Natural clinical course of progressive supranuclear palsy in Chinese patients in Hong Kong

• Published in: Hong Kong medical journal = Xianggang yi xue za zhi 2019-12, Vol.25 (6), p.444-452
• Main Authors: Shea, Y F, Shum, A C K, Lee, S C, Chiu, P K C, Leung, K S, Kwan, Y K, Mok, F C K, Chan, F H W
• Format: Article
• Language: English
• Subjects: Age; Apraxia; Brain damage; Brain diseases; Caregivers; Dysphagia; Enteral nutrition; Geriatrics; Institutionalization; Language disorders; Larynx; Mortality; Patients; Pneumonia
• ISSN: 1024-2708; 2226-8707
• DOI: 10.12809/hkmj198101

Progressive supranuclear palsy (PSP) is a common type of atypical parkinsonism. To the best of our knowledge, there has been no study of its natural clinical course among Chinese patients. This retrospective study included 21 patients with PSP who had radiological evidence of midbrain atrophy (confirmed by magnetic resonance imaging) from the geriatrics clinics of Queen Mary Hospital and Tuen Mun Hospital. Clinical information was retrieved from clinical records, including age at onset, age at presentation, age at death, duration of symptoms, level of education, sex, presenting scores on Cantonese version of Mini-Mental State Examination, clinical symptoms, and history of levodopa or dopamine agonist intake and response. Clinical symptoms were clustered into the following categories and the dates of development of these symptoms were determined: motor symptoms, bulbar symptoms, cognitive symptoms, and others. Motor symptoms developed early in the clinical course of disease. Cox proportional hazards modelling showed that the number of episodes of pneumonia, time to vertical gaze palsy, and presence of pneumonia were predictive of mortality. Apathy, dysphagia, pneumonia, caregiver stress, and pressure injuries were predictive of mortality when analysed as time-dependent covariates. There was a significant negative correlation between the age at presentation and time to mortality from presentation (Pearson correlation=-0.54, P=0.04). Approximately 40% of caregivers complained of stress during the clinical course of disease. Important clinical milestones, including the development of dysphagia, vertical gaze palsy, significant caregiver stress, pressure injuries, and pneumonia, may guide advanced care planning for patients with PSP.