Disease course and treatment patterns in progressive supranuclear palsy: A real-world study

• Published in: Journal of the neurological sciences 2021-02, Vol.421, p.117293-117293, Article 117293
• Main Authors: Morgan, John C., Ye, Xiaolan, Mellor, Jennifer A., Golden, Keisha J., Zamudio, Jorge, Chiodo, Louis A., Bao, Yanjun, Xie, Tao
• Format: Article
• Language: English
• Subjects: Cross-Sectional Studies; Diagnosis; Disease course; France; Germany; Humans; Italy; Progressive supranuclear palsy; Real-world; Spain; Supranuclear Palsy, Progressive - diagnosis; Supranuclear Palsy, Progressive - therapy; Symptoms; Treatment
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2020.117293

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. •Progressive supranuclear palsy (PSP) carries a considerable clinical burden.•Speed of diagnosis is improving but still averages over a year from symptom onset.•Neurologists (especially movement disorder specialists) can expedite diagnosis.•Only symptomatic treatments are available with very poor treatment satisfaction.•Disease-modifying agents are urgently needed for this debilitating terminal disease.