This scoping review aims to summarize the literature pertaining to survival in Progressive Supranuclear Palsy (PSP) and Multiple System Atrophy (MSA), with a focus on: 1) clinical milestones, 2) illness trajectory, and 3) other predictors of mortality as they relate to neuropalliative care in this population.

PSP and MSA are progressive neurodegenerative disorders with complex symptom burden and unpredictable disease trajectories. The ideal timing for initiation of neuropalliative care is uncertain given the variable illness trajectory of both disorders.

Medline and Embase databases were used to identify publications investigating clinical milestones and survival in PSP and MSA. A medical librarian co-developed the search strategy.  Primary research studies or systematic reviews of the literature pertaining to clinical milestones, illness trajectory and other predictors of mortality in PSP and MSA were included. Studies were excluded if they related only to symptom management, did not assess survival/prognosis, or were published prior to 2010. Results were rated for quality and qualitatively combined to suggest triggers for targeted palliative care throughout the illness trajectory. 

A total of 2092 records were screened, 2012 studies were deemed irrelevant, and 79 studies underwent full text review. Twenty-four studies were ultimately included. ‘Clinical milestones’ are clinically relevant disease points that prompt further care. Important milestones include frequent falls, cognitive impairment, severe dysarthria, severe dysphagia, wheelchair dependence, urinary catheterization, and institutionalization. In PSP, early milestones included falls and cognitive impairment, while wheelchair dependence and urinary catheterization occurred later. In MSA, autonomic dysfunction and falls occurred early in the illness trajectory whereas institutionalization was a marker of advanced illness.

Clinical milestones can be used to follow disease progression and help predict survival in PSP and MSA. We propose a milestone-driven framework in which milestones are used as triggers for targeted palliative care interventions including the early initiation of neuropalliative care.