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

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.

Neuropalliative care for progressive neurological diseases: A scoping review on models of care and priorities for future research.

BACKGROUND: Neuropalliative care is a newly-defined subspeciality bringing specific aspects of fields of neurology and palliative care together to better meet the complex care needs of people with progressive neurological diseases. Examining these needs would help provide guidance about developing relevant models of care and identify gaps in research knowledge. AIM: To identify current models and approaches to neuropalliative care for people with progressive neurological diseases and the priorities for future research work. DESIGN: A scoping literature review following the methods described by the Joanna Briggs Institute. DATA SOURCES: An electronic search of the literature was undertaken from six sources including MEDLINE (Ovid), EMCARE, PsycINFO and CINAHL covering the years January 2011 to September 2021. RESULTS: Twenty-eight studies were found examining neuropalliative care from the perspectives of 4795 PND patients, 774 informal carers and 138 health professionals. All studies held themes of integrative care, with most studies employing outpatient models of multidisciplinary care. Topics discussed included: overcoming local system-issues, providing education for professionals, patients and carers, early referral and capturing outcome measures for quality-assurance and future research work. CONCLUSIONS: Most models of neuropalliative care described in the international literature are predominantly outpatient, multidisciplinary and integrative. Clinicians typically utilise existing neurology and palliative care infrastructure to provide care. More high-quality research and outcome tools are needed to guide the design of evidence-based palliative care for people with progressive neurological diseases.