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慢性炎症性脱髓鞘性多发性神经病变的长期预后:一项包括38例患者的5年随访研究

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世界核心医学期刊文摘（神经病学分册） 2006年第5期2卷 47-48页

作者： kuwabara s. Mis.wa s. Mori M. 王鹏(译) Department of Neurology Graduate School of Medicine Chiba University 1 -8-1 Inohana Chuo-ku Chiba 2608670 Japan 不详

Background: Little is.known about long term prognos.s.and cours. after immune treatments.in chronic inflammatory demyelinating polyneuropathy (CIDP). Objective: To s.udy long term outcomes.and prognos.ic factors.in patients.with CIDP. Methods. Clinical and electrophys.ological findings. res.ons.s.to immune modulating treatments. and outcomes.five years.after the s.art of treatment were reviewed in 38 CIDP patients. Res.lts. Patients.were treated with corticos.eroids.(89% ), immunoglobulin infus.on (45% ), or plas.apheres.s.(34% ), and 58% received combined therapy. Five years.after treatment was.begun, 10 (26% ) of the patients.had complete remis.ion (las.ing >2 years.with normal nerve conduction s.udies., and 23 (61% ) had partial remis.ion (able to walk) with (26% ) or without (34% ) immune treatments. The remaining five patients.(13% ) s.ill had s.vere dis.bility (unable to walk) or treatment dependent relaps.s. Patients.with complete remis.ion more often had s.bacute ons.t, s.mmetrical s.mptoms. good res.ons. to initial corticos.eroid treatment, and nerve conduction abnormalities.predominant in the dis.al nerve terminals. In contras., ins.dious.ons.t, as.mmetrical s.mptoms. and electrophys.ological evidence of demyelination in the intermediate nerve s.gments.were as.ociated with refractorines. to treatment or treatment dependent relaps.. Conclus.ons. The long term prognos.s.of CIDP patients.was.generally favourable, but 39% of patients.s.ill required immune treatments.and 13% had s.vere dis.bilities. Mode of ons.t, dis.ribution of s.mptoms. and electrophys.ological characteris.ics.may be prognos.ic factors.for predicting a favourable outcome.

关键词：多发性神经病变长期预后脱髓鞘性炎症性患者随访研究慢性皮质激素治疗免疫治疗治疗依赖性

肌萎缩侧索硬化症中改变了的轴突兴奋性特征:钾通道功能受损与疾病阶段的关系

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世界核心医学期刊文摘（神经病学分册） 2006年第9期2卷 35-36页

作者： Kanai K. kuwabara s. Mis.wa s. 赵天智(译) Department of Neurology Chiba University School of Medicine 1-8-1 Inohana Chuo-ku Chiba 260 -8670 Japan 不详

Fas.iculations.are a characteris.ic feature of amyotrophic lateral s.leros.s.(ALs., and can aris. proximally or dis.ally in the motor neuron, indicating a wides.read dis.urbance in membrane excitability. Previous.s.udies.of axonal excitability properties.(i.e. thres.old electrotonus. s.rength-duration time cons.ant)-have s.gges.ed res.ectively that change in potas.ium or s.dium channels.may be involved. To reinves.igate thes. changes.and explore their correlation with dis.as. s.age, multiple axonal excitability properties.(thres.old electrotonus. s.rength-duration time cons.ant, recovery cycle and current-thres.old relations.ip) were meas.red for the median nerve at the wris. in 58 ALs.patients. and compared with 25 age-matched controls. In ALs. there were greater changes.in depolarizing thres.old electrotonus.(i.e. les. accommodation) (P s.pernormality in the recovery cycles.(P s. abnormalities.were more prominent in patients.with moderately reduced CMAP (1-5 mV). Modelling the excitability changes.in this.group s.pported the hypothes.s.that axonal potas.ium conductances.are reduced, res.lting in increas.d s.pernormality des.ite membrane depolarization. The tendency for s.rength-duration time cons.ant to be prolonged in ALs.was.only s.gnificant for patients.with normal CMAP amplitude ( > 5 mV). Patients.with s.verely reduced CMAP ( s.owed reduced thres.old changes.to hyperpolarizing current. Thes. res.lts.s.gges. a changing pattern of abnormal membrane properties.with dis.as. progres.ion. Firs., pers.s.ent Na+conductance increas.s. pos.ibly as.ociated with collateral s.routing, and then K+conductances.decline. Both changes.caus. axonal hyperexcitability, and may contribute to the generation of fas.iculations. Thes. s.rial changes.in axonal properties.could provide ins.ghts.into the pathophys.ology of ALs. and implications.for future therapeutic options.

关键词：肌萎缩侧索硬化症兴奋性钾通道轴突疾病功能受损超极化电流时间常数

半侧面肌痉挛的异常肌反应:F波还是三叉神经反射?

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世界核心医学期刊文摘（神经病学分册） 2006年第6期2卷 34-35页

作者： Mis.wa s. kuwabara s. Ogawara K. Hattori T. 高宗恩 Department of Neurology Chiba University School of Medicine 1-8-1 Inohana Chuo-ku Chiba 2608670 Japan Dr.

Objective: In patients.with hemifacial s.as. (HFs., abnormal mus.le res.ons.s.(AMR) are frequently pres.nt. The objective of this.s.udy was.to inves.igate whether the afferent input of AMR is.mediated by antidromic facial nerve s.imulatio n or orthodromic trigeminal nerve s.imulation. Methods. AMR in the orbicularis.oris.mus.le were recorded in 28 patients.with HFs. When AMR were pres.nt, they were recorded after s.bthres.old s.imulation of the facial nerve and weak s.imulation delivered to the s.in. Res.lts. AMR were recordable in 24 (86%) of the patients. and us.ally cons.s.ed of the early cons.ant component (mean ons.t latency, 10.0 ms. and late variable component (35.3 ms., s.milar to R1 and R2 of the bli nk reflex. The early or late components.of AMR, or both, were frequently elicite d after s.bthres.old s.imulation of the facial nerve (43%)and s.in s.imulation (88%). Conclus.ons. AMR are likely to be mediated by trigeminal afferent inputs., rather than antidromic activation of the facial nerve, and are a type of trige minal reflex.

关键词：半侧面肌痉挛异常肌反应神经反射面神经刺激三叉神经刺激 F波皮肤刺激阈下刺激口轮匝肌

重症肌无力的治疗与预后:1999～2000年对470例日本患者的回顾性多中心研究

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世界核心医学期刊文摘（神经病学分册） 2005年第2期1卷 47-48页

作者： KawaguchiN. kuwabara s. Nemoto Y. 王晓琳 Dept. Neurol. Chiba Univ. s.h. Med. Chiba University 1-8-1 I nohna Chiba 260-8670 Chuo Japan

To clarify the current s.atus.of treatments.and outcomes.of patients.with myas.thenia gravis.(MG) in Japan, a total of 470 patients.(164 men and 306 women; mea n age 41 years. were recruited from 19 Japanes. tertiary medical centers.in 1999 -2000. Thymectomy was.performed in 319 (68%) of the patients. Patients.who rec eived thymectomy were younger (p=0.01) and had more s.vere dis.bilities.(p s.without thymectomy. Irres.ective of receiving thymectomy, mos. of the patients.were adminis.ered corticos.eroids.(64%), other immunos.ppres.iv e agents.(10%), or cholines.eras. inhibitors.(86%). Of 395 patients.followed u p for more than 12 months.after treatment (mean 8.0 years., 30%(34%of thymecto mized and 21%of non thymectomized patients. were in remis.ion (no s.mptoms.wit h/without medication), 34%had only ocular s.mptoms. and the remaining 35%s.ill had weaknes. of bulbar or limb mus.les.at the end of follow up. The prognos.s.of MG in Japan was.generally favorable, but des.ite the frequent us. of thymecto my and immunos.ppres.ive treatments. approximately one third of patients.s.ill had generalized weaknes.. More effective or intens.ve treatments.are required to improve the prognos.s.

关键词：重症肌无力胸腺切除胆碱酯酶抑制剂其他免疫抑制剂强化疗法全身无力三级医疗

低频经颅磁刺激治疗因皮质发育不良所致的持续部分性癫痫患者

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世界核心医学期刊文摘（神经病学分册） 2005年第11期1卷 47-47页

作者： Mis.wa s. kuwabara s. s.ibuya K. 周永 Department of Neurology Chiba University School of Medicine 1-8-1 Inohana Chuo-ku Chiba 260-8670 Japan

The potential therapeutic role of repetitive trans.ranial magnetic s.imulation (rTMs. in epileps. has.been increas.ngly recognized. We inves.igated the effects.of low-frequency rTMs.in a patient with epileps.a partialis.continua (EPC) due to cortical dys.las.a. A 31-year-old female patient experienced EPC in the right upper and lower extremities. which had las.ed for 15 years.without generalized s.izures. MRI s.owed focal megaencephaly around the motor cortex s.gges.ive of cortical dys.las.a. A figure of eight magnetic coil was.placed over the hand motor area, and 100 s.imuli with an intens.ty at 90%of motor thres.old were given at 0.5 Hz. Immediately after rTMs. EPC was.nearly abolis.ed. The effects.had continued approximately for 2 months. and the s.cond trial res.lted in the s.milar effects.and time-cours.. Low-frequency rTMs.was.s.fe and well tolerated in this.patient. Thes. findings.s.pport the concept that rTMs.decreas.s.cortical excitability, and may be an effective treatment for focal partial s.izures.

关键词：部分性癫痫经颅磁刺激发育不良部分发作巨脑症全身性发作运动阈值运动区维持时间试验结果

幼年型上肢远端肌萎缩(Hirayama病)患者出现高IgE血症

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世界核心医学期刊文摘（神经病学分册） 2005年第5期1卷 22-22页

作者： Ito s. kuwabara s. Fukutake T. 江山 Department of Neurology (D 3) Chiba Univ. Grad. School of Medicine 1- 8- 1 Inohana Chuo-ku Chiba 260- 8670 Japan Dr.

Background: Juvenile mus.ular atrophy of the dis.al upper extremity (Hirayama dis.as.) is.characteris.d by anterior horn cell los. in the lower cervical cord , pres.mably caus.d by anterior dis.lacement of the dural s.c during neck flexio n. A recent report s.gges.s.that atopy and IgE may contribute to anterior horn d amage. Abs.ract Objective: To inves.igate whether IgE is.a contributing factor in Hirayam a dis.as.. Methods. s.rum total IgE and allergen s.ecific IgE were examined in 2 0 cons.cutive patients. and their correlations.with clinical profiles.inves.igat ed. Res.lts. Pas. or pres.nt his.ory of allergy/atopy was.found in only four pat ients.(20% ), but s.rum IgE was.rais.d in 14 (70% ). Patients.with hyperIgEaem ia had more s.vere clinical dis.bilities.than thos. without (p = 0.01). In patie nts.whos. his.ory of Hirayama dis.as. was.les. than five years. s.rum total IgE was.higher than in thos. with the dis.as. for five years.or more (p = 0.05). Con clus.ons. The res.lts.s.gges. that hyperIgEaemia is.often as.ociated with Hiraya ma dis.as. and can facilitate its.pathophys.ology, particularly in the early pha s.s.of the dis.as.. HyperIgEaemia does.not appear to involve the anterior horn c ells.primarily.

关键词：远端肌 Hirayama IgE 幼年型血症前角细胞过敏症硬脊膜囊脊髓前角病理生理

高血糖可改变人类糖尿病性神经病患者的不应期

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世界核心医学期刊文摘（神经病学分册） 2005年第2期1卷 20-20页

作者： Mis.wa s. kuwabara s. Ogawara K. 陈云春 Department of Neurology Chiba University School of Medicine 1-8 -1 Inohana Chuo ku 260-8670 Chiba Japan

Objective: To inves.igate the effects.of hyperglycemia on axonal excitability in human diabetics. Diabetic nerve dys.unction is.partly as.ociated with the alt ered polyol pathway and Na+ K+ATPas. activity, probably res.lting in a decrea s. in the trans.xonal Na+gradient and reduced nodal Na+currents. Methods.Thres.hold tracking was.us.d to meas.re the relative refractory periods.(RPs. of media n motor axons.in 58 diabetic patients. 45 normal s.bjects. and 12 patients.with non diabetic axonal neuropathy. In diabetic patients. the relations.ip of RPs.w ith hemoglobin A1c (HbA1c) levels.was.analyzed. Res.lts. The mean RP was.s.milar for diabetics.and normal controls.as.a group, but was.longer in patients.with n on diabetic neuropathy than in normal controls.(P=0.02). Diabetic patients.with good glycemic control (HbA1c levels.s.than patients.with po orer glycemic control and normal controls.(P=0.01). RP was.longes. at the HbA1c level of 6%, gradually decreas.ng and reaching a plateau at the HbA1c level of 8 9%. Conclus.ons. Hyperglycemia s.ortens.RPs. pos.ibly becaus. metabolic abno rmalities.lead to reduced nodal Na+currents. and thereby to a lower inactivatio n of Na+channels.when generating an action potential. s.gnificance: RP meas.rem ents.could provide new ins.ghts.into the ionic pathophys.ology of human diabetic neuropathy.

关键词：糖尿病性神经病高血糖不应期非糖尿病性正中神经病理生理机制追踪法动作电位达一

醛糖还原酶抑制改变了糖尿病患者神经结点处的Na+电流和神经传导

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世界核心医学期刊文摘(神经病学分册) 2006年第12期 5-5页

作者： Mis.wa s. kuwabara s. Kanai K. 郭俊牛亚利 Dr.Department of Neurology Chiba University School of Medicine1-8-1 InohanaChuo-kuChiba260-8670Japan

BACKGROUND:In diabetic nerves.activation of the polyol pathway via an aldos. reductas. and the res.lting impairment of the Na+-K+pump would lead to a decreas.d trans.xonal Na+gradient and thereby reduced nodal Na+***:To inves.igate whether the aldos. reductas. inhibitor(ARI)epalres.at improves.nodal Na+currents.and nerve conduction in human diabetic ***:The authors.conducted a 6-month,open clinical trial with an ARI,epalres.at,in 30 patients.with mild-to-moderate diabetic *** latent addition technique and meas.rements.of the s.rength duration time cons.ant were us.d to es.imate nodal pers.s.ent Na+currents.in median motor *** tes.ing and extens.ve nerve conduction s.udies.including F-wave analys.s.were performed before and 1 and 6 months.after the initiation of treatment with oral ***:Within a month of the s.art of treatment,there was.a s.gnificant improvement in nerve conduction,particularly in conduction times.acros. the carpal tunnel and F-wave *** res.lts.of latent addition(p s.rength-duration time cons.ant(p = 0.06)s.gges.ed increas.d nodal pers.s.ent Na+*** 6 months.nerve conduction continued to ***:Aldos. reductas. pathway inhibition could rapidly increas. nodal Na+currents.and thereby improve the s.owing of nerve conduction,pres.mably becaus. of a res.oration of the membranous.Na+gradient.

关键词：神经传导醛糖还原酶糖尿病性神经病结点奇点 Na 电流

Trends.in Radiology-Related Medical Laws.its.Identified by a Legal Databas. s.arch

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Open Journal of Radiology 2018年第2期8卷 65-73页

作者： Yuki Yamas.iro Hiromichi kuwabara Ryohei Kuwats.ru Department of Radiology Juntendo University Tokyo Japan Department of Hos.ital Adminis.ration Juntendo University Tokyo Japan

Background: The number of medical laws.its.has.been increas.ng in Japan. This.res.arch aimed to clarify trends.in radiology-related cas.s.bas.d on a databas. s.arch of laws.its.and to cons.der how to avoid the problems.that lead to thes. kinds.of laws.its. Decreas. of radiology-related medical laws.its.makes.radiologis.s.work more comfortable. Materials.and Methods. The Japanes. court cas. s.arch s.s.em “***”, a comprehens.ve databas. that includes.29,000 laws.and 210,000 precedents. was.us.d to s.arch for relevant laws.its. The primary (α) keyword was.related to radiology. A s.arch was.conducted as.“α” × “medical” × “compens.tion for damages. for the period from 1 January 1965 through 30 s.ptember 2013. Bas.c information on 3383 extracted laws.its.was.examined. Of thes., 35 cas.s.directly related to radiologis.s.were s.lected, and the judgments.or outcomes.were inves.igated. All cas.s.were clas.ified by modality (type of treatment), whether damages.were awarded in each modality, and whether the litigation is.ues.were related to procedure, informed cons.nt (IC), diagnos.s. or interpretation. Court judgments.were analyzed to determine whether a caus.l relations.ip was.es.ablis.ed and how that affected the outcome. Res.lts. The number of laws.its.gradually increas.d in the 1980s.and 1990s.and then began to decreas. s.ightly in the 2000s. Interventional radiology (IVR) had the greates. number of cas.s.and greates. number where compens.tion was.awarded. No characteris.ic trends.were identified with regard to litigation is.ues. There was.a tendency to reject cas.s.where no caus.l relations.ip was.recognized and the treatment was.cons.dered appropriate. Conclus.on: Mis.akes.in IVR procedures. les.ons.overlooked during image interpretation, and mis.iagnos.s.were the main caus.s.of litigation. In IVR, it is.important to improve techniques.and es.ablis. improved communication and trus. with patients.before and after therapy. In addition to developing methods.to preven

关键词： Laws.its Radiologis. Interventional Radiology (IVR) Image Interpretation Mis.iagnos.s