家族性周期性麻痹8例报告和文献复习

目的报告8例儿童家族性周期性麻痹(FPP)病例,复习相关文献,以提高对其认识。方法回顾性总结8例FPP儿童的家系遗传特性、临床特征、辅助检查结果及治疗,分析其病因和诊断。结果6例为家族性低血钾型周期性麻痹,具有常染色体显性遗传的特征;发作时血清钾1.9~2.8 mmol/L,平均2.4 mmol/L;心电图出现U波等低血钾改变;其中1例血糖减低。另2例属于家族性正常血钾型周期性麻痹,也有常染色体显性遗传特性;发作时血清钾正常;1例血糖降低。8例甲状腺功能、肾功能和肌电图均正常。结论FPP为一组少见遗传性的骨髂肌离子通道病,根据家系遗传学特征、临床表现和相关辅助检查可确诊。     

膈神经与面神经吻合的解剖学研究

目的:探讨膈神经移位与面神经干吻合的解剖学基础。方法:解剖11具22侧成年尸体头颈部,观察并测量:①膈神经根与面神经总干腮腺区分叉处之间的距离;②膈神经干在颈部可利用长度;③面神经总干腮腺区分叉处游离至水平半规管下缘可利用长度,面神经总干腮腺区分叉处游离至水平半规管下缘较游离至茎乳孔处增加的面神经干可利用长度;④面神经总干腮腺区分叉处至水平半规管下方切断处膈神经干及面神经干神经移位后并列放置重合长度。结果:①膈神经根与面神经总干腮腺区分叉处之间的距离为(7.2±0.9)cm。②膈神经起点至锁骨下静脉上缘平面为(7.2±1.6)cm。③面神经总干腮腺区分叉处至水平半规管下方切断处的长度2.7~3.5 cm,较面神经干自茎乳孔下方切断增加1.0~1.5 cm。④神经移位后重合长度为0.4~1.8 cm。结论:在锁骨下静脉上缘平面切断20侧标本膈神经可与自乳突内切取的面神经干无张力吻合,两侧膈神经不能与自乳突内切取的面神经干无张力吻合。     

无锡市控制和消灭脊髓灰质炎现状

目的 通过对无锡市控制与消灭脊髓灰质炎（下称脊灰）的现状分析，提出要继续保持高水平的急性驰缓性麻痹（AFP）病例监测系统敏感性，特别要作好流动儿童常规免疫和强化免疫工作，及时发现、应对各种野病毒输入情况，才能巩固无脊灰成果。方法 根据对该市人群免疫水平、疫苗免疫原性监测及15岁以下儿童的（AFP）病例监测系统的敏感性评价等分析该市控制与消灭脊灰的现状。结果 控制与消灭脊灰的现状分析结果表明，人群有较好的免疫水平，AFP监测系统敏感；但是，免疫空白的外来儿童是控制和消灭脊灰的薄弱环节，应引起高度重视。结论 该市的控制和消灭脊灰的现状表明，从上世纪印年代使用脊灰疫苗（OPV），特别是实施儿童计划免疫以来，接种率和人群免疫状况维持和稳定在较高水平。上世纪90年代脊灰发病已得到有效控制，已连续15a无脊灰野毒病例报告。要继续保持高水平的AFP病例监测系统敏感性，特别要作好流动儿童常规免疫和强化免疫工作，及时发现和应对野病毒的输入，才能巩固无脊灰成果。     

河南省1999～2000年急性弛缓性麻痹病例肠道病毒感染流行病学分析

河南省 1999～ 2 0 0 0年从 10 87例急性弛缓性麻痹 (AFP)病例粪便标本中 ,共分离出 92株脊髓灰质炎 (脊灰 )病毒 (PV)和 70株非脊灰肠道病毒 (NPEV) ,分离率分别为 8 4 6 %和 6 4 4 %。Ⅱ型为PV的主要型别 ,共 4 5株 ,占4 8 91%。NPEV中埃可 (ECHO)病毒 6 5株 ,占 92 86 % ,病毒型别以ECHO11、7、12 为优势血清型。柯萨奇A组病毒(Cox A) 1株 ,柯萨奇B组病毒 (Cox B) 4株。检出PV多在冬春季 ,NPEV流行高峰期在 8～ 10月 ;感染均以小年龄儿童为主。多数肠道病毒 (EV)感染者伴有发热、腹泻或肌肉疼痛等症状。 38例发病 6 0d后仍残留麻痹 ,其中多数为PV感染 ,且未完成全程免疫。EV感染病例临床诊断以格林 巴利综合征和NPEV感染为主。在无脊灰证实以后 ,应该加强对PV和NPEV的监测和研究     

针刺治疗假性球麻痹

本文从头针和舌针治疗假性球麻痹阐述了针刺是治疗该病的重要方法之一。针刺疗法作为治疗本病的主要手段,以局部取穴为主,远端取穴为辅,将近治与远治相结合,是治疗本病的重要方法。     

Rhino-oculo Cerebral Mucormycosis with Multiple Cranial Nerve Palsy in Diabetic Patient: Review of Six Cases

AIM of the study is to evaluate etiopathogenesis role played by predisposing conditions (Diabetes, Immunosupression), precipitating factors (trauma/surgery/ketoacidosis) and possible role of occupational hazard is discussed briefly. Clinical presentation and management of patients presenting with rhinoorbitocerebral mucormycosis is discussed. The prospective study of patient undergoing treatment of mucormycosis] without control Setting was done in ENT Deptt. NSCB Medical College, Jabalpur (tertiary referral centre of mid India). Subject were patients presenting with invasive fungal rhino sinusitis presenting with orbital involvement and cranial nerve palsies undergoing treatment. The detailed history, clinical examination including cranial nerve examination, blood test, CTscan and biopsy. Nasal endoscopy, CWL surgery and medical management with 6 month follow up. All six patients were diabetic when evaluated on presentation. Two patients had ketoacidosis. Four had history of surgery in recent past. Blood stained nasal discharge and dysaesthesia of face are early warning signs. They had necrotic lesion in nose and infraorbital area with 2, 3, 4, 5, 6 and 7 cranial nerve involvement. Skin necrosis/Mucosal necrosis, facial palsy and diplopia signify advanced disease. Altered sensorium, panopthalmitis & diabetes complicated with ketoacidosis signify bad prognosis. In present study two patients with advanced disease, altered sensorium and ketoacidosis succumbed within 72 hours in spite of anti fungal medicine. Of the four surviving patients, all responded well to treatment but had residual sixth and seventh nerve palsy. One patient defaulted in diabetes control & had recurrence after 6 months. Early diagnosis, aggressive surgical debridement and proper management of underlying metabolic abnormality along with amphotericin B can avert the bad prognosis of rhinoorbitocerebral mucormycosis.     

Functional Analysis of Dynamin Isoforms in Drosophila Melanogaster

Dynamin and dynamin-like proteins are required for endocytosis. synaptic vesicle recycling and membrane trafficking. From the shibire locus in Drosophila melanogaster, six different isoforms of dynamin are generated by alternative splicing. However, the roles of the individual isoforms in cellular processes are unknown. To investigate functional differences among the dynamin isoforms, transgenic lines were generated that individually expressed each of 3 different isoforms under UAS^GAL4 control. The expression of the isoforms was controlled by neural promoter (elav)-driven GAL4, or by a shibire-promoter driven GAL4 transgene. Reporter gene expression indicated that the shi promoter is active during embryogenesis, and in larvae, pupae, and adults in a pattern consistent with normal dynamin expression. To assay for the ability of dynamin isoforms to function in vivo, the isoforms expressed via these GAL4 drivers were tested for the ability to rescue shibire phenotypes. When expressed at very high levels all individual isoforms tested rescued the temperature-sensitive paralytic phenotype of shl^ls2 flies; however, this rescue was partial, suggesting that no single tested isoform is sufficient for synaptic vesicle recycling in vivo. When tested for ability to rescue lethality induced by heat-pulsing larvae during development, shi- promoter driven expression of individual isoforms conferred significant resistance to heat treatment during larval development. However, all 3 isoforms were unable to rescue the lethality of shi^12-12B mutants which are severely hypomorphic (or null) for shibire function. Taken together. these observations suggest that individual shibire isoforms have specific molecular activities in vivo.     

杨介宾教授针灸治疗面瘫经验撷萃

从明病因、定病位,述病机、详辨证,依分期、异治法3方面阐述了杨介宾教授治疗面瘫的特色经验。重点阐述杨介宾教授完全运用中医针灸理论治疗面瘫的观点,强调依照中医经典治疗本病具有疗效确切、快速治愈等多种优点,具有很好的借鉴意义。