, including cone-beam CT and selective angiography, is useful for accurate diagnosis and appropriate treatment.</p>

DOI： 10.2531/spinalsurg.37.278

Other Link： https://search.jamas.or.jp/link/ui/2024097559

Language：Japanese  

We reported a case of molecularly defined isocitrate dehydrogenase (IDH)-mutant astrocytoma that recurred twice with aggressive behavior and increased anaplastic morphology. Primary and recurrent tumors were analyzed using custom-made DNA-based cancer gene and RNA-based fusion panels for next-generation sequencing (NGS). NGS analyses revealed that recurrent astrocytoma, in addition to IDH1 and tumor protein 53 mutations detected in the primary lesion, harbored cyclin-dependent kinase inhibitor (CDKN) 2 A/B homozygous deletion and neurotrophic tropomyosin receptor kinase 2 (NTRK2) fusion genes that consisted of golgin A1- and cyclin-dependent kinase 5 regulatory subunit associated protein 2-NTRK2 fusions. Anaplasia and necrosis were observed in the recurrent tumors, but not in the primary lesion. Therefore, the integrative diagnosis was primary IDH-mutant astrocytoma grade 2 and recurrent IDH-mutant astrocytoma grade 4 with NTRK2 fusions. This is a worthwhile report describing a case of IDH-mutant astrocytoma that showed genomic evolution during tumor recurrence. Our report suggests that NTRK fusion and CDKN2A/B homozygous deletion promote high-grade transformation and indicate an unfavorable prognosis of IDH-mutant astrocytoma.

DOI： 10.1016/j.prp.2022.154163

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PubMed

Language：English   Publisher：(一社)日本脳神経外科学会  

日本人患者のびまん性神経膠腫にみられる、分子遺伝学的プロファイルについて調査した。著者等の大学で収集した、びまん性神経膠腫患者303例の腫瘍組織試料を用いて、神経膠腫に特化した遺伝子パネルから、分子遺伝学的プロファイルを解析した。その結果、膠芽腫(GBM)患者185例に最も変異が多く認められた遺伝子は、TERTp遺伝子、TP53遺伝子、PTEN遺伝子、NF1遺伝子とPDGFRA遺伝子であった。また、欠失が最も多く認められた遺伝子は、PTEN遺伝子およびCDKN2A/B及びRB1遺伝子で、増加/増幅が最も多く認められた遺伝子は、EGFR遺伝子とPDGFRA遺伝子およびCDK4遺伝子であった。さらに、グレードIII乏突起膠腫22例のうち3例には、CDKN2A/B遺伝子のホモ接合性欠失が、4例にはARID1A遺伝子変異が検出され、ARID1A遺伝子変異が無増悪生存転帰不良と関連することが示唆された。なお、WHO脳腫瘍分類2021により、グレードII/IIIでIDH野生型星状細胞腫の62.5%が、IDH野生型のGBMに再分類された。本報により、神経膠腫に特化した遺伝子パネルが、WHO脳腫瘍分類2021に準じた分子診断に適用可能であることが確認された。

Language：Japanese   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.98.s.hpt_63

Language：Japanese   Publisher：(一社)日本内分泌学会  

50歳代男性。頭痛の精査目的に施行された頭部MRIで鞍上部腫瘍を認められ、当科に紹介された。MRI所見は鞍上部に最大径17mmの腫瘍性病変を認め、腫瘍は視路に接していた。内分泌学的には明らかな異常は認めず、視野検査でも明らかな障害は認めなかった。無症候性ではあるものの、腫瘍が視路に接しており将来的には症候性となることが予想されたことなどから早期の手術介入の方針とし、開頭経鼻同時手術を施行した。腫瘍は赤色調で周囲脳や硬膜との癒着は目立たなかったが、右の視神経との癒着が強固であった。術中所見から右海綿静脈洞内への腫瘍進展が疑われたため、同部位と視神経周囲は愛護的に操作して腫瘍を摘出し、病理組織所見から傍神経節腫と診断した。術後に全身検索を行い、中枢神経単発の非機能性傍神経節腫と最終診断した。右海綿静脈洞周囲の残存腫瘍が緩徐に増大し、摘出術から17ヵ月後にガンマナイフ照射を行った。

Language：Japanese   Publishing type：Research paper (scientific journal)  

PURPOSE: Delayed postoperative hyponatremia (DPH) is a unique complication of transsphenoidal surgery (TSS) in pituitary tumors. Growth hormone (GH) enhances renal sodium reabsorption; however, the association between postoperative GH reduction and DPH in acromegaly is unclear. This study was performed to clarify the incidence of and the predictive factors for DPH in patients with acromegaly who underwent TSS. METHODS: Ninety-four patients with active acromegaly were examined retrospectively. During the postoperative course, patients with serum sodium levels ≤ 134 mEq/L were classified into the DPH group. We compared basic clinical characteristics, tumor characteristics, and preoperative and postoperative examination findings between the DPH and non-DPH groups. RESULTS: DPH occurred in 39 patients (41.5%), and the lowest serum sodium levels were generally observed during postoperative days (PODs) 7-9. They needed a 3-day longer hospital stay than those without DPH. The DPH group had lower preoperative body weight and body mass index. In addition, a transient increase in body weight during PODs 5-7 occurred with a transient decrease in urinary volume in the DPH group. Preoperative and postoperative GH and insulin-like growth factor-1 levels did not differ between the two groups. CONCLUSION: The findings suggested that lower preoperative weight and a postoperative transient gain in body weight are associated with an increased risk of DPH in acromegaly patients undergoing transsphenoidal surgery.

DOI： 10.1007/s11102-022-01288-y

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)  

Background: Cerebrospinal fluid (CSF) leakage is a common complication of ventriculoperitoneal shunt (VPS) and has the potential to induce shunt infection. Especially in infants and children, these are serious complications. DuraGen is a collagen matrix dural substitute used to reduce the risk of CSF leakage in various neurosurgeries. We report our VPS procedure with DuraGen for preventing postoperative CSF leakage in patients aged <1 year. Methods: We used DuraGen to prevent postoperative CSF leakage in six VPS surgeries. Antibiotic-impregnated shunt catheters and programmable valves with anti-siphon devices were also used in all cases. DuraGen was placed inside and atop the burr hole. All cases had an initial shunt pressure of 5 cmH2O. Fibrin glue was not used. Results: The patients underwent follow-up for a year after VPS surgery. There was no postoperative subcutaneous CSF collection or leakage after all six VPS surgeries. Furthermore, no postoperative shunt infections or DuraGen-induced adverse events were noted. Conclusion: We speculate that DuraGen has a preventive effect on postoperative CSF leakage in VPS cases aged <1 year.

DOI： 10.25259/SNI_629_2022

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PubMed

Language：Japanese  

BACKGROUND: Solitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are rare mesenchymal tumors of nonmeningothelial origin that comprises <1% of all central nervous system tumors. CASE DESCRIPTION: A 45-year-old male presented with sleep apnea (apnea-hypopnea index was 17.1 events/hour) and dysesthesias of the right upper and lower extremities. The magnetic resonance demonstrated a heterogeneous intradural extra-axial C1 mass with syringobulbia and syringomyelia. The right vertebral angiography revealed a hypervascular mass (i.e., intense tumor staining). With the preoperative diagnosis of a spinal hemangioblastoma, the patient underwent tumor removal. However, intraoperative findings demonstrated that the ventral component of the tumor was intramedullary without a dural attachment. Further, the histological diagnosis was consistent with SFT/HPC (HPC phenotype). The postoperative course was uneventful, and the patient's symptoms and the syrinxes spontaneously regressed. CONCLUSION: A 45-year-old male presented a rare spinal intradural lesion at C1 appeared to be a spinal hemangioblastoma, but proved to be SFT/HPC (HPC phenotype) with intramedullary invasion.

DOI： 10.25259/SNI_655_2022

Scopus

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

Objective: To identify the risk factors for psychological distress in electroencephalography (EEG) technicians during the coronavirus disease 2019 (COVID-19) pandemic. Method: In this national-level cross-sectional survey initiated by Japan Young Epilepsy Section (YES Japan), a questionnaire was administered to 173 technicians engaged in EEG at four clinics specializing in epilepsy care and 20 hospitals accredited as (quasi-) epilepsy centers or epilepsy training facilities in Japan from March 1 to April 30, 2021. We collected data on participants' profiles, information about work, and psychological distress outcome measurements, such as the K-6 and Tokyo Metropolitan Distress Scale for Pandemic (TMDP). Linear regression analysis was used to identify the risk factors for psychological distress. Factors that were significantly associated with psychological distress in the univariate analysis were subjected to multivariate analysis. Results: Among the 142 respondents (response rate: 82%), 128 were included in the final analysis. As many as 35.2% of EEG technicians have been under psychological distress. In multivariate linear regression analysis for K-6, female sex, examination for patients (suspected) with COVID-19, and change in salary or bonus were independent associated factors for psychological distress. Contrastingly, in multivariate linear regression analysis for TMDP, female sex, presence of cohabitants who had to be separated from the respondent due to this pandemic, and change in salary or bonus were independent associated factors for psychological distress. Conclusion: We successfully identified the risk factors associated with psychological distress in EEG technicians during the COVID-19 pandemic. Our results may help in understanding the psychological stress in EEG technicians during the COVID-19 pandemic and improving the work environment, which is necessary to maintain the mental health of EEG technicians. (c) 2021 Elsevier Inc. All rights reserved.

DOI： 10.1016/j.yebeh.2021.108361

Web of Science

Scopus

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER  

Background: Spinal sagittal malalignment affects a patient's quality of life, and the condition has gained increasing importance. The cranial center of the gravity-sagittal vertical axis (CCG-SVA) helps in assessing sagittal alignment. Two major landmarks of the CCG are used for measuring the CCG-SVA: the midpoint of the nasion-inion line (MNI) and the external auditory canal (EAC). However, the correspondence between these two points has not been proved. In this study, we radiologically examined the positional relationship between them.Methods: We obtained lateral skull radiographs from 87 patients aged between 21 and 91 years. We evaluated the following: the vertical distance between the MNI and the upper margin of the EAC (Distance A), the horizontal distance between the plumb line from the MNI and the anterior margin of the EAC (Distance B), and the horizontal distance between the plumb line from the MNI and the center of the EAC (Distance C).Results: The average values of Distances A, B, and C were 19.6 +/- 5.0, 1.65 +/- 5.7, and 5.45 +/- 5.5 mm, respectively, indicating that the plumb lines dropped from the MNI and the EAC did not match exactly. The measured values showed no sex-specific differences (P > 0.05, Mann-Whitney U test).Conclusions: The present study demonstrated that the MNI and EAC locations had minimal horizontal differences. The CCG-SVA should be measured by considering the differences between the MNI and the EAC.

DOI： 10.1016/j.inat.2021.101299

Web of Science

Scopus

Language：English   Publisher：(一社)日本脳神経外科学会  

症例は22歳男性で、頭痛、めまい、失見当識、全般発作を呈していた。小児期からの再発性口内潰瘍および結節性紅斑の既往歴、てんかんの家族歴があった。MRIにて脳橋に病変が認められた。ステロイドパルス療法を行ったところ、症状の改善が認められた。しかし、発症から1年後に再発を認め、神経ベーチェット病(NBD)と診断された。MRI T2強調画像にて、脳幹、小脳、右内側側頭葉、両側基底核に高信号が認められた。また、左海馬の萎縮も認めた。ステロイドおよび免疫抑制剤の投与により、NBDはコントロールされた。しかし、NBD発症から3年後、内側側頭葉てんかんを発症した。薬剤耐性となり、NBD発症から12年後に手術が検討された。術前MRIにて左海馬硬化が認められた。NBD発症から13年後に左側頭葉前部切除術が施行された。術後2年以上、無発作状態が認められた。

Language：English   Publisher：(一社)日本脳神経外科学会  

症例は13歳男児で、先天性脳性麻痺と精神遅滞があった。過去にウエスト症候群と診断され、副腎皮質刺激ホルモン療法を受けたが、毎日のように体の両側に筋反射や痙攣を伴う薬剤抵抗性のてんかんを発症していた。11歳時に約1分間続く強直発作を呈し、数分おきに数回繰り返された。てんかん重積の診断に基づき、ミダゾラム持続静脈注射(IV-MDZ)や経口薬などで薬物治療が行われ、1分未満の強直発作を1日2〜8回繰り返す散発性けいれん群発(SC)が月に1〜2回発生する状態になった。2年後、持続性SCを呈してミダゾラム持続静脈注射(IV-MDZ)や経口薬などで薬物治療が行われたが改善しなかった。迷走神経刺激(VNS)デバイスを植え込み、VNSを迅速に導入した。IV-MDZを漸減させてもSCが悪化しなかったため、VNS導入4週間後にMDZを中止した。難治性でないSCもVNSの10ヵ月後には消失した。発作の重症度は改善し、頻度は毎日から数ヵ月に1回に減少した。脳波上のてんかん活動も有意に低下した。

Language：English   Publisher：Hiroshima University Medical Press  

<p>Background: Gadolinium-enhanced magnetic resonance imaging (MRI) can be used to observe the progression of cerebral infarction, which sometimes mimics malignant brain tumors. While the β2-microglobulin (β2MG) level in blood plasma or cerebrospinal fluid (CSF) is useful for the diagnosis of malignant tumors or degenerative diseases, these results may create confusion regarding a definitive diagnosis, because it is not a specific marker. We present a rare case of symptomatic developmental venous anomaly (DVA), accompanied by transient, irregular, enhanced cerebral lesions and elevated β2MG in the CSF.</p><p>Case Description: A 56-year-old woman developed dysarthria and underwent MRI, which revealed a right frontal hyperintense area around a previous lesion on diffusion-weighted imaging (DWI). She was treated based on the tentative diagnosis of an ischemic cerebrovascular event, and symptoms subsided in 3 days. MRI on day 7 revealed an enlargement of the hyperintense area on DWI. Post-gadolinium MRI showed multiple, enhanced patchy areas in the right frontal lobe and an abnormally large vein connected to dilated medullary venules, indicating DVA. Magnetic resonance angiography showed no stenosis or arterial occlusion. The β2MG level in the CSF was elevated at 2,061 μg/l, and a differential diagnosis from malignant tumor was required. However, MRI on day 23 revealed total disappearance of the enhanced lesions and a decrease in the high intensity area on DWI. Considering the clinical course, the DVA was symptomatic because of the perfusion disturbance.</p><p>Conclusion: Careful evaluation is necessary when considering the associated pathologies and potential complications of DVA if detected near a gadolinium-enhanced lesion.</p>

DOI： 10.24811/hjms.68.1_13

Language：Japanese   Publisher：一般社団法人 日本てんかん学会  

DOI： 10.3805/jjes.36.657

Language：English   Publishing type：Research paper (scientific journal)  

Glioblastomas (GBMs) are the most aggressive types of central nervous system tumors. Although certain genomic alterations have been identified as prognostic biomarkers of GBMs, the histomorphological features that predict their prognosis remain elusive. In this study, following an integrative diagnosis of 227 GBMs based on the 2021 World Health Organization classification system, the cases were histologically fractionated by cellular variations and abundance to evaluate the relationship between cellular heterogeneity and prognosis in combination with O-6-methylguanine-DNA methyltransferase gene promoter methylation (mMGMTp) status. GBMs comprised four major cell types: astrocytic, pleomorphic, gemistocytic, and rhabdoid cells. t-distributed stochastic neighbor embedding analysis using the histological abundance of heterogeneous cell types identified two distinct groups with significantly different prognoses. In individual cell component analysis, the abundance of gemistocytes showed a significantly favorable prognosis but confounding to mMGMTp status. Conversely, the abundance of epithelioid cells was correlated with the unfavorable prognosis. Linear model analysis showed the favorable prognostic utility of quantifying gemistocytic and epithelioid cells, independent of mMGMTp. The evaluation of GBM cell histomorphological heterogeneity is more effective for prognosis prediction in combination with mMGMTp analysis, indicating that histomorphological analysis is a practical and useful prognostication tool in an integrative diagnosis of GBMs.

DOI： 10.1038/s41598-024-76826-8

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Previously, we constructed a DNA-based next-generation sequencing (NGS) panel for an integrated diagnosis of gliomas according to the 2021 World Health Organization classification system. The aim of the current study was to evaluate the feasibility of a modified panel to include fusion gene detection via RNA-based analysis. Using this bimodal DNA/RNA panel, we analyzed 210 cases of gliomas and others to identify fusion genes in addition to gene alterations, including TERT promoter (TERTp) mutation and 1p/19q co-deletion, in formalin-fixed paraffin-embedded tissues. Of the 210 patients, fusion genes were detected in tumors of 35 patients. Eighteen of 112 glioblastomas (GBs) harbored fusion genes, including EGFR and FGFR3 fusions. In IDH-mutant astrocytoma, 6 of 30 cases showed fusion genes such as MET and NTRK2 fusions. Eleven molecular GBs and 20 not-elsewhere-classified cases harbored no gene fusions. Other 11 tumors including ependymoma, pilocytic astrocytoma, diffuse hemispheric glioma, infant-type hemispheric glioma, and solitary fibrous tumors exhibited diagnostic fusion genes. Overall, our results suggest that the all-in-one bimodal DNA/RNA panel is reliable for detecting diagnostic gene alterations in accordance with the latest WHO classification. The integrative pathological and molecular strategy could be valuable in confirmation of diagnosis and selection of treatment options for brain tumors.

DOI： 10.1016/j.prp.2024.155598

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Primary spinal cord gliomas are rare and are associated with high mortality. Unlike brain tumors, the clinicopathological features of spinal cord gliomas are not well defined. We analyzed clinical, histopathology, and immunohistochemical features and overall survival (OS) of 25 patients with primary spinal cord gliomas treated between 1994 and 2023 at 4 institutions. IDH1 R132H, H3K27M, and p53 were assessed by immunohistochemistry (IHC). Four (16%), 5 (20%), 2 (8%), and 13 (52%) patients were diagnosed as having grades 1, 2, 3, and 4 gliomas according to the World Health Organization (WHO) 2021 classification, respectively. One case (4%), with a circumscribed diffuse midline glioma, H3K27-altered, had a rare molecular profile and could not be graded. IHC demonstrated H3K27M positivity, indicative of H3F3A K27M or HIST1H3B K27M mutation, in 9 (36%) patients. H3K27me3-loss was evident in 13 (52%) patients. In one patient with a grade 1 tumor that showed negative staining for H3K27M and H3K27me3 loss, numbers of EZHIP-positive cells were increased, suggesting diffuse midline glioma, H3K27-altered (WHO grade 4). H3K27me3 loss, frequency of p53 positive cells (≥10%), MIB-1 index (≥10%), and high histopathological grades significantly correlated with poor OS. These results indicate the pathological and immunohistochemical characteristics of primary spinal cord gliomas that impact prognosis.

DOI： 10.1093/jnen/nlae084

PubMed

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本小児神経外科学会  

Language：Japanese   Publisher：(一社)日本小児神経外科学会  

Language：English   Publishing type：Research paper (scientific journal)  

A prompt and reliable molecular diagnosis for brain tumors has become crucial in precision medicine. While Comprehensive Genomic Profiling (CGP) has become feasible, there remains room for enhancement in brain tumor diagnosis due to the partial lack of essential genes and limitations in broad copy number analysis. In addition, the long turnaround time of commercially available CGPs poses an additional obstacle to the timely implementation of results in clinics. To address these challenges, we developed a CGP encompassing 113 genes, genome-wide copy number changes, and MGMT promoter methylation. Our CGP incorporates not only diagnostic genes but also supplementary genes valuable for research. Our CGP enables us to simultaneous identification of mutations, gene fusions, focal and broad copy number alterations, and MGMT promoter methylation status, with results delivered within a minimum of 4 days. Validation of our CGP, through comparisons with whole-genome sequencing, RNA sequencing, and pyrosequencing, has certified its accuracy and reliability. We applied our CGP for 23 consecutive cases of intracranial mass lesions, which demonstrated its efficacy in aiding diagnosis and prognostication. Our CGP offers a comprehensive and rapid molecular profiling for gliomas, which could potentially apply to clinical practices and research primarily in the field of brain tumors.

DOI： 10.1007/s10014-023-00476-3

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Spondylotic changes in the cervical spine cause degeneration, leading to cervical spinal canal stenosis. This stenotic change can affect cerebrospinal fluid (CSF) dynamics by compressing the dural sac and reducing space in the subarachnoid space. We examined CSF dynamics at the craniovertebral junction (CVJ) using time-spatial labeling inversion pulse magnetic resonance imaging (Time-SLIP MRI) in patients with cervical spinal canal stenosis. METHODS: The maximum longitudinal movement of the CSF at the CVJ was measured as length of motion (LOM) in the Time-SLIP MR image of 56 patients. The sum of ventral and dorsal LOM was defined as the total LOM. Patients were classified into three groups depending on their spinal sagittal MRI findings: control (n=27, Kang classification grades 0 and 1), stenosis (n=14, Kang classification grade 2), and severe stenosis (n=15, Kang classification grade 3). RESULTS: Time-SLIP MRI revealed pulsatile movement of the CSF at the CVJ. The mean total, ventral, and dorsal LOM was 14.2 ± 9, 8.1 ± 5.7, and 3.8 ± 2.9 mm, respectively. The ventral LOM was significantly larger than the dorsal LOM. The total LOM was significantly smaller in the severe stenosis group (6.1 ± 3.4 mm) than in the control (16.0 ± 8.4 mm) or stenosis (11 ± 5.4 mm) groups (p < 0.001, Kruskal-Wallis H-test). In five patients, postoperative total LOM was improved after adequate decompression surgery. CONCLUSIONS: This study demonstrates that CSF dynamics at the CVJ are influenced by cervical spinal canal stenosis. Time-SLIP MRI is useful for evaluating CSF dynamics at the CVJ in patients with spinal canal stenosis.

DOI： 10.1016/j.wneu.2024.02.020

Scopus

PubMed

Language：Japanese   Publisher：(一社)日本病理学会  

Language：Japanese   Publisher：(一社)日本定位・機能神経外科学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本定位・機能神経外科学会  

Language：Japanese   Publisher：(一社)日本定位・機能神経外科学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：English  

BACKGROUND: Although rare, cases of hypophysitis resembling a pituitary abscess (PA) have been reported. Differential diagnosis between hypophysitis and PA is crucial as the two diseases require different treatments. CASE DESCRIPTION: A 38-year-old woman with headaches underwent head magnetic resonance imaging (MRI), which revealed an 11-mm mass lesion in the sella turcica. Due to breastfeeding, contrast-enhanced MRI was avoided. Pituitary adenomas and Rathke's cleft cyst (RCC) were suspected, and she was initially treated conservatively. Five months later, she acquired syndrome coronavirus two infections, and while the fever subsided with acetaminophen, the headache persisted. One month later, the headache worsened, followed by fever and diabetes insipidus. MRI revealed a pituitary cystic mass with ring-shaped contrast enhancement on T1-weighted MRI and increased signal intensity on diffusion-weighted imaging (DWI). PA was suspected, and emergency endoscopic transsphenoidal surgery was performed. The microbiological examination of the yellowish-brown content drained from the cystic mass was negative. Microscopically, the cystic lesion was covered with ciliated columnar epithelium and stratified squamous epithelium, with a dense inflammatory cell infiltrate consisting mainly of lymphocytes and plasma cells observed around the cyst. This supported the diagnosis of secondary hypophysitis associated with RCC without PA. CONCLUSION: We report a case of hypophysitis secondary to RCC resembling PA with ring-shaped contrast enhancement on MRI and increased signal intensity on DWI. This case emphasizes the need for cautious diagnosis of secondary hypophysitis due to RCC in individuals with MRIs and clinical manifestations resembling an abscess.

DOI： 10.25259/SNI_947_2023

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: In this study, we examined the impact and degree of lumbar stenosis on cerebrospinal fluid (CSF) protein concentration. METHODS: In this retrospective study, we analyzed protein concentrations in CSF samples of 61 patients with lumbar spinal stenosis (LSS) obtained during pre-operative myelography. Patients were divided into two groups: those showing no block to contrast (Group A) versus those showing medium block to contrast below the lumbar puncture level (Group B). RESULTS: The CSF protein concentration in Group B (104.3 ± 59 g/dL) patients with medium block was significantly greater than that in Group A (65.1 ± 33 g/dL) patients without medium block. CONCLUSION: A higher average CSF protein concentration was seen in Group B patients with significant lumbar stenosis versus Group A patients without significant lumbar stenosis. Theoretically, damage to the cauda equina in patients with LSS may cause these elevated CSF protein levels.

DOI： 10.25259/SNI_610_2024

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Utilizing computed tomography (CT) studies, we correlated cervical spinal canal diameters (SCDs) with pedicle size between the C3 and C7 levels to more safely perform posterior cervical surgery. METHODS: We retrospectively analyzed CT studies for 71 patients with cranial or spinal disorders and correlated the cervical SCD with the pedicle outer width (POW) between the C3 and C7 levels. Patients were divided into normal (SCD ≥12 mm at any level, n = 30) and stenosis groups (SCD <12 mm at any level, n = 41). RESULTS: C7 exhibited the largest SCD and POW values, while C3 and C4 exhibited the smallest SCD and POW values. Moderate correlations (r = 0.3, P = 0.002) were observed at the C3 and C4 levels but no significant correlations were observed from the C5 to C7 levels. For SCD values, the normal group demonstrated significantly greater values between the C3 and C7 levels versus the stenosis group. For POW values, only the C4 level differed significantly between the two groups (P = 0.014, Mann-Whitney U-test). CONCLUSION: Preoperative pedicle size evaluation remains an essential manoeuvre before performing cervical C3-C7 pedicle screw placement. In 71 cervical CT studies, we found no consistent correlation between POW and SCD values, indicating that it is difficult to estimate POW values based on spinal canal size.

DOI： 10.25259/SNI_590_2024

Scopus

PubMed

Language：English   Publisher：The Japan Neurosurgical Society  

<p>Internal carotid artery aplasia or hypoplasia above the cervical bifurcation is rare, occurring in less than 0.01% of the general population. Unilateral neurocristopathy complicated by unilateral internal carotid artery agenesis or hypogenesis has been reported, but bilateral internal carotid artery hypoplasia is rare and scarcely reported. Herein, we report a novel case of Treacher Collins syndrome complicated by bilateral internal carotid artery hypoplasia. A 94-year-old woman presented with complaints of headache and vomiting. Computed tomography revealed a subarachnoid hemorrhage and dysplasia of the bilateral zygoma, mandible, and external auditory meatus. The patient had severe hearing loss and visual impairment. Computed tomography angiography revealed bilateral internal carotid artery hypoplasia and multiple aneurysmal changes in the intracranial arteries. We diagnosed the patient with a ruptured anterior inferior cerebellar artery aneurysm and performed coil embolization. The patient's unique facial features were consistent with neurocristopathy, especially Treacher Collins syndrome. Developmental anomalies of neural crest cells can present as vascular abnormalities and craniofacial malformations. Special care is required for endovascular treatment and airway management in cases of neurocristopathy because of the specific craniofacial anomalies.</p>

DOI： 10.2176/jns-nmc.2022-0267

PubMed

Language：English   Publisher：The Japan Neurosurgical Society  

<p>Hemorrhagic pilocytic astrocytomas (PAs) are rare, accounting for 1.1%-8.0% of all PA cases. They are reported to occur more frequently in older populations, with a male predominance. In this study, we report a case of a 14-year-old boy who presented with a headache, vertigo, and diplopia. As per his brain computed tomography scan, a small hematoma was observed in the left inferior cerebellar peduncle. Follow-up magnetic resonance imaging (MRI) revealed repeated minor bleeding from the lesion and mild expansion, with no neurological deficits. Four years later, the patient developed nausea, vomiting, and left abducens palsy. MRI revealed a mulberry-shaped mass surrounded by a hypointense rim, suggesting a cavernous angioma. The lesion was surgically resected via midline occipital craniotomy with the opening of the cerebellomedullary fissure. Histopathological examination of the lesion revealed PA. Next-generation sequencing analyses revealed that PAs harbored mutations in the <i>ARID1A</i>, <i>ATM</i>, and <i>POLE</i> genes but not in the <i>BRAF</i> gene. To the best of our knowledge, there are yet no reported studies on these mutations in PAs to date. Thus, PA should be considered in the differential diagnosis of cerebellar hemorrhage, especially in young adults and children. </p>

DOI： 10.2176/jns-nmc.2023-0152

PubMed

Language：Japanese   Publisher：(株)日本臨床社  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: This study aimed to elucidate the impact of effective diffusion time setting on apparent diffusion coefficient (ADC)-based differentiation between primary central nervous system lymphomas (PCNSLs) and glioblastomas (GBMs) and to investigate the usage of time-dependent diffusion magnetic resonance imaging (MRI) parameters. METHODS: A retrospective study was conducted involving 21 patients with PCNSLs and 66 patients with GBMs using diffusion weighted imaging (DWI) sequences with oscillating gradient spin-echo (Δeff = 7.1 ms) and conventional pulsed gradient (Δeff = 44.5 ms). In addition to ADC maps at the two diffusion times (ADC7.1 ms and ADC44.5 ms), we generated maps of the ADC changes (cADC) and the relative ADC changes (rcADC) between the two diffusion times. Regions of interest were placed on enhancing regions and non-enhancing peritumoral regions. The mean and the fifth and 95th percentile values of each parameter were compared between PCNSLs and GBMs. The area under the receiver operating characteristic curve (AUC) values were used to compare the discriminating performances among the indices. RESULTS: In enhancing regions, the mean and fifth and 95th percentile values of ADC44.5 ms and ADC7.1 ms in PCNSLs were significantly lower than those in GBMs (p = 0.02 for 95th percentile of ADC44.5 ms, p = 0.04 for ADC7.1 ms, and p < 0.01 for others). Furthermore, the mean and fifth and 95th percentile values of cADC and rcADC were significantly higher in PCNSLs than in GBMs (each p < 0.01). The AUC of the best-performing index for ADC7.1 ms was significantly lower than that for ADC44.5 ms (p < 0.001). The mean rcADC showed the highest discriminating performance (AUC = 0.920) among all indices. In peritumoral regions, no significant difference in any of the three indices of ADC44.5 ms, ADC7.1 ms, cADC, and rcADC was observed between PCNSLs and GBMs. CONCLUSIONS: Effective diffusion time setting can have a crucial impact on the performance of ADC in differentiating between PCNSLs and GBMs. The time-dependent diffusion MRI parameters may be useful in the differentiation of these lesions.

DOI： 10.1186/s40644-023-00639-7

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

To assess the use of indocyanine green (ICG) fluorescence endoscopy to evaluate pituitary blood flow in craniopharyngioma resection and its possible impact on intraoperative decisions regarding pituitary stalk processing. Patients with craniopharyngiomas who had undergone transsphenoidal surgery since March 2021, when an ICG endoscope was introduced at the Kagoshima University Hospital, were included in the study. When targeted tumor removal was approaching completion, 10 mg of ICG was administered intravenously to evaluate blood flow in the pituitary stalk and gland. ICG signals and endocrinological status before and after surgery were evaluated retrospectively. Pituitary stalk and gland blood flow were evaluated as positive (++), weakly positive (+), and no signal (-).Ten patients with craniopharyngiomas underwent transsphenoidal surgery using an ICG endoscope (mean age 56.6 ± 14.2 years; 40% male). Among the eight patients in whom the pituitary stalk was preserved, pituitary function with positive signal on the stalk was intact in two. Two other patients with weakly positive stalk and positive pituitary gland signals showed intact function or minimal pituitary dysfunction. Four patients had impairments in more than three axes with poor ICG signals in the stalk or pituitary gland. Two patients underwent pituitary amputation because of high tumor invasion and lack of ICG signal in the stalk after tumor removal, resulting in panhypopituitarism. A negative ICG signal in the pituitary stalk is likely to indicate postoperative pituitary function loss. Craniopharyngioma surgery using ICG endoscopy may be useful for predicting endocrine prognosis and improving tumor outcomes.

DOI： 10.1007/s10143-023-02223-w

Scopus

PubMed

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(一社)日本病理学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：English   Publisher：(一社)日本癌治療学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Perampanel is an oral anti-seizure medication, which is approved in Japan for focal-onset seizures, with/without focal to bilateral tonic-clonic seizures, as monotherapy/adjunctive therapy in patients aged 4 years and older. Treatment for generalized tonic-clonic seizures as adjunctive therapy in patients aged 12 years and older is approved as well. We evaluated the feasibility of intravenous (IV) administration of perampanel as an alternative to oral administration. METHODS: Study 240 (NCT03754582) was an uncontrolled, open-label study of IV perampanel, conducted in 21 Japanese patients with epilepsy who received a stable dose of 8-12 mg/day of oral perampanel. Patients received 30-minute IV infusions at equivalent daily doses of oral perampanel for 4 days, then were switched back to oral perampanel. Safety, tolerability, plasma concentration, and maintenance of efficacy throughout the transition between IV and oral dosing of perampanel were assessed. As supportive data, a subgroup analysis was also conducted using data from healthy Japanese subjects (n = 18) who were enrolled in Study 050 (NCT03376997) investigating the pharmacokinetics and safety of IV perampanel in healthy subjects who received an IV infusion (30-, 60-, or 90-minute) of perampanel 12 mg and a single oral administration of perampanel 12-mg tablet. RESULTS: In Study 240, the transition between 30-minute IV and oral perampanel dosing was associated with a ≤1.4-fold increase in the mean change in maximum observed concentration of perampanel. Seizure outcomes demonstrated no considerable changes in efficacy before, during, or after 30-minute IV dosing of perampanel. The safety profiles were similar between IV and oral formulations. In Study 050, the pharmacokinetics of 30- or 60-minute IV infusion of perampanel further support the interchangeability between oral and IV formulations in the Japanese subjects. SIGNIFICANCE: These results support that 30-minute IV perampanel may be a potential short-term alternative to oral formulations for patients with epilepsy.

DOI： 10.1002/epi4.12804

Scopus

PubMed

Language：English  

Amplification of the epidermal growth factor receptor gene (EGFR) and its variants are the most commonly detected pathogenic gene alterations in glioblastoma. Herein, we report a case of molecularly defined glioblastoma harboring an EGFR variant III (EGFRvIII) without EGFR amplification. The initial histological diagnosis was isocitrate dehydrogenase (IDH)-wildtype low-grade glioma, due to an absence of anaplasia, necrosis, and microvascular proliferation, and a low Ki-67 labeling index. DNA-based next-generation sequencing (NGS) panel analysis revealed a TERTp promoter mutation but no EGFR mutation or amplification, supporting the diagnosis of "molecular glioblastoma." However, RNA-based NGS panel analysis revealed mRNA expression of EGFRvIII. Therefore, the final integrative diagnosis was glioblastoma with non-amplified EGFRvIII. Our report suggests that non-amplified EGFRvIII might be an early molecular event in glioblastoma tumorigenesis. In addition to the usual DNA-based analysis, RNA-based analysis is required to identify exon-skipping EGFR variants without EGFR amplification.

DOI： 10.1016/j.prp.2023.154712

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)  

The hypothalamus is part of the diencephalon and regulates not only endocrine functions but also various physiological functions, including controlling sleep and wakefulness, eating and drinking, and regulating body temperature. The pituitary gland can be divided into the adenohypophysis and neurohypophysis based on its developmental origin. The hypothalamus and pituitary gland play important roles in maintaining homeostasis by closely coordinating hormones. In the treatment of hypothalamic and pituitary diseases, it is important to understand the functions of the hypothalamus and pituitary gland, preserve hypothalamic function, evaluate the endocrine function, and follow up appropriately throughout life, including hormone replacement without excess or deficiency. Additionally, when interpreting hormone data, it is necessary to understand the factors that influence test values.

DOI： 10.11477/mf.1436204790

PubMed

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point ・視床下部と下垂体は,ホルモンを介した綿密な連携で生体の恒常性維持に重要な役割を担っている.・ホルモンデータを解釈する際は,検査値に影響を与える因子を理解しておく必要がある.

Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01228&link_issn=&doc_id=20230801210005&doc_link_id=10.11477%2Fmf.1436204790&url=https%3A%2F%2Fdoi.org%2F10.11477%2Fmf.1436204790&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：English  

INTRODUCTION: Intracranial electroencephalography is a crucial diagnostic technique for epilepsy surgery, though it is associated with a range of complications, including infection, intracranial hemorrhage, increased intracranial pressure, and cerebral infarction. This case study presents an uncommon occurrence of stenosis of the left posterior cerebral artery (PCA) following intracranial electrode implantation. CASE PRESENTATION: A woman in her thirties with drug-resistant focal impaired awareness seizures underwent implantation of subdural and depth electrodes on the bilateral temporal lobes to lateralize seizure onset. A left anterior-temporal lobectomy was performed based on the evaluation results. Following the resection of the hippocampus, stenosis of the left PCA, with a pinched appearance, was observed. Postoperatively, extensive cerebral edema in the bilateral temporal lobes and a defect in the left PCA were detected on magnetic resonance (MR) imaging. MR imaging performed the day after surgery showed cerebral infarction in the left medial temporal lobe and left lateral thalamus. A video review indicated that surgical manipulation was not the cause of vascular stenosis. MR angiography one week later confirmed the recanalization of the PCA. DISCUSSION: We surmised that the subdural electrodes inserted along the middle skull base might have induced the PCA stenosis or spasms. The patient did not experience any significant sequelae, with no episodes of seizures for more than five years after surgery. CONCLUSION: It is essential to note that subdural grid electrodes placed in the medial temporal lobe can cause vascular stenosis, albeit with an extremely rare occurrence.

DOI： 10.1016/j.ijscr.2023.107988

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: There is a large disparity between the quality of neurosurgical research in developed and developing nations, including the Association of Southeast Asian Nations (ASEAN). Bibliometric analysis is an appropriate method for evaluating the quality of research in a geographic region. We analyzed the neurosurgery reports published by the ASEAN countries to highlight regional productivity in neurosurgery research. METHODS: We performed a bibliometric analysis of neurosurgery reports from the ASEAN countries between 2011 and 2020. We described the publication number, study type, and field of study of the publication articles. In addition, we assessed the correlations between the neurosurgery research productivity of these countries and the neurosurgeon ratio, medical doctor ratio, per capita gross domestic product, and health expenditure per capita. RESULTS: We identified 1939 neurosurgeons in the ASEAN region; 570 articles were published by neurosurgeons in the region between 2011 and 2020. Singaporean neurosurgeons were the most productive, with 177 articles, accounting for 31% of the total ASEAN neurosurgeon publications in the study period. However, there has been a rapid recent increase in the number of articles. Case reports and tumors were the dominant type and field of the articles, respectively. There was no significant correlation between the neurosurgical research productivity of the ASEAN countries and the neurosurgeon ratio, medical doctor ratio, per capita gross domestic product, and health expenditure per capita. CONCLUSIONS: By analyzing the neurosurgery publications from ASEAN countries over the previous decade, we highlight the status of neurosurgical research in these countries.

DOI： 10.1016/j.wneu.2023.01.059

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Language：Japanese   Publisher：(一社)日本小児神経外科学会  

Language：English   Publishing type：Research paper (scientific journal)  

Background  Postdural puncture headache (PDPH) is defined as a prolonged orthostatic headache secondary to a lumbar puncture. The mechanism underlying this unpleasant complication and the reasons explaining its higher incidence in the young are not well understood. Here, we speculate on the mechanisms underlying PDPH based on spinal magnetic resonance imaging (MRI) in patients with PDPH and an anatomical study on the size of the intervertebral foramen. Methods  Brain and spinal MRI findings were examined in two young women with PDPH. The relationship between age and size of the intervertebral foramen on computed tomography was assessed in 25 female volunteers (22-89 years old) without spinal disease. Results  The causative interventions leading to PDPH were epidural anesthesia for painless delivery in a 28-year-old woman and lumbar puncture for examination of the cerebrospinal fluid (CSF) in a 17-year-old woman. These two patients developed severe orthostatic hypotension following the procedure. Brain MRI showed signs of intracranial hypotension, including subdural effusion, in one patient, but no abnormality in the other. Spinal MRI revealed an anterior shift of the spinal cord at the thoracic level and CSF exudation into the paravertebral space at the lumbar level. Treatment involving an epidural blood patch in one patient and strict bed rest with sufficient hydration in the second led to improvement of symptoms and reduction of paravertebral CSF exudation. The size of the intervertebral foramen at the L2-3 level in the 25 volunteers showed a decrease in an age-dependent manner (Spearman's rho -0.8751, p  < 0.001). Conclusion  We suggest that CSF exudation from the epidural space of the vertebral canal to the paravertebral space through the intervertebral foramen, which is generally larger in the younger population, is the causative mechanism of PDPH.

DOI： 10.1055/s-0043-1763526

PubMed

Language：English  

INTRODUCTION AND IMPORTANCE: Intracranial germinomas are germ cell tumors that commonly develop in the pineal or neurohypophysis regions. As ectopic germinomas are rarely observed within the cerebrum and are associated with atypical image findings, diagnosis is challenging. CASE PRESENTATION: A 14-year-old boy was admitted to our hospital with complaints of vomiting and headache. Gadolinium-enhanced magnetic resonance imaging revealed ring-enhancing lesions in his left frontal lobe and basal ganglia. Susceptibility-weighted imaging indicated that the subependymal veins passing through the lesion centers were engorged, while electrophoretic analysis of cerebrospinal fluid identified oligoclonal bands (OCBs); both were typical of multiple sclerosis (MS). Tumor biopsy revealed many cells with atypical mitotic figures and nuclear enlargements, suggesting malignant disease. As the tumor rapidly proliferated, we opted for surgical excision of the lesions. Histopathological analyses revealed "two-cell patterns" characteristic of germinoma. Immunohistochemistry was positive for placental alkaline phosphatase and c-KIT. The definitive diagnosis was germinoma. After chemoradiotherapy, the patient was discharged without neurological deficits. CLINICAL DISCUSSION: OCBs and several magnetic resonance imaging features (including open ring enhancement, T2 hypointense rims, mild mass effects, mild perilesional edema, peripheral restriction around the lesion, and vessel-like structures running through the lesion center) are useful diagnostic signs for the radiological discrimination of MS from germinoma. However, owing to these factors, some cases are difficult to diagnose. CONCLUSION: Our case report of an unusual ectopic cerebral germinoma illustrates the difficulty of distinguishing it from MS. Therefore, we recommend proper tissue sampling in such cases, especially in adolescent patients, to make definitive germinoma diagnoses.

DOI： 10.1016/j.ijscr.2023.107884

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Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a neurological disorder presenting a triad including dementia and ventricular enlargement. The mechanism causing excessive cerebrospinal fluid (CSF) accumulation in the ventricles in iNPH is poorly understood. We hypothesized that the age-related degradation of the spinal shock-absorbing system composed of a spinal dural sac (SDS) and surrounding soft tissue, preventing ventricular enlargement caused by wide CSF pulsation driven by heartbeats, may be involved in the ventricular enlargement observed in iNPH. METHODS: Sixty-four patients with iNPH in their seventies who underwent a lumboperitoneal shunt and a control group of 79 people in the same age group who underwent brain check-ups were included in the study. We compared the sizes of the cervical and upper parts of the thoracic SDS using magnetic resonance imaging between the two groups. RESULTS: The anterior-posterior distances of the dural sac at C5 were shorter in patients with iNPH of both sexes than those in the control group (P = 0.0008 in men and P = 0.0047 in women). The number of disc levels with disappeared CSF space surrounding the cervical cord was more in iNPH (P = 0.0176 and P = 0.0003). The midsagittal area of the upper part of the spinal sac, C2-Th4, was smaller in iNPH (P = 0.0057 and P = 0.0290). CONCLUSION: Narrowing of the cervical dural sac and midsagittal area in the upper part of the SDS in patients with iNPH may reflect the degradation of the shock-absorbing mechanism for CSF pressure pulsations, which may cause iNPH or at least aggravate iNPH by other unknown causes.

DOI： 10.25259/SNI_474_2023

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Telomerase reverse transcriptase promoter (TERTp) mutations are a biological marker of glioblastoma; however, the prognostic significance of TERTp mutational status is controversial. We evaluated this impact by retrospectively analyzing the outcomes of patients with isocitrate dehydrogenase (IDH)- and TERTp-wild-type glioblastomas. METHODS: Using custom next-generation sequencing, we analyzed 208 glioblastoma samples harboring wild-type IDH. RESULTS: TERTp mutations were detected in 143 samples (68.8%). The remaining 65 (31.2%) were TERTp-wild-type. Among the TERTp-wild-type glioblastoma samples, we observed a significant difference in median progression-free survival (18.6 and 11.4 months, respectively) and overall survival (not reached and 15.7 months, respectively) in patients with and without phosphatase and tensin homolog (PTEN) loss and/or mutation. Patients with TERTp-wild-type glioblastomas with PTEN loss and/or mutation were younger and had higher Karnofsky Performance Status scores than those without PTEN loss and/or mutation. We divided the patients with TERTp-wild-type into 3 clusters using unsupervised hierarchical clustering: Good (PTEN and TP53 alterations; lack of CDKN2A/B homozygous deletion and platelet-derived growth factor receptor alpha (PDGFRA) alterations), intermediate (PTEN alterations, CDKN2A/B homozygous deletion, lack of PDGFRA, and TP53 alterations), and poor (PDGFRA and TP53 alterations, CDKN2A/B homozygous deletion, and lack of PTEN alterations) outcomes. Kaplan-Meier survival analysis indicated that these clusters significantly correlated with the overall survival of TERTp-wild-type glioblastoma patients. CONCLUSIONS: Here, we report that PTEN loss and/or mutation is the most useful marker for predicting favorable outcomes in patients with IDH- and TERTp-wild-type glioblastomas. The combination of 4 genes, PTEN, TP53, CDKN2A/B, and PDGFRA, is important for the molecular classification and individual prognosis of patients with IDH- and TERTp-wild-type glioblastomas.

DOI： 10.1093/noajnl/vdad078

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Glioblastoma (GBM) is a malignant brain tumor, with radiological and genetic heterogeneity. We examined the association between radiological characteristics and driver gene alterations. METHODS: We analyzed the driver genes of 124 patients with IDH wild-type GBM with contrast enhancement using magnetic resonance imaging. We used a next-generation sequencing panel to identify mutations in driver genes and matched them with radiological information. Contrast-enhancing lesion localization of GBMs was classified into 4 groups based on their relationship with the subventricular zone (SVZ) and cortex (Ctx). RESULTS: The cohort included 69 men (55.6%) and 55 women (44.4%) with a mean age of 66.4 ± 13.3 years. EGFR and PDGFRA alterations were detected in 28.2% and 22.6% of the patients, respectively. Contrast-enhancing lesion touching both the SVZ and Ctx was excluded because it was difficult to determine whether it originated from the SVZ or Ctx. Contrast-enhancing lesions touching the SVZ but not the Ctx had significantly worse overall survival than non-SVZ lesions (441 days vs. 897 days, P = .002). GBM touching only the Ctx had a better prognosis (901 days vs. 473 days, P < .001) than non-Ctx lesions and was associated with EGFR alteration (39.4% vs. 13.2%, P = .015). Multiple contrast lesions were predominant in PDGFRA alteration and RB1-wild type (P = .036 and P = .031, respectively). CONCLUSIONS: EGFR alteration was associated with cortical lesions. And PDGFRA alteration correlated with multiple lesions. Our results suggest that clarifying the association between driver genes and tumor localization may be useful in clinical practice, including prognosis prediction.

DOI： 10.1093/noajnl/vdad110

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

<p>Rapid technological advances in molecular biology, including next-generation sequencing, have identified key genetic alterations in central nervous system (CNS) tumors. Accordingly, the fifth edition of the World Health Organization (WHO) CNS tumor classification was published in 2021. We analyzed 303 patients with diffuse glioma using an amplicon-based glioma-tailored gene panel for detecting 1p/19q codeletion and driver gene mutations such as <i>IDH1/2</i>, <i>TERTp</i>, <i>EGFR</i>, and <i>CDKN2A/B</i> on a single platform. Within glioblastomas (GBMs), the most commonly mutated genes were <i>TERTp</i>, <i>TP53</i>, <i>PTEN</i>, <i>NF1</i>, and <i>PDGFRA</i>, which was the most frequently mutated tyrosine kinase receptor in GBM, followed by <i>EGFR</i>. The genes that most commonly showed evidence of loss were <i>PTEN</i>, <i>CDKN2A/B</i>, and <i>RB1</i>, whereas the genes that most commonly showed evidence of gain/amplification were <i>EGFR</i>, <i>PDGFRA</i>, and <i>CDK4</i>. In 22 grade III oligodendroglial tumors, 3 (14%) patients had <i>CDKN2A/B</i> homozygous deletion, and 4 (18%) patients had <i>ARID1A</i> mutation. In grade III oligodendroglial tumors, an <i>ARID1A</i> mutation was associated with worse progression-free survival. Reclassification based on the WHO 2021 classification resulted in 62.5% of grade II/III <i>isocitrate dehydrogenase</i> (<i>IDH</i>) -wildtype astrocytomas being classified as <i>IDH</i>-wildtype GBM and 37.5% as not elsewhere classified. In summary, our glioma-tailored gene panel was applicable for molecular diagnosis in the WHO 2021 classification. In addition, we successfully reclassified the 303 diffuse glioma cases based on the WHO 2021 classification and clarified the genetic profile of diffuse gliomas in the Japanese population.</p>

DOI： 10.2176/jns-nmc.2022-0103

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Language：English   Publisher：(一社)日本癌学会  

Language：English   Publisher：(一社)日本癌学会  

Language：English   Publisher：(一社)日本癌学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(株)メディカ出版  

Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J03120&link_issn=&doc_id=20220704260007&doc_link_id=issn%3D0917-1495%26volume%3D32%26issue%3D4%26spage%3D523&url=http%3A%2F%2Fwww.pieronline.jp%2Fopenurl%3Fissn%3D0917-1495%26volume%3D32%26issue%3D4%26spage%3D523&type=PierOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00005_2.gif

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Oxford University Press (OUP)  

Abstract

Background

Platelet-derived growth factor receptor alpha (PDGFRA) is the second most frequently mutated tyrosine kinase receptor in glioblastoma (GBM). However, the prognostic impact of PDGFRA amplification on GBM patients remains unclear. Herein, we evaluated this impact by retrospectively analyzing outcomes of patients with IDH wild-type GBM.

Methods

Using a custom-made oncopanel, we evaluated PDGFRA gain/amplification in 107 GBM samples harboring wild-type IDH, along with MGMT promoter (MGMTp) methylation status.

Results

We detected PDGFRA gain/amplification in 31 samples (29.0%). PDGFRA gain/amplification predicted poor prognosis (P = .003). Compared to unamplified PDGFRA, PDGFRA gain/amplification in GBM was associated with higher patient age (P = .031), higher Ki-67 score (P = .019), and lower extent of surgical resection (P = .033). Unmethylated MGMTp also predicted poor prognosis (P = .005). As PDGFRA gain/amplification and unmethylated MGMTp were independent factors for poor prognosis in multivariate analyses, we grouped GBM cases based on PDGFRA and MGMTp status: poor (PDGFRA gain/amplification and unmethylated MGMTp), intermediate (PDGFRA gain/amplification or unmethylated MGMTp), and good (PDGFRA intact and methylated MGMTp) prognosis. The Kaplan-Meier survival analysis indicated that these groups significantly correlated with the OS of GBM patients (P &amp;lt; .001).

Conclusions

Here we report that PDGFRA gain/amplification is a predictor of poor prognosis in IDH wild-type GBM. Combining PDGFRA gain/amplification with MGMTp methylation status improves individual prognosis prediction in patients with IDH wild-type GBM.

DOI： 10.1093/noajnl/vdac097

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PubMed

Other Link： https://academic.oup.com/noa/article-pdf/4/1/vdac097/45178840/vdac097.pdf

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: We aimed to evaluate the mutation profile, transcriptional variants, and prognostic impact of the epidermal growth factor receptor (EGFR) gene in isocitrate dehydrogenase (IDH)-wildtype glioblastomas (GBMs). METHODS: We sequenced EGFR, evaluated the EGFR splicing profile using a next-generation sequencing oncopanel, and analyzed the outcomes in 138 grade IV IDH-wildtype GBM cases. RESULTS: EGFR mutations were observed in 10% of GBMs. A total of 23.9% of the GBMs showed EGFR amplification. Moreover, 25% of the EGFR mutations occurred in the kinase domain. Notably, EGFR alterations were a predictor of good prognosis (p = 0.035). GBM with EGFR alterations was associated with higher Karnofsky Performance Scale scores (p = 0.014) and lower Ki-67 scores (p = 0.005) than GBM without EGFR alterations. EGFRvIII positivity was detected in 21% of EGFR-amplified GBMs. We identified two other EGFR variants in GBM cases with deletions of exons 6-7 (Δe 6-7) and exons 2-14 (Δe 2-14). In one case, the initial EGFRvIII mutation transformed into an EGFR Δe 2-14 mutation during recurrence. CONCLUSIONS: We found that the EGFR gene profiles of GBM differ among cohorts and that EGFR alterations are good prognostic markers of overall survival in patients with IDH-wildtype GBM. Additionally, we identified rare EGFR variants with longitudinal and temporal transformations of EGFRvIII.

DOI： 10.1002/cam4.4939

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The impact of the coronavirus disease 2019 (COVID-19) pandemic on epilepsy care across Japan was investigated by conducting a multicenter retrospective cohort study. METHODS: This study included monthly data on the frequency of (1) visits by outpatients with epilepsy, (2) outpatient electroencephalography (EEG) studies, (3) telemedicine for epilepsy, (4) admissions for epilepsy, (5) EEG monitoring, and (6) epilepsy surgery in epilepsy centers and clinics across Japan between January 2019 and December 2020. We defined the primary outcome as epilepsy-center-specific monthly data divided by the 12-month average in 2019 for each facility. We determined whether the COVID-19 pandemic-related factors (such as year [2019 or 2020], COVID-19 cases in each prefecture in the previous month, and the state of emergency) were independently associated with these outcomes. RESULTS: In 2020, the frequency of outpatient EEG studies (-10.7%, p<0.001) and cases with telemedicine (+2,608%, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 % per case, p<0.001) and EEG monitoring (-3.81*10-3 % per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9%, p<0.001), outpatient EEG (-32.3%, p<0.001), telemedicine for epilepsy (+12,915%, p<0.001), epilepsy admissions (-35.3%; p<0.001), EEG monitoring (-24.7%: p<0.001), and epilepsy surgery (-50.3%, p<0.001). SIGNIFICANCE: We demonstrated the significant impact that the COVID-19 pandemic had on epilepsy care. These results support those of previous studies and clarify the effect size of each pandemic-related factor on epilepsy care.

DOI： 10.1002/epi4.12616

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Language：Japanese   Publisher：(一社)日本小児神経外科学会  

Language：English   Publishing type：Research paper (scientific journal)  

Background: We previously found the usefulness of dural sac shrinkage signs (DSSSs), which are the anterior shift of the spinal cord and dura mater behind the cord, detected by magnetic resonance imaging (MRI) at the thoracic level for the diagnosis of spontaneous intracranial hypotension (IH). This is a retrospective survey on the usefulness of DSSSs for the early detection of iatrogenic IH caused by overdrainage through a lumboperitoneal shunt (LPS) for patients with idiopathic normal pressure hydrocephalus (INPH). Methods: Forty-five INPH patients had an LPS using a pressure programmable valve equipped with an anti-siphon device. Results: Nine patients complained of orthostatic headache after the LPS, indicating IH due to overdrainage, which persisted for more than a week in three patients and 2-7days in six patients. The headache was transient/ nonorthostatic in ten patients and absent in 26 patients. The DSSSs and accompanying enlargement of the venous plexus were observed in all three patients with prolonged orthostatic headaches. Only the anterior shift of the dura mater was observed in 1 (4%) among 25 patients who had short-term orthostatic headache, transient/ nonorthostatic headache, or absent headache, and underwent spinal MRI. A patient with prolonged severe orthostatic headache with both DSSSs eventually developed intracranial subdural effusion and underwent tandem valve surgery, which provided a quick improvement of symptoms. The DSSSs on thoracic MRI also disappeared promptly. Conclusion: DSSSs may serve as objective signs for the diagnosis of IH due to overdrainage through an LPS for INPH.

DOI： 10.25259/SNI_291_2022

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Publisher：Scientific Reports  

DOI： 10.1038/s41598-021-84404-5

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Pin-type head frame systems have become a worldwide standard procedure, but they can cause some complications on rare occasions. This study aimed to examine the incidence and associated risk factors of depressed skull fracture and related intracranial hematoma (DSFH) due to the use of head frames in our institute over the past 10 years. METHODS: This study included 1,749 patients who underwent neurosurgical surgeries using pin-type head frames, including the Mayfield skull clamp (721 cases) and the Sugita head frame (1,028 cases). We retrospectively reviewed hospital records of our institute to identify cases of DSFH, and documented the type of head frame used, as well as patient characteristics. RESULTS: The incidence of DSFH was 0.29% (5/1,749 cases). All 5 cases had an associated epidural hematoma, with a single case having an additional dural laceration (without subdural damage). All perforation sites, located at the parietal bone near the pterion, occurred by the unilateral horizontal screw of the Sugita head frame. None of the patients experienced postoperative neurological decline. CONCLUSIONS: Even in the adult population, the DSFH by the pin-type head frame can occur infrequently. Based on our results, we recommend that the following factors should be considered when the pin-type head frame is used for neurosurgical procedures: the location of pin application, thickness and fragility of the skull, and adequate control of compressive forces exerted by the head frame.

DOI： 10.1016/j.wneu.2021.08.070

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Spontaneous intracranial hypotension (SIH) is secondary to a cerebrospinal fluid leak at the spinal level without obvious causative events. Several signs on brain and cervical spine magnetic resonance (MR) imaging (MRI) have been associated with SIH but can be equivocal or negative. This retrospective study sought to identify characteristic SIH signs on thoracic spinal MRI. METHODS: Cranial and spinal MR images of 27 consecutive patients with classic SIH symptoms, who eventually received epidural autologous blood patches (EBPs), were analyzed. RESULTS: The most prevalent findings on T2-weighted MRI at the thoracic level were anterior shift of the spinal cord (96.3%) and dorsal dura mater (81.5%), probably caused by dural sac shrinkage. These dural sac shrinkage signs (DSSS) were frequently accompanied by cerebrospinal fluid collection in the posterior epidural space (77.8%) and a prominent epidural venous plexus (77.8%). These findings disappeared in all six patients who underwent post-EBP spinal MRI. Dural enhancement and brain sagging were minimum or absent on the cranial MR images of seven patients, although DSSS were obvious in these seven patients. For 23 patients with SIH and 28 healthy volunteers, a diagnostic test using thoracic MRI was performed by 13 experts to validate the usefulness of DSSS. The median sensitivity, specificity, positive-predictive value, negative-predictive value, and accuracy of the DSSS were high (range, 0.913-0.931). CONCLUSIONS: Detection of DSSS on thoracic MRI facilitates an SIH diagnosis without the use of invasive imaging modalities. The DSSS were positive even in patients in whom classic cranial MRI signs for SIH were equivocal or minimal.

DOI： 10.1007/s00701-021-04933-w

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Microvascular decompression (MVD) is the most effective procedure for the long-term management of trigeminal neuralgia (TGN). However, retrospective and single-center studies are inherently biased, and there are currently no prospective, multicenter studies. OBJECTIVE: To evaluate the short- and long-term outcomes and complications in patients with TGN who underwent MVD at specialized Japanese institutions. METHODS: We enrolled patients with TGN who underwent MVD between April 2012 and March 2015. We recorded their facial pain grade and complications at 7 d (short term), 1 yr (mid-term), and 3 yr (long term) postoperatively. RESULTS: There were 166 patients, comprising 60 men and 106 women (mean age 62.7 yr). Furthermore, 105 patients were aged over 60 yr. We conducted neuromonitoring in 84.3% of the cases. The complete pain relief, mortality, and complication rates at the short-term follow-up were 78.9%, 0%, and 16.3%, respectively. Overall, 155 patients (93.4%) completed the long-term follow-up, with the complete pain relief and complication rates of 80.0% and 5.2%, respectively. CONCLUSION: In the hands of experienced neurosurgeons, MVD for TGN can achieve high long-term curative effects. In addition, complications are uncommon and usually transient. Our results indicate that MVD is an effective and safe treatment for patients with TGN, including elderly patients.

DOI： 10.1093/neuros/nyab229

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Elsevier {BV}  

DOI： 10.1016/j.inat.2020.101080

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Language：Japanese   Publisher：株式会社医学書院  

DOI： 10.11477/mf.1436204439

PubMed

Publisher：Neurosurgery  

DOI： 10.1093/neuros/nyaa549

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PubMed

Language：English   Publisher：The Japan Neurosurgical Society  

Seizure clusters (SCs) are acute repetitive seizures with acute episodes of deterioration during seizure control. SCs can be defined as a series of grouped seizures with short interictal periods. Vagus nerve stimulation (VNS) is a treatment option for drug-resistant epilepsy. We present a case where VNS suppressed epileptic SCs, which had persisted for several months. A 13-year-old boy with congenital cerebral palsy and mental retardation had drug-resistant epilepsy with daily jerking movements and spasms in both sides of his body. The seizures were often clustered, and he experienced two sustained SC episodes that persisted for a few months even with prolonged use of continuous intravenous midazolam (IV-MDZ). The patient underwent VNS device placement at the second sustained SC and rapid induction of VNS. Because the tapering of IV-MDZ did not exacerbate the SC, midazolam was discontinued 4 weeks after VNS initiation. Non-refractory SCs also disappeared 10 months after VNS. The seizure severity was improved, and the frequency of seizures reduced from daily to once every few months. The epileptic activity on electroencephalography (EEG) significantly decreased. This case highlights VNS as an additional treatment option for SC. VNS may be a therapeutic option if SC resists the drugs and sustains. Additional studies are necessary to confirm our findings and to investigate how device implantation and stimulation parameters affect the efficacy of VNS.

DOI： 10.2176/nmccrj.cr.2020-0137

PubMed

Language：English   Publisher：The Japan Neurosurgical Society  

Behçet's disease (BD) is a rare chronic inflammatory disease associated with systemic vasculitis. Involvement of the nervous system in BD is called neuro-BD (NBD). Epilepsy related to NBD is uncommon but responds well to anti-epileptic drugs. We present a case of NBD with drug-resistant mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS). The patient presented with headache, dizziness, disorientation, and generalized seizures. Magnetic resonance imaging (MRI) identified pontine lesions. Chronic inflammation was suspected, and steroid pulse therapy improved his symptoms. He relapsed 1 year after onset and was diagnosed with NBD. MRI revealed bilateral mesial temporal lesions, with the right being edematous and the left atrophic. NBD was controlled by steroid and immunosuppressive medication. Three years after the onset of NBD, the patient suffered MTLE, and MRI suggested left hippocampal atrophy. His seizures became drug-resistant and surgical therapy was considered 12 years after NBD onset. Pre-surgical MRI clearly showed left HS. After evaluations, the patient had left anterior temporal lobectomy (ATL) 13 years after NBD onset under stable NBD. The patient was seizure-free for > 2 years after surgery. Surgery will be an effective treatment for drug-resistant MTLE with HS even in patients with NBD, of course the effects of surgical intervention should be considered.

DOI： 10.2176/nmccrj.cr.2020-0218

PubMed

Publisher：Scientific Reports  

DOI： 10.1038/s41598-020-64968-4

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PubMed

Publisher：Epilepsy and Behavior  

DOI： 10.1016/j.yebeh.2020.107255

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Publisher：広島大学医学出版会  

症例は56歳女性で、構音障害を主訴とし、拡散強調画像(DWI)により、右前頭葉病変周囲に高強度領域が描出された。虚血性脳血管障害として治療を行ったところ、3日で症状の鎮静が得られたが、7日後のMRI検査で高強度領域の拡大が観察された。また、ガドリニウム(Gd)造影MRIでは、延髄細静脈に異常に大きな静脈が描出され、髄液中β2-ミクログロブリン(β2MG)濃度は2061μg/ lにまで上昇した。悪性腫瘍との鑑別を要したが、術後23日目のMRIにより、病変部の完全消失とDWI上での高輝度領域減少が認められたことから、本例に観察された発生学的静脈形成異常(DVA)は灌流障害による症候性と判断された。

Language：Japanese   Publishing type：Research paper (scientific journal)  

Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

DOI： 10.4103/ajns.AJNS_266_17

PubMed

Publisher：Epilepsy and Behavior Reports  

DOI： 10.1016/j.ebr.2019.100337

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)  

Publisher：Epilepsia Open  

DOI： 10.1002/epi4.12260

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

Publisher：Acta Neurochirurgica  

DOI： 10.1007/s00701-017-3336-4

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

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Publisher：Neuroradiology Journal  

DOI： 10.1177/1971400916678228

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Publisher：一般社団法人 日本脳神経外科学会  

<p>Given the anatomical proximity of tuberculum sellae meningioma (TSM) to the hypothalamo-pituitary system, pituitary function impairments are of great concern. We retrospectively investigated pituitary function changes following surgery in patients with TSM using pituitary provocation tests (PPTs). Thirty-one patients (27 females and 4 males) with TSM underwent initial transcranial surgery (29 patients) or transsphenoidal surgery (two patients); surgeries were performed carefully to avoid injuring the pituitary stalk. In 24 patients, the PPTs were performed via a triple bolus injection with regular insulin, thyrotropin-releasing hormone (TRH), and luteinizing hormone releasing hormone (LH-RH). Seven patients underwent a quadruple test (growth-hormone-releasing factor, corticotrophin-releasing hormone, TRH, and LH-RH). The preoperative and postoperative target hormone levels of the anterior pituitary were normal in 93.5% and 96.8% of patients, respectively. At least one hormonal axis demonstrated impaired PPT responses in two patients (6.5%) preoperatively and in one patient (3.2%) postoperatively. The growth hormone (GH) response was also well preserved. A compromised GH peak level was only observed in one patient (3.2%) preoperatively. Postoperatively, transient diabetes insipidus and transient hyponatremia were observed in four (12.9%) and eight (25.8%) patients, respectively. No patients needed permanent postoperative hormone replacement. The preoperative pituitary function was well preserved in most patients, including those with large tumors pushing against the pituitary stalk considerably or embedded in it. After careful surgery to avoid damaging the pituitary stalk, pituitary function was preserved. However, transient postoperative hyponatremia occurred in 25.8% of patients; thus, surgeons should pay careful attention to this issue.</p>

DOI： 10.2176/nmc.oa.2017-0079

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PubMed

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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Language：Japanese   Publishing type：Research paper (scientific journal)  

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Responsible for pages：185-194   Language：English

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point　・視床下部と下垂体は,ホルモンを介した綿密な連携で生体の恒常性維持に重要な役割を担っている.・ホルモンデータを解釈する際は,検査値に影響を与える因子を理解しておく必要がある.

Language：Japanese   Publisher：大阪 : メディカ出版  

CiNii Books

Other Link： https://ndlsearch.ndl.go.jp/books/R000000004-I032963961

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point ・適切な薬物治療によって60～70%の患者で発作が消失する.・抗てんかん薬の服用はしばしば長期に及ぶため,発作型だけではなく,副作用を含めた各薬剤の特性と患者のライフサイクルを念頭に置いた薬剤選択を行う.・新規抗てんかん薬は,従来薬に比べて薬物相互作用や長期内服に伴う副作用が軽減している.

Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01228&link_issn=&doc_id=20230130160014&doc_link_id=10.11477%2Fmf.1436204720&url=https%3A%2F%2Fdoi.org%2F10.11477%2Fmf.1436204720&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point　・適切な薬物治療によって60～70%の患者で発作が消失する.・抗てんかん薬の服用はしばしば長期に及ぶため,発作型だけではなく,副作用を含めた各薬剤の特性と患者のライフサイクルを念頭に置いた薬剤選択を行う.・新規抗てんかん薬は,従来薬に比べて薬物相互作用や長期内服に伴う副作用が軽減している.

Language：Japanese   Publisher：(一社)日本定位・機能神経外科学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

脳梁離断術はてんかん性スパズム、強直発作、脱力発作などの転倒する発作で最も有効とされる。今回、非定型欠神発作重積に脳梁離断術が有効だった1例を経験したので報告する。症例は6歳女児。生後Down症候群と診断された。8ヵ月時にWest症候群を発症した。5歳時から強直発作、ミオクロニー発作、非定型欠神発作などの多彩な発作を認めるようになり、Lennox-Gastaut syndrome spectrumと診断した。6歳0ヵ月時には最大で1日100回以上の非定型欠神発作が出現して、脳波検査では発作時、発作間欠時脳波ともに全般性変化を呈した。難治に経過し、薬剤治療による欠神発作重積の抑制は困難と判断して6歳1ヵ月時に脳梁離断術を行った。手術直後から各種発作は消失した。術後の脳波では全般性棘徐波が右前頭部に局在化した。脳梁を介した神経興奮の拡がりが脳梁離断術によって抑制されたことによって、全般発作である非定型欠神発作が抑えられた可能性を考えた。本症例の経験から、特に欠神発作重積が薬剤抵抗性で退行を呈するなどの緊急性を要する症例では、脳梁離断術の適応を積極的に検討すべきと考えた。(著者抄録)

Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01742&link_issn=&doc_id=20230214200003&doc_link_id=10.3805%2Fjjes.40.541&url=https%3A%2F%2Fdoi.org%2F10.3805%2Fjjes.40.541&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_2.gif

J-GLOBAL

Language：English  

BACKGROUND: Rosette-forming glioneuronal tumors (RGNTs) are rare and slow-growing (WHO grade 1); they mainly involve the posterior fossa. We here report a rare RGNT originating from the thalamus; after subtotal removal it remained stable for more than 11 years.
CASE PRESENTATION: A woman in her early 20s consulted us due to a 6-month history of memory difficulties, headaches, and blurred vision. Magnetic resonance imaging (MRI) showed obstructive hydrocephalus and an 18-mm non-cystic third ventricular tumor that arose at the right thalamus. It was isointense on T1-wighted images, highintense on T2-weighted images, and non-enhances T1-weighted images. Through a right trans-ventricular subchoroidal approach we made subtotal resection of the soft tumor, leaving a small remnant attached to the posterior thalamic wall. Histologically, the tumor was composed of an alveolar component that included rosettes surrounding cores of eosinophilic neuropils or small vessels and a solid component resembling pilocytic astrocytoma. The cells composing the rosettes were positive for olig-2, MAP, and synaptophysin. The Ki-67 index was around 1%. Postoperatively her symptoms disappeared, and she commenced her engineering career. MRI performed 11.3 years after the surgery found the absence of recurrence.
CONCLUSION: This RGNT is quite unique because it arose from the thalamus, very rare site from which RGNTs originate, and remained stable for more than 11 years after subtotal resection.

CiNii Books

Language：Japanese   Publisher：(有)科学評論社  

Language：Japanese   Publisher：(有)科学評論社  

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(一社)日本頭痛学会  

Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

Language：English   Publisher：(一社)日本癌学会  

Language：English   Publisher：(一社)日本癌学会  

Language：English   Publisher：(一社)日本癌学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本内分泌学会  

50歳代男性。頭痛の精査目的に施行された頭部MRIで鞍上部腫瘍を認められ、当科に紹介された。MRI所見は鞍上部に最大径17mmの腫瘍性病変を認め、腫瘍は視路に接していた。内分泌学的には明らかな異常は認めず、視野検査でも明らかな障害は認めなかった。無症候性ではあるものの、腫瘍が視路に接しており将来的には症候性となることが予想されたことなどから早期の手術介入の方針とし、開頭経鼻同時手術を施行した。腫瘍は赤色調で周囲脳や硬膜との癒着は目立たなかったが、右の視神経との癒着が強固であった。術中所見から右海綿静脈洞内への腫瘍進展が疑われたため、同部位と視神経周囲は愛護的に操作して腫瘍を摘出し、病理組織所見から傍神経節腫と診断した。術後に全身検索を行い、中枢神経単発の非機能性傍神経節腫と最終診断した。右海綿静脈洞周囲の残存腫瘍が緩徐に増大し、摘出術から17ヵ月後にガンマナイフ照射を行った。

Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J01160&link_issn=&doc_id=20220817460019&doc_link_id=10.1507%2Fendocrine.98.S.HPT_63&url=https%3A%2F%2Fdoi.org%2F10.1507%2Fendocrine.98.S.HPT_63&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(株)メディカ出版  

Language：Japanese   Publisher：(株)メディカ出版  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：日本脳腫瘍病理学会  

Language：Japanese   Publisher：(株)中外医学社  

Language：Japanese   Publisher：(一社)日本小児神経外科学会  

Language：Japanese   Publisher：(一社)日本定位・機能神経外科学会  

J-GLOBAL

J-GLOBAL

J-GLOBAL

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point　・臓器機能や認知機能の低下に対して高齢者総合機能評価(CGA)を行い,治療強度を適切に調整する.・脆弱(unfit)の高齢者患者に対して寡分割放射線照射やテモゾロミド単独療法などが開発され,非劣性が示された.・高齢者グリオーマに対するてんかん治療では,臓器機能や認知機能と薬物代謝に配慮が必要である.

J-GLOBAL

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J-GLOBAL

J-GLOBAL

J-GLOBAL

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J-GLOBAL

J-GLOBAL

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J-GLOBAL

J-GLOBAL

Publisher：(一社)日本てんかん学会  

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

Publisher：(株)メディカルレビュー社  

Publisher：(一社)日本てんかん学会  

シナプス小胞糖タンパク2A(synaptic vesicle glycoprotein 2A:SV2A)は、シナプス小胞に特異的に発現する膜タンパクで、即時放出可能プールのシナプス前膜への融合、プライミング、synaptotagmin-1との相互作用などの、エキソサイトーシス機構を調節することにより、神経伝達物質の放出を制御すると考えられている。抗てんかん薬レベチラセタム(LEV)がSV2Aに結合することが発見されて以来、SV2Aとてんかん発現との関連が注目されてきた。疾患モデルやヒトでのてんかん研究ではSV2A発現が低下するとの報告が多く、てんかんの発現には抑制系のSV2Aが関連していることが推測される。一方で、LEVの作用機序に関する報告は、LEVが興奮系のSV2Aを介して作用することを示唆する。SV2Aは興奮性と抑制性シナプスの両方に存在し、また実験系や背景疾患などによってSV2A発現に相違があるため、その役割を現時点で明確に特定することは難しい。SV2Aの生理的機構や関連する病態の解明に向けて研究の発展が望まれる。(著者抄録)

Publisher：(株)メディカ出版  

Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

Event date： 2012.9

Language：Japanese  

Venue：横浜  

国内学会

Event date： 2012.7

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2012.6

Language：Japanese  

Venue：大分  

国内学会

Event date： 2012.1

Language：Japanese  

Venue：東京  

国内学会

Event date： 2012.1

Language：Japanese  

Venue：東京  

国内学会

Event date： 2011.10

Language：Japanese  

Venue：横浜  

国内学会

Event date： 2011.10

Language：Japanese  

Venue：新潟  

国内学会

Event date： 2011.9

Language：Japanese  

Venue：横浜  

国内学会

Event date： 2010.8

Language：English  

Venue：インドネシア  

国際学会

Event date： 2010.8

Language：English  

Venue：インドネシア  

国際学会

Event date： 2010.4

Language：English  

Venue：鹿児島  

国際学会

Event date： 2010.3

Language：Japanese  

Venue：鹿児島  

国内学会

Event date： 2010.1

Language：Japanese  

Venue：東京  

国内学会

Event date： 2009.10

Language：Japanese  

Venue：弘前  

国内学会

Event date： 2009.10

Language：Japanese  

Venue：東京  

国内学会

Event date： 2009.9

Language：Japanese  

Venue：東京  

国内学会

Event date： 2009.8

Language：English  

Venue：アメリカ合衆国  

国際学会

Event date： 2009.8

Language：English  

Venue：アメリカ合衆国  

国際学会

Event date： 2009.8

Language：English  

Venue：アメリカ合衆国  

国際学会

Event date： 2009.7

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2009.6

Language：Japanese  

Venue：久留米  

国内学会

Event date： 2009.6

Language：English  

Venue：Osaka  

国際学会

Event date： 2009.5

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2009.3

Language：Japanese  

Venue：広島  

国内学会

Event date： 2009.3

Language：Japanese  

Venue：広島  

国内学会

Event date： 2009.2

Language：Japanese  

Venue：福岡  

研究会

Event date： 2008.12

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2008.10

Language：Japanese  

Venue：東京  

国内学会

Event date： 2008.10

Language：Japanese  

Venue：東京  

国内学会

Event date： 2008.7

Language：Japanese  

Venue：鹿児島  

その他

Event date： 2008.7

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2008.7

Language：Japanese  

Venue：鹿児島  

研究会

Event date： 2008.6

Language：Japanese  

Venue：福岡  

国内学会

Event date： 2008.1

Language：Japanese  

Venue：東京  

国内学会

Event date： 2007.12

Language：English  

Venue：アメリカ合衆国  

国際学会

Event date： 2007.11

Language：Japanese  

Venue：福岡  

国内学会

Event date： 2006.9

Language：Japanese  

Venue：石川  

国内学会

Event date： 2006.3

Language：Japanese  

Venue：神奈川  

国内学会

Event date： 2005.10

Language：Japanese  

Venue：北海道  

国内学会

Event date： 2005.8

Language：English  

Venue：フランス  

国際学会

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

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Language：Japanese  

Language：English  

Language：Japanese  

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Language：Japanese  

Language：Japanese  

Language：English  

Language：Japanese  

Language：Japanese  

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Language：Japanese  

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Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：English  

Language：English  

Language：Japanese  

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Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：English  

Language：Japanese  

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Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese