Palliative care and its emerging role in Multiple System Atrophy and Progressive Supranuclear Palsy

Abstract

Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are sporadic, neurodegenerative conditions and belong to a group known as the atypical Parkinsonian disorders. The atypical Parkinsonian (AP) disorders have some features of idiopathic Parkinson's disease (PD) but often with poor or transient levodopa response [1], distinct symptom profiles, more rapid progression and reduced survival. They have significant symptom burden [2]. It is beginning to be appreciated that AP disorders not only carry a heavy symptom burden equivalent to that of patients with advanced cancer but that AP patients experience significant morbidity for an extended period of time. PSP and MSA patients reach clinical milestones such as wheelchair-dependence or frequent falls early in the course of their disease and can live with these accumulating burdens for some years [3]. The acknowledgement of the benefits of emphasizing quality of life in progressive disorders and the growing evidence for palliative care need in non-malignant conditions suggests that MSA and PSP would benefit from an integrated palliative approach. Studies to determine palliative need and possibility of benefit from intervention are beginning to yield results in Parkinsonian conditions. This review aims to discuss the trajectory of disease in MSA and PSP, explore significant symptoms and summarize some evidence which exists for palliative care need and quality of life in these conditions. The challenges of a palliative approach with this group including advance care planning will also be discussed.