变构蛇神经毒素治疗运动神经元病114例的临床观察

本文报道变构蛇神经毒素对运功神经元病114例治疗的临床观察。以2ml(500ug/500μg/ml)每日一次,肌内注射用于治疗肌萎缩侧索硬化68例、进行性肌萎缩40例,原发性侧索硬化6例,其中83例随访3～28个月,病情好转20例(24.17％),病情稳定26例(31.3％),无效37例(44.6％)。对疗效作了评价。讨论工作用机理,主要是在细胞受体部位的阻断作用,防止有害物质的侵犯,也有诱发干扰素的作用。     

Intermittent subcutaneous apomorphine injection treatment for Parkinsonian motor oscillations

Eight patients with severe Parkinsonian motor oscillations have been treated with the dopamine receptor agonist apomorphine by intermittent subcutaneous self-injection as an adjunct to oral anti-Parkinsonian medication. The dopamine receptor antagonist domperidone was also given by mouth to prevent nausea. Six patients remain on chronic treatment (mean period 6.5 months) with improved control of motor function in each case. Four have had major enhancement of their quality of life. Benefits of this treatment stem from the training of patients to use intelligent behaviour to administer a promptly acting and effective pharmacological agent, thereby exercising a degree of direct control over previously unpredictable variations in motor performance.     

Effects of lingual nerve and chewing cortex stimulation upon activity of the swallowing neurons located in the region of the hypoglossal motor nucleus

This study focuses on motoneurons and interneurons in the region of the hypoglossal nucleus (XIIth) related to swallowing and chewing. In sheep anesthetized with halothane, we have used extracellular microelectrodes to study the effects of stimulation of the superior laryngeal nerve (SLN), the lingual nerve (LN) and the chewing cortex (CCx) upon activities of the swallowing neurons (SNs). Ipsilateral stimulation (1-5 pulses at 500 Hz) of the peripheral afferents or CCx did not generally induce a short latency activation of the hypoglossal swallowing motoneurons (Group I SNs) since only 4 motoneurons (69 tested) were activated by the SLN, 4 motoneurons (56 tested) by the LN and none by the CCx. In contrast, the same stimulations were more effective with swallowing interneurons (Group II SNs) located in the reticular formation close to the XIIth motor nucleus since 12 neurons (30 tested) were activated with short latencies (9 +/- 1.8 ms; mean latency +/- S.D.) by the SLN, 9 neurons (21 tested) by the LN (latency; 8 +/- 1.8 ms) and 5 neurons (18 tested) by the CCx (latency: 13 +/- 1.7 ms). Seven neurons were activated by two or three modes of stimulation indicating the existence of convergent inputs upon some Group II SNs. During chewing movements induced by a prolonged stimulation (20-40 Hz) of the CCx, 10 Group I SNs (16 tested) versus only one Group II SN (8 tested) were found to fire in association with the jaw opening. Moreover, 3 motoneurons and 4 interneurons inactive during swallowing discharged during chewing movements.(ABSTRACT TRUNCATED AT 250 WORDS)     

Role of the area postrema of medulla oblongata in the regulation of canine int erdigestive migrating motor complex

Objective To determine the effect of the area postrema (AP) of the medulla oblongata on gastrointestinal interdigestive migrating motor complex (MMC) and the plasma motil in level. Methods Interdigestive MMC activities of the antrum and duodenum were recorded by strain gauge implanted on the serosa of 8 conscious dogs.A cannula was intubated in femoral vein for motilin injection.The plasma motilin concentration was measur edby RIA.We observed: (1) normal interdigestive MMC activity and fluctuatio ns in plasma motilin concentration; (2) the effects of electrically damaging the AP on MMC activity and plasma motilin level; (3) whether intravenous injection of motilin could induce phase Ⅲ contractions after the AP was destroyed.Results (1) Typical interdigestive MMC with phase Ⅰ, Ⅱ, Ⅲ, and Ⅳ was recorded in nor mal dogs.Phase Ⅲ was concurrent with the peak of plasma motilin level.(2) I n damaged AP dogs, antroduodenal interdigestive MMC contractions were suppressed ; cyclic, phasic and migratory pattern of MMC was disrupted.Plasma motilin con centration was decreased.Intravenous injection of motilin could not induce pha se Ⅲ contractions. Conclusions The area postrema might play an important role, which is mediated by motilin, on the regulation of interdigestive MMC.     

Evaluations of combined premotor and supplementary motor cortex lesions on a visually guided arm movement

The kinematics of a visually guided, multi-joint arm movement were examined before and after combined bilateral premotor and supplementary motor cortex lesions. Two rhesus monkeys were trained to move a manipulandum from a start zone to one of three equally spaced target zones and then return to the initial start zone. Various features of the movement trajectory including space error, time error, peak velocity and turnaround time were quantified and analyzed before and after the premotor and supplementary motor cortex ablations. Statistical analysis showed no major differences in the trajectories toward or away from the target between the pre- and postlesion state. The major difference following the ablation was increase in the time spent in the target zone, reflecting an increase in time spent in redirecting the trajectory. Normalization of the movement duration revealed a slight delay in the initial part of the movement. These results suggest the premotor and supplementary motor cortex are involved in redirecting the trajectory and/or obtaining the target zone during the execution of a complex movement.     

Does orthotopic liver transplantation heal Wilson's disease? Clinical follow-up of two liver-transplanted patients

Two patients with Wilson's disease (WD) underwent orthotopic liver transplantation, one for subacute liver failure and the other for severe oesophageal haemorrhage. After transplantation both patients fully recovered within five months, and copper metabolism returned to normal. Follow-up examinations were continued for 4 and 6 years. Clinical as well as electrophysiological testing in these two patients yielded better results than in most of 12 WD-patients being conventionally treated for a similar period or even longer.     

Using an Optic Sensor and a Scanning Keyboard Emulator to Facilitate Writing by Persons with Pervasive Motor Disabilities

The present two studies assessed the use of an optic sensor together with a scanning keyboard emulator to enable two young adults with pervasive motor disabilities to click keyboard keys and, as a result, write. The optic sensor used for the participant of Study 1 was a light-dependent resistor that the participant activated with his tongue. The optic sensor used for the participant of Study II was a photoelectric device mounted on an eyeglasses’ frame that the participant activated by turning her eyes. The results showed that the optic sensor together with a scanning keyboard emulator allowed the participants to write fairly satisfactorily. Writing time per letter as well as numbers of words and letters written significantly improved during intervention sessions as opposed to baseline sessions. Preference checks showed that both participants favored the optic sensor and related tongue or eye response over the technology and responses available in baseline. Moreover, teacher trainees and psychology students involved in social validation checks of the intervention program provided fairly positive ratings.     

脑性瘫痪儿童精细运动功能评估量表的心理测量学特性研究

目的分析0—3岁脑性瘫痪儿童精细运动功能评估量表（FMFM）的信度、效度和反应度等心理测量学特性。方法共有375名0—3岁脑性瘫痪儿童参加了此项研究，分析FMFM精细运动能力分值的重测信度与评定员之间信度（等级相关系数分析）、平行效度（与PDMS-FM原始分的Pearson相关分析）、结构效度（样组间差异分析）和反应度（效应尺度）。结果FMFM量表精细运动能力分值具有良好的重测信度（ICC=0．9833）和评定员之间信度（ICC=0．9924）；与PDMS-FM量表原始分之间的相关系数为0．9199：能有效地区分同一偏瘫儿童健患侧上肢精细运动能力分值之间差异，同时也能有效地区分相同月龄段双瘫和四肢瘫儿童精细运动能力之间的差异；此外本量表具有较好的效应尺度。结论FMFM量表具有良好的信度、效度和反应度，可以有效地评定脑性瘫痪儿童精细运动能力。