Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.
Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.
复制标题
DOI：
10.1007/s11940-016-0422-5
复制
发表时间：
2016-09
影响因子：
2
通讯作者：
Morris HR
中科院分区：
文献类型：
--
作者：
Lamb R;Rohrer JD;Lees AJ;Morris HR
目前还没有针对进行性核上性麻痹（PSP）或皮质基底变性（CBD）的疾病改善治疗方法，也没有被批准的有效控制其症状的药物或治疗方法。大多数药物治疗方案的使用是基于其他疾病的经验或非随机历史对照、病例系列或专家意见。左旋多巴可以在一定程度上改善PSP和CBD患者的帕金森症状（特别是运动迟缓和僵硬）；然而，证据是相互矛盾的，在现有的情况下，好处往往可以忽略不计，而且是短暂的。事实上，对左旋多巴的“不良”反应是NINDS-SPSP诊断PSP标准的一部分，也是诊断CBD的共识标准（Lang Mov disorders . 20补刊1:S83-91， Litvan等）。神经学,48:119-25;Armstrong等人。神经学。80(5):496 - 503年)。有证据表明，涎腺内注射肉毒杆菌毒素对治疗有问题的唾液有帮助，肌内注射肉毒杆菌毒素和巴氯芬有助于减轻肌张力障碍，包括眼睑痉挛。苯二氮卓类药物也可用于治疗肌张力障碍。肌阵挛可使用左乙拉西坦和苯二氮卓类药物治疗。经许可用于阿尔茨海默病的药物（如乙酰胆碱酯酶抑制剂和n -甲基- d -天冬氨酸受体拮抗剂）已在标签外用于PSP、CBD和其他牛头病变，目的是改善认知；然而，有有限的证据表明它们是有效的，副作用的风险可能大于益处。不建议老年人或痴呆相关患者使用非典型抗精神病药物治疗行为症状，大多数抗精神病药物会加重帕金森病。抗抑郁药可能对行为症状和抑郁症有用，但由于副作用，通常耐受性差。在缺乏针对CBD和PSP的根本原因的有效药物治疗的情况下，管理应侧重于优化生活质量，缓解症状并帮助患者进行日常生活活动（ADL）。患者应由多学科团队管理，包括神经学家、物理治疗师（PT）、职业治疗师（OT）、言语和语言治疗师（SALT）、营养师、眼科医生、心理学家和姑息治疗专家。
There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived. In fact, “poor” response to levodopa forms part of the NINDS-SPSP criteria for the diagnosis of PSP and consensus criteria for the diagnosis of CBD (Lang Mov Disord. 20 Suppl 1:S83–91,; Litvan et al. Neurology. 48:119–25,; Armstrong et al. Neurology. 80(5):496–503,). There is some evidence that intrasalivery gland botulinum toxin is useful in managing problematic sialorrhea and that intramuscular botulinum toxin and baclofen are helpful in reducing dystonia, including blepharospasm. Benzodiazepines may also be useful in managing dystonia. Myoclonus may be managed using levetiracetam and benzodiazepines. Pharmacological agents licensed for Alzheimer’s disease (such as acetylcholinesterase inhibitors and N-Methyl-D-aspartate receptor antagonists) have been used off-label in PSP, CBD, and other tauopathies with the aim of improving cognition; however, there is limited evidence that they are effective and risk of adverse effects may outweigh benefits. The use of atypical antipsychotics for behavioural symptoms is not recommended in the elderly or those with demetia associated conditions and most antipsychotics will worsen Parkinsonism. Antidepressants may be useful for behavioral symptoms and depression but are often poorly tolerated due to adverse effects. In the absence of an effective drug treatment to target the underlying cause of CBD and PSP, management should focus on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL). Patients should be managed by a multidisciplinary team consisting of neurologists, physiotherapists (PT), occupational therapists (OT), speech and language therapists (SALT), dieticians, ophthalmologists, psychologists, and palliative care specialists.