Motor neurone disease: A point-prevalence study of patient reported symptom prevalence, severity and palliative care needs

Abstract

Background: Motor neurone disease is a rare but debilitating illness with incomplete evidence regarding patients’ symptom burden. Palliative care and generalist clinicians are often in-experienced in caring for these patients and assessing their needs. Aim: To identify the symptom prevalence and severity experienced by patients with motor neurone disease. Secondary objectives were to examine differences in symptom burden and clusters according to phenotype, functional status, palliative care provision and those in their last months of life. Design: A point prevalence study assessing patient-reported symptoms using a modified IPOS-Neuro assessment tool, incorporating 41 symptom items. Setting/participants: Patients with motor neurone disease attending the State-wide Progressive Neurological Disease Service or inpatient unit at Calvary Health Care Bethlehem, Melbourne Australia, from March to December 2021. Results: A total of 102 patients participated, the majority diagnosed with lumber-onset (30.4%), bulbar-onset (28.4%) and cervical-onset (25.5%) phenotypes. Patients experienced a median of 17 symptoms (range 2–32) with a median of 3 symptoms rated as severe/overwhelming (range 0–13). Motor and functional symptoms predominated, with differences in symptom clusters present according to phenotype. Patients had a higher number of severe/overwhelming symptoms if they were accessing palliative care services ( p = 0.005), in their last 6 months of life ( p = 0.003) and experiencing moderate or severe functional impairment ( p < 0.001). Conclusions: Patients with motor neurone disease report high symptom burden. A validated motor neurone disease-specific symptom assessment tool is needed to accurately assess patients, including important variations in symptom clusters according to phenotype. Further research must focus on evidence-based treatment guidelines for symptoms experienced commonly and severely.