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Paraneoplastic motor neurone disease — Clinical Guidelines

Overview

Paraneoplastic motor neurone disease refers to motor neurone disease (MND) symptoms potentially triggered by an underlying malignancy, complicating the clinical presentation and management of this progressive neurodegenerative disorder. 1 2

Diagnosis

Clinical presentation includes progressive muscle weakness, atrophy, and fasciculations.

Neurological examination essential to assess motor function, bulbar function, and cognitive status.

No specific diagnostic tests; diagnosis often relies on exclusion of other conditions and characteristic clinical features. 2

Management

First-line treatments: Riluzole (50 mg twice daily) and edaravone (60 mg/day intravenously for 14 days) may modestly slow disease progression. 2

Multidisciplinary approach: Involves general practitioners, rehabilitation specialists, palliative care, and allied health services to address physical, psychological, and social needs. 2

Palliative care: Crucial throughout the disease trajectory to improve quality of life for patients and caregivers. 1 2

Symptom management: Includes respiratory support, nutritional assistance, and psychological support tailored to individual needs. 2

Special Populations

No specific guidance provided in the abstracts regarding management in pregnancy, pediatrics, or elderly populations. 2

Comorbidities: Management complexity increases with comorbidities; requires tailored multidisciplinary support. 2

Key Recommendations

Implement a multidisciplinary team approach involving palliative care early in the disease course to enhance quality of life for patients and caregivers. (Evidence: Strong 1 2)

Consider riluzole and edaravone for potential slowing of disease progression, based on available evidence. (Evidence: Moderate 2)

Address barriers to palliative care access, such as service contracts and resource limitations, to ensure comprehensive support for patients with MND. (Evidence: Expert opinion 4)

References

1 Flemming K, Turner V, Bolsher S, Hulme B, McHugh E, Watt I. The experiences of, and need for, palliative care for people with motor neurone disease and their informal caregivers: A qualitative systematic review. Palliative medicine 2020. link 2 Lau FS, Brennan FP, Gardiner MD. Multidisciplinary management of motor neurone disease. Australian journal of general practice 2018. link 3 Etkind SN. Terminal sedation: an emotional decision in end-of-life care. Journal of medical ethics 2012. link 4 McKenna C, MacLeod R. Access to palliative care for people with motor neurone disease in New Zealand. The New Zealand medical journal 2005. link

Paraneoplastic motor neurone disease