Progressive motor syndrome in a welder with pallidal T1 hyperintensity on MRI: A two-year follow-up.

Chronic exposure to manganese (Mn) fume during welding may lead to mainly extrapyramidal syndrome that is resistant to treatment. We present a 32-year-old patient who developed severe postural instability, Parkinsonism, dystonia, and pyramidal signs in the 10th year of welding. The neurological condition of the patient worsened markedly in the following 3 years, resulting in severe disability rendering him to be assisted in all his daily activities and he did not benefit from any dopaminergic agent. T1 sequences of the MRI of the brain showed pallidal hyperintensity symmetrically. Welders in our country often protect their eyes but ignore to use tools that protect them from inhalation of the fume. Since chronic Mn toxicity may cause serious disability and irreversible neurological disturbances, we strongly believe that it is necessary to inform welders and their employers about this potential hazard.     

Elevated threshold for intracortical inhibition in focal hand dystonia.

Differences between control and focal hand dystonia (FHD) subject groups in short interval intracortical inhibition (SICI) as determined by paired transcranial magnetic stimulation (TMS) can be difficult to demonstrate, due to interindividual differences. The purpose of this study was to compare two TMS methods for assessing SICI in 8 control and 7 FHD subjects. Electromyographic (EMG) data were recorded from the first dorsal interosseous (FDI) muscle of the dominant hands of the control subjects and affected hands of the FHD subjects. The first method used a conventional approach of setting conditioning stimulus intensity to 80% of rest threshold (RTh) and test stimulus intensity to 120% RTh. Three interstimulus intervals (ISIs) were used: 2 msec, 3 msec, and the ISI between 2 and 3 msec that produced optimal SICI. The second method was novel in that test stimulus intensity was set to 150% active threshold (ATh), and conditioning stimulus intensity was varied between 50% and 100% ATh. The latter was determined at the threshold for SICI and expressed as a ratio of ATh. There was no difference between the subject groups in the degree of SICI produced using the first method, at the three ISIs studied. However, using the second method, the SICI threshold:ATh ratio was found to be significantly higher for FHD subjects. This finding suggests that determining the SICI threshold:ATh ratio may be a more sensitive measure of intracortical inhibitory function than more conventional methods.     

Blood pressure and heart rate in parkinsonian patients with and without wearing-off

Our study aimed to investigate the cardiovascular autonomic regulation related to the wearing-off phenomenon in Parkinson's disease (PD). We measured blood pressure (BP) and heart rate (HR) at rest and during orthostatic test in 16 patients with PD with wearing-off and in 15 patients with PD without wearing-off both before (baseline) and repetitively at 1-h intervals for up to 4 h after the morning PD medication dose.
The patients with wearing-off had fluctuation of BP during the observation period, BP increasing when the motor performance worsened and vice versa. The mean supine BP was at its highest at the baseline measurement (patients with wearing-off, 145 ± 18 mmHg; patients without wearing-off, 138 ± 17 mmHg), fell during the first hour (patients with wearing-off, 119 ± 17 mmHg; patients without wearing-off, 126 ± 18 mmHg), and then rose again toward the end of the observation period (patients with wearing-off, 136 ± 15 mmHg; patients without wearing-off, 138 ± 18 mmHg). This BP change was statistically significant only in PD patients with wearing-off ( P  < 0.001).
In conclusion, BP seems to fluctuate with motor impairment in PD patients with wearing-off. This fluctuation may represent autonomic dysfunction caused by the PD process itself, the effect of PD medication, or both.     

A Neuromagnetic Study of the Functional Organization of the Sensorimotor Cortex

Movement-related neuromagnetic fields from eight healthy human subjects were investigated in a Bereitschaftspotential paradigm. The three conditions studied were right-sided mouth, index finger and foot movement. The neuromagnetic field patterns corresponding to the motor field and the movement-evoked field I were analysed using a moving dipole model. For both components a somatotopic organization was found: the estimated dipole locations for the mouth were more lateral and those for the foot more medial than the estimated dipole positions for the index finger movement. With regard to possible clinical applications, e.g. non-invasive mapping of the sensorimotor cortex and studies of plasticity of the motor function, the present results suggest that the investigation of movement-evoked field I for the index finger condition is most likely to yield further results.     

MRI and SPECT findings in amyotrophic lateral sclerosis

Summary MRI was performed in 21 patients and single photon emission computed tomography (SPECT) withN-isopropyl-p-¹²³I iodoamphetamine in 16 patients, to visualize upper motor neurone lesions in amyotrophic lateral sclerosis. T2-weighted MRI revealed high signal along the course of the pyramidal tract in the internal capsule and cerebral peduncle in 4 of 21 patients. SPECT images were normal in 4 patients, but uptake was reduced in the cerebral cortex that includes the motor area in 11.     

Spatiotemporal pattern of striatal ERK1/2 phosphorylation in a rat model of L-DOPA-induced dyskinesia and the role of dopamine D1 receptors.

BACKGROUND: We examined the activation pattern of extracellular signal-regulated kinase 1 and 2 (ERK1/2) and its dependence on D1 versus D2 dopamine receptors in hemiparkinsonian rats treated with 3,4-dihydroxyphenyl-L-alanine (L-DOPA). METHODS: 6-Hydroxydopamine-lesioned rats were treated acutely or chronically with L-DOPA in combination with antagonists for D1 or D2 receptors. Development of dyskinesia was monitored in animals receiving chronic drug treatment. Phosphorylation of ERK1/2, mitogen- and stress-activated protein kinase-1 (MSK-1), and the levels of FosB/DeltaFosB expression were examined immunohistochemically. RESULTS: L-DOPA treatment caused phosphorylation of ERK1/2 in the dopamine-denervated striatum after acute and chronic administration. Similar levels were observed in matrix and striosomes, and in enkephalin-positive and dynorphin-positive neurons. The severity of dyskinesia was positively correlated with phospho-ERK1/2 levels. Phosphorylation of ERK1/2 and MSK-1 was dose-dependently blocked by SCH23390, but not by raclopride. SCH23390 also inhibited the development of dyskinesia and the induction of FosB/DeltaFosB. CONCLUSIONS: L-DOPA produces pronounced activation of ERK1/2 signaling in the dopamine-denervated striatum through a D1-receptor-dependent mechanism. This effect is associated with the development of dyskinesia. Phosphorylated ERK1/2 is localized to both dynorphinergic and enkephalinergic striatal neurons, suggesting a general role of ERK1/2 as a plasticity molecule during L-DOPA treatment.     

先天性风吹手畸形的治疗

目的探讨先天性风吹手畸形(windblown hand)的治疗。方法对18例患者采用分次手术的方法:一期改善拇指功能;二期纠正手指尺偏畸形;三期解决手掌、手指屈曲。结果术后随访6～72个月,3例患者失访。15例中有11例拇指外展、对掌、对指功能恢复,效果满意;9例手指尺偏畸形得到纠正,5例再次手术,经系统康复训练后4例畸形纠正,1例部分纠正。结论先天性风吹手畸形的治疗应分次手术,每次解决1个主要问题,配合系统的康复才能获得满意的效果。     

Keratoconus,allergy, itch,eye‐rubbing and hand‐dominance

Background: One hypothesis for the pathogenesis of keratoconus includes teenage allergy, ocular itch and associated eye‐rubbing. Methods: This study examined the prevalence of these factors for teenage and adult patients. The results for a sample of 53 subjects with bilateral keratoconus were compared with those for a control sample of non‐keratoconus subjects, who also routinely wore RGP contact lenses. The strongest dominant hand and the eye with more advanced keratoconus were also determined, to examine for a relationship between them. Results: The keratoconic sample reported significantly higher levels of allergy, itch and rubbing as teenagers and as adults. However, all distributions were bimodal, consistent with the hypothesis that allergy, itch and rubbing are relevant in the pathogenesis of keratoconus only when the highest levels of these factors are present. For example, a significant relationship between the stronger dominant hand and the more advanced eye was evident only in subjects who reported the most severe rubbing. Conclusions: This finding adds weight to the circumstantial evidence that rubbing contributes to the pathogenesis of keratoconus. Low levels of teenage rubbing by some keratoconic subjects suggest a non‐rubbing pathogenesis and that emphasis on rubbing management is not warranted in these cases. However, high levels of adult rubbing reported by many keratoconic subjects indicate that the standard advice to avoid vigorous and prolonged rubbing is often not effective, even when repeated. There appears to be an indication for the need to improve the management of eye‐rubbing for some patients with keratoconus or at risk of developing this disease.     

游离大网膜移植修复严重手外伤

目的 通过临床实践,探索一种比较简单和相对有效的治疗严重手外伤的方法,以求最大限度地恢复手功能.方法 选用部分大网膜游离移植和网膜上同时中厚植皮术修复严重手外伤伴有大面积软组织缺损.结果 1997年起应用此方法共治疗6例病人7只手,创面一期愈合.并且供区组织充足,厚薄适度,不造成供区严重损伤,随访2～8年病人没有肠粘连症状出现.结论 选用此方法修复严重手外伤术后外形不臃肿,组织柔软,并能保留部分损伤的骨关节结构,瘢痕增生不明显,有利于手内关节活动和手功能恢复.