干细胞移植治疗帕金森病

帕金森病是常见于中老年期的慢性进展性神经系统变性疾病.1817年帕金森病首先由英国医生Parkinson描述,临床表现为静止性震颤、运动困难、肌肉强直和姿势步态异常等.帕金森病多发于中老年人,其中40岁以上中年人患病率为0.1%～0.2%,而65岁以上老年人则达到1%.帕金森病的主要病理改变是中脑黑质多巴胺能神经元变性、缺失以及脑内路易体的形成.     

Stereotaxic neurosurgery for treatment of Parkinson disease

Three target structures are available in stereotactic surgery for Parkinson's disease: the thalamus, the globus pallidus and the subthalamic nucleus. The subthalamic nucleus appears to be the most promising structure. However, the thalamus can be considered in the case of an incapacitating tremor presenting as a primary symptom. Surgery in the globus pallidus may be as effective as in the subthalamic nucleus, but in the latter it is often accompanied by a reduction in dopaminergic medication. The surgical technique of electrical stimulation causes fewer adverse effects than that of coagulation and can therefore be applied bilaterally, but does require more intense postoperative care. In the selection of patients for surgery, levodopa responsiveness plays an important role in predicting effectiveness, except in the case of tremor.     

Successful treatment of depression in a Parkinson disease patient with bupropion

A 70-year-old female patient with Parkinson's disease was admitted to hospital with a medication-resistant depression. Electroconvulsion therapy was considered indicated, but it was decided to try treatment with bupropion chloride first. This resulted in a quick and complete remission of depressive symptoms, without any negative effects on motor symptoms. Bupropion has a unique mechanism of action: inhibition of the presynaptic reuptake of dopamine in addition to noradrenergic activity. Furthermore, it lacks the negative adverse effects on the extrapiramidal symptoms, that may be a problem if other antidepressants are used in the treatment. Bupropion is useful as an antidepressant in specific patient groups, notably patients with Parkinson's disease.     

造血干细胞移植治疗自身免疫性疾病

近十余年来,造血干细胞移植技术(hematopoi eticstemcelltransplantation,HSCT)治疗难治性自身免疫性疾病(autoimmunedisease,ADs),业已取得了可喜的成绩。国内外学者把病情进展快,造成明显残疾或早期死亡的ADs,归为“严重自身免疫病”(SADs),其预期生存期为5～10年;近年随着医学科学的进展,自身造血干细胞移植的治疗相关病死率为1%～5%;对SADs患者,在发展到不可逆脏器损伤前,进行积极的治疗是合理的、必要的。现将HSCT治疗ADs的机制、适应证、方式和过程做简要叙述。一、HSCT治疗ADs的机制、适应证及方式欧洲抗风湿联盟(EULAR…     

帕金森病患者营养不良的研究进展

帕金森病是第二常见神经系统变性疾病。帕金森病患者除常见的运动和非运动症状外,还常伴有营养不良等营养状况方面的症状,但相比于运动症状以及抑郁、焦虑、睡眠障碍等非运动症状,帕金森病患者的营养不良症状并未得到足够的重视。本文从帕金森病患者营养不良发生情况、营养不良发生的危险因素、营养不良的类型、具体缺乏的营养素、饮食习惯以及营养不良的干预措施等方面综述了帕金森病患者营养状况方面的研究现况。     

人胚腺垂体椎管蛛网膜下腔移植治疗垂体侏儒症

目的探索人胚腺垂体移植治疗垂体性侏儒症的新途径。方法选用妊娠20～28周水囊引产人胚腺垂体经腰穿方法移植于Ⅰ型垂体性侏儒症患者的椎管下段蛛网膜下腔。结果治疗23例,经1～5年随访,复查激素水平均有不同程度升高,1年身高增长2～4cm14例,4～6cm9例,其中3例5年内共移植3次,身高分别增长18cm、16cm和13cm,其他相应体征明显改善。结论人胚腺垂体椎管蛛网膜下腔移植治疗垂体性侏儒症有显著疗效。     

Nasolabial allergic reaction to intranasal administration of apomorphine in Parkinson disease]

A patient is presented who used apomorphine, a potent dopamine agonist in the treatment of Parkinson patients. He suffered of severe dystonia of the legs, which did not respond to conservative treatment. The apomorphine was delivered bij intranasal spray. The patient developed an allergic reaction with swollen nose and lips, which disappeared within one day after discontinuation of the treatment. Subcutaneous administration caused no symptoms. Cutaneous challenge with apomorphine caused a positive reaction. We raise the hypothesis that the allergic reaction was due to the binding of apomorphine to certain proteins in cutis or nasal mucosa.     

Developmental pesticide models of the Parkinson disease phenotype

It has been hypothesized that developmental insults could contribute to Parkinson disease (PD), a neurodegenerative disorder resulting from the loss of the dopamine neurons of the nigrostriatal pathway. Two models of developmental pesticide exposures in mice are presented here that yield PD phenotypes consistent with this possibility. Combined exposures to the herbicide paraquat (PQ) and the fungicide maneb (MB), both of which adversely affect dopamine systems, administered from postnatal days 5-19, produced selective losses of dopamine and metabolites and reduced numbers of dopamine neurons in the substantia nigra. Effects were greater than those produced by adult-only exposures. Moreover, developmental PQ + MB exposures enhanced vulnerability to this pesticide regimen when administered subsequently in adulthood. In a second model, exposure to MB from gestational days 10-17 markedly increased vulnerability to PQ exposures during adulthood, with reductions in dopamine and metabolites and numbers of dopamine neurons in the substantia nigra. Females evidenced protection in both models. Collectively, these models demonstrate that developmental exposures can produce progressive, permanent, and cumulative neurotoxicity of the nigrostriatal dopamine system and enhance vulnerability to subsequent environmental insults. Finally, effects of PQ + MB were greater than those of either pesticide alone in the postnatal model. This is consistent with a multiple-hit hypothesis predicting that multiple concurrent insults occurring at different target sites within a system (here nigrostriatal dopamine) may constrict the range and flexibility of compensatory mechanisms, thereby compromising the integrity and viability of the system. As such, this hypothesis presents a biologic strategy for identifying potentially significant neurotoxic mixtures for hazard identification in future studies.     

Incidence of Parkinson disease in Wakayama,Japan

To estimate an accurate annual incidence of Parkinson disease in Wakayama, a mail survey was conducted in 1998. A questionnaire was delivered to each clinic where Parkinson disease would be potentially diagnosed. The survey was conducted in February 1998 by the Research Committee on Parkinson disease of Wakayama. A total of 792 clinics and 87 hospitals were listed as candidates. Physicians in these 879 medical facilities were asked and instructed to register all newly diagnosed patients with Parkinson disease in 1997 according to the diagnostic criteria proposed by the Japanese Research Committee on Neuro-degenerative Diseases. Of 879 facilities, 873 ones including 81 hospitals replied (response rate: 99%). A total of 229 patients were reported as newly diagnosed cases in 1997. Of these cases, 183 cases were classified as Yahr I to III. The annual incidence rate was 16.9 per 100,000 population (95% confidence interval: 14.5-19.3). Male-to-female ratio was 1:1.4 as a whole, and the dominant age stratum was 70 to 79 years old. When Parkinson disease incidence was observed from northern part of Wakayama to south by district, crude rates (95% CIs) were 15.9(12.9-18.9), 18.1(12.0-24.2), and 19.3(13.4-25.2). After age-adjustment using the Japanese Model Population in 1985, differences of Parkinson disease incidence became attenuated and adjusted rates (95% CIs) turned to 10.8(9.1-12.7), 10.4(8.6-12.2), and 9.9(6.9-12.9), respectively.     

帕金森病患者血清中微量元素测定分析

目的 探讨帕金森病(PD)患者血清中微量元素水平与PD的关系.方法 选择PD患者40例作为病例组,另选择年龄、性别等与病例组患者相匹配的40例作为对照组.用石墨炉原子吸收法测定两组受检者血清中锰水平,电感耦合等离子体发射光谱ICP直接测定法测定血清中铮、铜、铁水平.结果 病例组血清中锰水平高于对照组[(0.269±0.326)μmol/L与(0.125±0.054)μmol/L比较,P<0.05],铁水平显著高于对照组[(1.512±0.949)μmol/L与(0.676±0.111)μmol/L比较,P<0.01],两组血清中铜、锌水平比较差异无统计学意义.结论 微量元素与PD的发生、发展有密切联系,其中金属元素铁、锰可能是PD的重要影响因素.     

Falling in Parkinson disease: more often due to postural instability than to environmental factors

OBJECTIVE: To document the circumstances surrounding falls in patients with Parkinson's disease. DESIGN: Prospective. METHOD: Parkinson patients with a check-up appointment in the period 1 May 1998-31 May 1999 at the Neurology Outpatient's Department of Leiden University Hospital, used standardized forms to record the circumstances surrounding all incidents of falling during the subsequent 6 months. For control purposes, a partner or acquaintance with comparable living conditions was asked to do the same. The researchers judged whether falls were related to physical impairment (personal, intrinsic factors) or environmental factors (extrinsic factors). RESULTS: 59 patients with Parkinson's disease (21 women (36%); mean age: 60.8 years; mean Hoehn and Yahr score: 2.3) and 55 controls (37 women (67%); mean age: 59.6 years) were studied. Patients reported 205 falls and controls 10 falls. Recurrent (> or = 2) falls occurred in 15 patients (25%) and two controls (4%) (relative risk (RR): 9.0: 95% confidence interval (95% CI): 2.0-41.7). Recurrent falls were more common among persons using benzodiazepines (RR: 5.0: 95% CI: 1.6-15.5). Reliable information was obtained for 160 falls in patients. Most falls (79%) had an intrinsic cause, like turning around, standing up, bending and stumbling. Abrupt movements were a particularly frequent cause of falls. Simultaneous performance of multiple tasks contributed to almost 50% of falls in patients. CONCLUSION: Falls are common among Parkinson patients. Most falls are related to intrinsic factors. Prevention of these intrinsic falls requires treatment of the underlying postural instability. Use of benzodiazepines must be avoided whenever possible. Interventions aimed at extrinsic factors, such as reduction of domestic hazards, are possibly less effective.