Update information October 2019: Some footnotes were amended by NICE to reflect a change in marketing authorisation status for botulinium neurotoxin type A preparations. July 2019: Because of a risk of abuse and dependence, gabapentin is controlled under the Misuse of Drugs Act 1971 as a class C substance and scheduled under the Misuse of Drugs Regulations 2001 as schedule 3 (as of 1 April 2019). A footnote has been added to this guideline to reflect this change.
Motor Neurone Disease: Assessment and Management
NICE Guideline, No. 42
Authors
National Clinical Guideline Centre (UK).Excerpt
Motor neurone disease (MND) is a neurodegenerative condition that affects the brain and spinal cord. MND is characterised by the degeneration of primarily motor neurones, leading to muscle weakness.
The presentation of the disease varies and can be as muscle weakness, wasting, cramps and stiffness of arms and/or legs, problems with speech and/or swallowing or, more rarely, with breathing problems. Whichever area the disease starts, as the disease progresses the pattern of signs and symptoms becomes similar, with increasing muscle weakness in the person's arms and legs, problems swallowing and communicating and weakness of the muscles used for breathing, which ultimately leads to death. Most people die within 2-3 years of developing symptoms, but 25% are alive at 5 years and 5-10% at 10 years. The most common type of MND is amyotrophic lateral sclerosis (ALS). There are rarer forms of MND such as progressive muscular atrophy and primary lateral sclerosis, which may have a slower rate of progression.
Every person with MND has an individual progression of the disease. About 10-15% of people with MND will show signs of frontotemporal dementia, which causes cognitive dysfunction and issues with decision-making. A further 35% of people with MND show signs of mild cognitive change, which may affect their ability to make decisions and plan ahead.
MND is a disorder which can affect adults of any age. However, incidence is highest in people aged 55-79; onset below the age of 40 years is uncommon. There are approximately 4,000 people living with MND in England and Wales at any one time. The cause of MND is unknown. About 5-10% of people with MND have a family history of the disease and several abnormal genes have been identified.
As there is no cure for MND, care focuses on maintaining functional ability and enabling people with MND and their family members to live life as fully as possible. Early diagnosis, without delay after investigation, may be helpful as it allows for the provision of medication and aids, as well as for communication about the disease and advance care planning to be undertaken appropriately.
Care of people with MND varies across England and Wales, with MND multidisciplinary team clinics and networks providing coordinated multidisciplinary care. However, some people with MND are left isolated and their care is less than ideal. This guideline aims to consider the clinical- and cost-effectiveness evidence for the care of people with MND from the time of diagnosis, including communication of the diagnosis. It covers monitoring of disease progression, management of symptoms (in particular muscle weakness, excess secretions, breathing and nutrition problems), ongoing support and services, mobility, emotional and psychological changes, and preparation for end of life. Particular emphasis is placed on determining the best way to organise the care and management of people with MND.
Disclaimer: Healthcare professionals are expected to take NICE clinical guidelines fully into account when exercising their clinical judgement. However, the guidance does not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of each patient, in consultation with the patient and, where appropriate, their guardian or carer.