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

Background: 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. Methods: 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. Results: 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. Conclusion: 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.