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首页> 外文期刊>The Internet Journal of Neurology >Cerebrolysin in Steel: Richardson-Olszewski Syndrome
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Cerebrolysin in Steel: Richardson-Olszewski Syndrome

机译：钢铁中的脑溶素：Richardson-Olszewski综合征

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Objectives: The authors investigated the effectiveness of Cerebrolysin, a neurotrophic factor, in 6 patients with Steel-Richardson-Olszwski (Progressive supranuclear palsy) also known by Opthalmo-facial-cervical dustonia. Methods: 6 patients (mean age 67.8 years) with progressive supranuclear palsy (PSP), mean duration of disease 3.8 years, were treated with Cerebrolysin 10 ml intravenous infusion on a daily basis for 20 days to be repeated every 2 months for a total of 6 months. Progressive supranuclear palsy rating scale and staging system (PSPRS) were assessed at baseline and after 6 months. Results: Five patients continued the study period; 5 patients showed modest improvement on (PSPRS) score. Side effect of Cerebrolysin was negligible. Conclusion: This study showed that Cerebrolysin has modest effect in the treatment and delaying progression in patients with PSP. Introduction Progressive Supranuclear Palsy (PSP) described by Steel – Richardson- Olszewski syndrome in 1964 (SROS) is a neurodegenerative disorder of unknown etiology. Pharmacological therapy has been disappointing 1,2.PSP usually presents itself in the seventh decade with early postural instability, vertical supranuclear gaze palsy( affectinf chiefly vertical gaze), pseudobulbar palsy, dysarthria,dystonic rigidity of the neck and upper trunk, frontal subcortical dementia, and parkinsonism. Clinical symptoms started between 48 and 51 years of age with slow progression during 2 to 4 years. Many of the symptoms present in PSP do not respond to dopamine stimulation. The potential benefits of these compounds are restricted to akinesia and rigidity3. Also the effectiveness of dopamine agonist is similar3.Cerebrolysin is a peptidergic solution containing free amino acids and biologically active peptides with proven neuroprotective and neurotrophic effect4 . This drug has previously been used in the treatment of brain dementia4, Parkinson disease5, and stroke6. Methods Six patients with PSP entered the open label study. Patients were recruited for the study during their routine outpatient visit to King Hussein Medical Center. All patients fulfilled the National Institute of Neurological Disorders and Stroke (NINDS) clinical criteria for the diagnosis of PSP. No levodopa or any dopamine agonist was allowed during the study period. Cerebrolysin was given 10 ml intravenous infusion on a daily basis for 20 days to be repeated every 2 months for a total of 6 months. Progressive supranuclear palsy rating scale and staging system were assessed at baseline and after 6 months.The Ethical committee of the King Hussein Medical Center approved this study. Results One of the six patients died 2 months after starting treatment due to myocardial infarction. Five patients (mean age 67.8 years, mean duration of disease 3.8 years) continued on Cerebrolysin for 6 months. There was modest improvement in the Progressive supranuclear palsy rating scale and staging system as seen in table 1. There was slowing in progression in our treated group according to (PSPRS) score in 2 patients. The remaining 3 patients had slight worsening in their PSPRS from baseline, but we still consider their condition as improved, since their PSPRS were lower than their expected PSPRS at 6 months. Side effects of Cerebrolysin was negligible.
机译：目的：作者研究了神经营养因子脑溶素对6例视神经-面部-宫颈尘埃沉着症所致的Steel-Richardson-Olszwski（进行性核上性麻痹）的有效性。方法：6例（平均年龄67.8岁）进行性核上性麻痹（PSP），平均病程3.8年的患者每天接受脑溶素10 ml静脉输注治疗20天，每2个月重复一次，总共6个月。在基线和6个月后评估进行性核上性麻痹评定量表和分期系统（PSPRS）。结果：5例患者继续研究期; 5例患者的（PSPRS）评分有所改善。脑溶素的副作用可忽略不计。结论：这项研究表明脑溶血素在治疗PSP患者中具有适度的作用并延迟其进展。简介1964年由Steel – Richardson-Olszewski综合征（SROS）描述的进行性核上性麻痹（PSP）是一种病因不明的神经退行性疾病。 1,2药理疗法令人失望。PSP通常在第七个十年出现，表现为早期姿势不稳，垂直核上凝视性麻痹（主要是垂直凝视），假球状麻痹，构音障碍，颈部和上躯干的僵直性僵硬，额叶皮层下痴呆和帕金森症。临床症状开始于48至51岁之间，并在2至4年内进展缓慢。 PSP中存在的许多症状对多巴胺刺激均无反应。这些化合物的潜在益处仅限于运动障碍和僵硬3。多巴胺激动剂的功效也相似。3脑溶素是一种含有游离氨基酸和生物活性肽的肽能溶液，具有经证明的神经保护和神经营养作用4。该药物以前曾用于治疗脑痴呆4，帕金森病5和中风6。方法六名PSP患者进入开放标签研究。在例行侯赛因国王医学中心的常规门诊期间招募了患者进行研究。所有患者均符合美国国家神经系统疾病和中风研究所（NINDS）的PSP诊断临床标准。在研究期间禁止使用左旋多巴或任何多巴胺激动剂。每天给予脑溶素10毫升静脉滴注，持续20天，每2个月重复一次，共6个月。在基线和6个月后评估渐进性核上性麻痹评定量表和分期系统。侯赛因国王医学中心伦理委员会批准了该研究。结果6例患者中有1例在开始治疗后2个月因心肌梗死死亡。五例患者（平均年龄67.8岁，平均病程3.8年）继续接受脑溶素治疗6个月。如表1所示，渐进性核上神经麻痹评定量表和分期系统有适度的改善。根据2个患者的（PSPRS）评分，我们治疗组的进展缓慢。其余3例患者的PSPRS较基线有轻度恶化，但我们仍认为他们的病情有所改善，因为其6个月时的PSPRS低于预期的PSPRS。脑溶素的副作用可忽略不计。

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