Giant axonal neuropathy: central abnormalities demonstrated by evoked potentials

Previous studies of giant axonal neuropathy have reported clinical and pathological findings that indicate involvement of the central nervous system. We studied 3 boys with giant axonal neuropathy, who were 14 to 16 years of age, using auditory, visual, and somatosensory evoked potentials. Absence of waveforms and prolongation of peak and interwave latencies were found. Abnormalities were noted in all modalities. The auditory brainstem evoked response in particular indicated a significant increase in brainstem conduction time. These studies add clinical neurophysiological confirmation of the central nervous system involvement in this disorder and may also provide a means of quantitative evaluation of its progression.     

偏头痛大鼠脑内5-羟色胺1F和诱导型一氧化氮合酶基因的表达变化及针刺的干预效应

目的:前期实验已证实针刺治疗偏头痛疗效优越。观察针刺对偏头痛大鼠脑内5-羟色胺1F和诱导型一氧化氮合酶mRNA表达的调控作用。方法:实验于2005-11/2006-05在中南大学湘雅医院中西医结合研究所实验室完成。①选用SD大鼠40只,按随机数字表法分为4组(n=10),除正常对照组外,其余3组均复制大鼠偏头痛模型。模型对照组只造模,不作其他处理;针刺治疗组造模后进行针刺;针刺预防组针刺后造模电刺激20min。针刺方法:针刺大鼠双侧太冲、阳陵泉穴20min。采用疏密波,电流强度0.3～0.6mA,留针20min,1次/d,共5次。②实验完毕后取脑干及三叉神经节匀浆,采用反转录-聚合酶链反应法测定5-羟色胺1F和诱导型一氧化氮合酶mRNA表达。结果:进入结果分析正常对照组10只,模型对照组、针刺治疗组、针刺预防组各9只,共脱失3只。①与正常对照组比较,模型对照组大鼠诱导型一氧化氮合酶mRNA表达显著增强(P<0.01),5-羟色胺1FmRNA表达显著减弱(P<0.01)。②与模型对照组比较,针刺预防组和针刺治疗组诱导型一氧化氮合酶mRNA表达明显减弱(P<0.01),5-羟色胺1FmRNA表达显著增强(P<0.01)。结论:针刺调控5-羟色胺1F和诱导型一氧化氮合酶mRNA的表达可能是针刺防治偏头痛的分子机制。     

Retrorenal colon: implications for percutaneous diskectomy

It has been recommended that computed tomography (CT) with the patient prone be performed in every patient undergoing percutaneous diskectomy; this would enable detection of a retrorenal location of the colon, which could interfere with the percutaneous procedure. In this evaluation of 346 prone CT studies, only one patient (0.29%) was found to have retrorenal or retropsoas bowel that would have been perforated at diskectomy. Because of this extremely low prevalence, the performance of prone CT in every patient undergoing percutaneous lumbar diskectomy is not believed to be necessary.     

Use of triple-lumen subclavian catheters for administration of total parenteral nutrition.

This study evaluated the safety of triple vs single-lumen catheters in intravenous nutrition. Patients who were judged likely to benefit from a triple-lumen catheter were randomized to receive either a single-lumen catheter, with additional peripheral or central venous access as needed, or a triple-lumen catheter. All patients were at increased risk of catheter-related infection because of one or more of the following conditions: > 60 years of age, breakdown of skin integrity, severe underlying illness, diagnosis of acute pancreatitis, recent head or neck surgery, or presence of a preexisting infection. Patients were excluded who had neutropenia, were immunosuppressed, had body burns > 40%, or had contaminated wounds in the subclavicular area. Of 204 patients entered between June 1989 and November 1991, 177 completed the required > or = 7 days of therapy. Seventy-eight of these patients were randomized to a single-lumen catheter and 99 to a triple-lumen catheter. Catheters were inserted and maintained by the Nutrition Support Team. Dressings were monitored daily and changed weekly using a bio-occlusive dressing. When parameters were met for a possible septic episode, simultaneous peripheral and central catheter blood cultures were obtained using the Isolator method. Catheter-related sepsis was considered present if the colony count from a central catheter lumen was > or = 5 times that of the peripheral blood. The incidence of catheter-related sepsis for single-lumen catheters was 2.6% (2 of 78) compared with 13.1% for triple-lumen catheters (13 of 99) (p < .01). No correlation was found with the number of insertion attempts, catheter days, or patient's age.(ABSTRACT TRUNCATED AT 250 WORDS)     

King syndrome: a genetically heterogenous phenotype due to congenital myopathies.

We report on a patient with myopathy, kyphoscoliosis, joint contractures, and a facial appearance consistent with King syndrome. Unlike other reported cases, our patient had hyperextensible joints, normal stature, and pectus excavatum. The cardiac ventricles, aorta, and pulmonary artery were dilated. Malignant hyperthermia did not occur under anaesthesia although there was a transient increase in CK levels. Muscle bulk and tone were significantly decreased but collagen and elastin fibres were normal. The variable clinical presentation of King syndrome suggests that the manifestations are caused by different congenital myopathies and in all cases there is probably an increased risk of malignant hyperthermia.     

A monoclonal antibody to a parasympathetic neurotrophic factor causes immunoparasympathectomy in mice

A monoclonal antibody capable of blocking the biological activity of the ciliary neurotrophic factor purified from bovine cardiac muscle has been produced. This antibody, when administered perinatally to mice, causes a failure of the normal development of the parasympathetic innervation of the iris as determined by assay for the activity of the cholinergic marker enzyme choline acetyltransferase. The same treatment has no effect on the adrenergic neuronal marker, tyrosine hydroxylase. This immunoparasympathectomy suggests that the ciliary neurotrophic factor has an essential role in regulating the development of the mammalian parasympathetic nervous system.     

AL01PACIFIC ISLANDS PROJECT – PAST PRESENT AND FUTURE

The Pacific Islands Project began in 1995 and in its early years had a focus on providing specialist services that were not available in the 10 island nations visited. In 2002 Nauru was added and PIP Phase III will end its 9 month bridging/extension phase in September 2007. During the last 12 years Fiji School of Medicine has commenced a postgraduate medical training program in surgery similar to that has been in existence in PNG since 1975. There are now a growing number of Pacific‐trained surgeons who can select suitable cases, do some of the surgery, and supervise the postoperative care. Increasingly visiting teams have focused on transferring skills and building local capabilities (capacity building). The RACS, the Project Director and the speciality coordinators have managed the first three phases of the project in Australia. Phase III had on‐going evaluation by an internal RACS committee under the chairmanship of Professor Hamish Ewing. AusAid also externally reviewed the project late in 2006. That review was generally complimentary as to what has been achieved but also points to some new goals for the future. At the time of writing this abstract the future direction of PIP is yet to be decided and designed. This will be done mid 2007. However, it is to be hoped there will be a new program, focused on capacity building, that is managed in the Pacific and employs the skills of Pacific Island Specialists wherever possible. RACS is likely to continue to play an important role in sourcing visiting specialists, organising training positions, arranging courses. We have much expertise to offer but there is no longer any need for us to set the agendas.     

Myoblast transfer in duchenne muscular dystrophy

One biceps muscle of 8 patients with Duchenne muscular dystrophy was injected at 55 sites with a total of 55 million viable, purified, and contamination-free normal myoblasts (myoblast transfer). The other biceps of each patient was injected with a placebo to serve as a control. The procedure was blinded to the patients, parents, and investigators. Myoblasts derived from a biopsy specimen of the fathers were cultured and purified under strict conditions and carefully screened for microbial contamination. All patients received cyclophosphamide for immunosuppression for 6 or 12 months. No serious complications were observed after myoblast transfer, indicating that the procedure is safe. The overall therapeutic efficiency of myoblast transfer was poor as judged by the results in maximal voluntary force generation, dystrophin content of the muscle, magnetic resonance imaging of the muscle, and the lack of donor-derived DNA and dystrophin messenger RNA in the injected muscle. An improved efficiency of the take of myoblasts might be achieved by using younger cells and injecting the myoblasts with a myonecrotic agent (to increase the prevalence of regeneration) and a basal laminal fenestrating agent.