Case Report: Second case of a successful pregnancy in maternal isovaleric acidaemia

Summary: A female patient with isovaleric acidaemia had a successful outcome from pregnancy.     

A rapid and reliable procedure to localize subdural electrodes in presurgical evaluation of patients with drug-resistant focal epilepsy.

OBJECTIVES: To evaluate a novel method for localization of subdural electrodes in presurgical assessment of patients with drug-resistant focal epilepsy. METHODS: We studied eight consecutive patients with posterior epilepsy in whom subdural electrodes were implanted for presurgical evaluation. Electrodes were detected on post-implantation brain CT scans through a semiautomated procedure based on a MATLAB routine. Then, post-implantation CT scans were fused with pre-implantation MRI to localize the electrodes in relation to the underlying cortical structures. The reliability of this procedure was tested by comparing 3D-rendered MR images of the electrodes with electrode position as determined by intraoperative digital photography. RESULTS: In each patient, all electrodes could be correctly localized and visualized in a stereotactic space, thus allowing optimal surgery planning. The agreement between the procedure-generated images and the digital photographs was good according to two independent raters. The mean mismatch between the 3D images and the photographs was 2 mm. CONCLUSIONS: While our findings need confirmation on larger samples including patients with anterior epilepsy, this procedure allowed to localize subdural electrodes and to establish the spatial relationship of each electrode to the underlying brain structure, either normal or damaged, on brain convessity, basal and medial cortex. SIGNIFICANCE: Being simple, rapid, unexpensive, and reliable, this procedure holds promise to be useful to optimize epilepsy surgery planning.     

A case of acute heart failure associated with Guillain-Barré syndrome

Abstract Guillain-Barré syndrome (GBS) is a disease of the peripheral nervous system, which is caused by aberrant immune responses directed against some components of peripheral nerves. GBS is rarely accompanied by cardiovascular involvement. We describe a case of acute neuropathy complicated by sudden heart failure and left ventricular dysfunction which had a presumably neurogenic origin. Pathogenesis of acute heart failure is probably due to transitorial stunned myocardium and neurogenic cardiac injury. We show a rare case of transitorial and acute cardiac dysfunction by echocardiography and laboratory markers of heart failure.     

Abnormal spinal interactions from hand afferents to forearm muscles in writer's cramp.

OBJECTIVE: Spinal reflexes from hand to wrist muscles were investigated in writer's cramp. METHODS: Stimulus-triggered rectified EMG averages after ulnar nerve and cutaneous stimulation, in wrist flexors and extensors during tonic contraction, were compared in 18 controls and 19 patients. RESULTS: On the patient dystonic side, ulnar-induced EMG suppression was decreased in wrist extensors, and facilitation in wrist flexors modified dependent on the dystonic wrist posture during writing. No change was found on the patient non-dystonic side. Cutaneous stimulation increased wrist flexor EMG on both sides of the patients with normal wrist posture during writing, but had no effect in controls and patients with abnormal wrist posture. CONCLUSIONS: Comparison between cutaneous and mixed nerve stimuli suggests that spindle afferents from intrinsic hand muscles may mediate patients' ulnar-induced EMG modulations. Abnormal proprioceptive control was only observed on dystonic side, while bilateral unusual cutaneous control was found in patients. Changes in spinal transmission were partly related to the dystonic wrist posture, suggesting that systems involved in sensory processing can be differentially altered in writer's cramp. SIGNIFICANCE: Changes in spinal transmission, probably related to peripheral and/or cortical inputs, might either take part in primary or adaptive mechanisms underlying writer's cramp.     

补充维生素D预防骨折——随机对照试验汇总分析

背景：对于脊椎以外的骨折而言，补充口服维生素D的预防作用和用量仍无定论。 目的：评估补充维生素D在预防老年髋骨骨折和非脊椎骨折方面的效果。 数据来源：使用MEDLINE、Cochrance对照试验记录（1960～2005年）以及EMBASE（1991-2005年），对英文和非英文文章进行系统回顾。通过与临床专家接触，通过检索美国社会骨和骨矿研究协会提供的参考文献和摘要（1995～2004年）．进一步寻找更多的研究。检索词包括随机对照试验（randomized controlled trial，RCT）、临床对照试验、随机分配、双盲法、维生素D3、维生素D2；25-羟基维生素D、骨折、人类、老年、摔倒和骨密度。 研究选取：纳入的研究仅限于口服补充维生素D（维生素D3、维生素D2、补钙或不补钙）与补钙或安慰剂比较的双盲RCTs。试验于检查髋部骨折或非脊椎骨折的老年人（年龄≥60岁）中进行。数据提取：两位作者根据预先规定独立提取相关数据，其中包括研究质量指标。 数据综合：所有的汇总分析均以随机效应模型为基础。5项有关髋部骨折的RCTs（n=9294）和7项有关非脊椎骨折危险的RCTs（n=9820）符合我们的纳入标准。所有试验均使用了维生素D3。对髋部和非脊椎骨折预防研究的异质性亦进行观察，用低剂量（400IU／d）和高剂量（700～800IU／d）分别合并RCTs后异质性消失。与补钙或安慰剂相比，每天服700～800IU的维生素D可使髋部骨折的相对危险（relative risk，aa）下降26％（3项RCTs共计5572人；RR，0．74；95％可信区间[confidence interval，CI]，0．61～0．88），使非脊椎骨折的相对危险下降23％（5项RCTs共计6098人；RR，0．77；95％CI，0．68～0．87）。每天服400IU的维生素D（2项RCTs共计3722人；髋部骨折RR，1．15；95％CI，0．88～1．50；非脊椎骨折RR，1．03；95％CI，0．86—1．24）未见明显获益。 结论：口服补充维生素D（700～800IU／d）可以降低尚能活动的老人或慈善机构收容的老年人发生髋部骨折和脊椎以外骨折的危险，每天口服400IU维生素D并不足以预防骨折。