Movement & Memory Disorders Program, Parkinson Foundation Center of Excellence, Department of Neurology, Medical College of Georgia, Augusta University, Augusta, GA, USA.
AbbVie Inc., North Chicago, IL, USA.
J Neurol Sci. 2021 Feb 15;421:117293. doi: 10.1016/j.jns.2020.117293. Epub 2020 Dec 25.
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder with symptoms including vertical gaze palsy, frequent falls, abnormal gait, and cognitive/language/behavioral changes, making diagnosis and treatment challenging.
Descriptive analysis was undertaken of cross-sectional, real-world data for patients with PSP provided by neurologists in France, Germany, Italy, Spain, UK, and USA.
Data on 892 PSP patients were obtained from patient records. Common initial symptoms included difficulty walking/maintaining gait, confusion/disorientation, loss of balance/falling, and rigidity. These symptoms and vertical gaze palsy commonly aided diagnosis. At data collection, dysphagia and blepharospasm were also very common. Mean times from symptom-onset to consulting a healthcare professional and PSP diagnosis were 5.2 and 15.0 months, respectively. General practitioners or movement disorder specialists were most commonly consulted initially; 98% of patients were diagnosed with PSP by a movement disorder specialist or general neurologist. Alternative diagnoses, including Parkinson's disease (67%) and dementia (10%), were considered for 41% of patients prior to PSP diagnosis. Non-wheelchair walking aids and wheelchairs were used by 60% and 23% of patients, respectively, with mean times from symptom-onset to use being 20.8 and 39.5 months, respectively. Symptomatic medication, most often levodopa and antidepressants, was prescribed for 87% of patients.
This study provided information on disease course and treatment for a large number of PSP patients from various countries. PSP carries a considerable clinical burden. Diagnosis is often delayed. Consulting a movement disorder specialist might expediate diagnosis. Currently, only symptomatic treatments are available with a poor satisfaction, and there is an urgent need for disease-modifying agents.
进行性核上性麻痹（PSP）是一种神经退行性疾病，其症状包括垂直性眼球运动障碍、频繁跌倒、步态异常以及认知/语言/行为改变，这使得诊断和治疗具有挑战性。
对来自法国、德国、意大利、西班牙、英国和美国的神经病学家提供的 PSP 患者的横断面真实世界数据进行描述性分析。
从患者记录中获得了 892 名 PSP 患者的数据。常见的首发症状包括行走/维持步态困难、意识模糊/定向障碍、失去平衡/跌倒以及僵硬。这些症状和垂直性眼球运动障碍通常有助于诊断。在数据采集时，吞咽困难和眼睑痉挛也非常常见。从症状出现到咨询医疗保健专业人员和 PSP 诊断的平均时间分别为 5.2 和 15.0 个月。最初最常咨询的是全科医生或运动障碍专家；98%的患者由运动障碍专家或普通神经科医生诊断为 PSP。在 PSP 诊断之前，有 41%的患者被考虑过其他诊断，包括帕金森病（67%）和痴呆症（10%）。分别有 60%和 23%的患者使用非轮椅助行器和轮椅，从症状出现到使用的平均时间分别为 20.8 和 39.5 个月。有 87%的患者使用了对症药物，最常见的是左旋多巴和抗抑郁药。
这项研究提供了来自不同国家的大量 PSP 患者的疾病进程和治疗信息。PSP 给患者带来了相当大的临床负担。诊断通常会延迟。咨询运动障碍专家可能会加速诊断。目前，只有对症治疗，患者满意度较差，迫切需要疾病修饰药物。
