Parkinson's Disease and Movement Disorders Department, Hygeia Hospital, Erythrou Stavrou 9, 151 23, Maroussi, Athens, Greece.
Neurology Clinic, Philipps-University, Marburg, Germany.
CNS Drugs. 2018 Sep;32(9):827-837. doi: 10.1007/s40263-018-0551-3.
Progressive supranuclear palsy, corticobasal degeneration and multiple system atrophy account for approximately 10% of neurodegenerative parkinsonism. Considerable clinical overlap exists between these disorders that extends to features considered characteristic of each disease. Clinical diagnostic criteria have attempted to increase the accuracy of clinical diagnosis as accurate diagnosis is necessary to inform prognosis and to facilitate the recognition of disease-modifying treatments. Currently no such treatment exists. Nevertheless, many clinical trials aiming to change the natural history of these diseases are ongoing. The spread and accumulation of abnormal proteins are among the pathophysiological mechanisms targeted. For the time being, however, only symptomatic treatment is available. Levodopa is used to treat parkinsonism, but patients usually show a poor or transient response. Amantadine is also used in practice for the same indication. Botulinum toxin can alleviate focal dystonic manifestations. Addressing non-motor manifestations is limited by the potential of available drugs to impact on other aspects of the disease. Most of the new symptomatic formulations under study are focused on orthostatic hypotension in multiple system atrophy. Exercise, occupational, physical, and speech therapy and psychotherapy should always accompany pharmacological approaches.
进行性核上性麻痹、皮质基底节变性和多系统萎缩约占神经退行性帕金森病的 10%。这些疾病之间存在相当大的临床重叠，延伸到每种疾病都认为具有特征性的特征。临床诊断标准试图提高临床诊断的准确性，因为准确的诊断对于告知预后和促进识别疾病修饰治疗是必要的。目前尚无此类治疗方法。然而，许多旨在改变这些疾病自然史的临床试验正在进行中。异常蛋白质的扩散和积累是靶向的病理生理机制之一。然而，目前仅提供对症治疗。左旋多巴用于治疗帕金森病，但患者通常表现出不佳或短暂的反应。金刚烷胺也用于相同的适应症。肉毒杆菌毒素可缓解局灶性肌张力障碍表现。解决非运动症状受到现有药物对疾病其他方面影响的潜力的限制。大多数正在研究的新对症制剂都集中在多系统萎缩的直立性低血压上。运动、职业、物理和言语治疗以及心理治疗应始终与药物治疗相结合。
