Clinical Study Motor neurone disease in Lancashire and South Cumbria in North West England and an 8 year experience with enteral nutrition Suresh Kumar Chhetri a,c,⇑ , Belinda Fay Bradley b , Tahir Majeed a , Robert William Lea c a Preston MND Care and Research Centre, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, UK b Learning Information Services, University of Central Lancashire, Preston, UK c School of Pharmacy and Biomedical Sciences, University of Central Lancashire, Preston, UK article info Article history: Received 5 May 2015 Accepted 19 July 2015 Keywords: Clinical characteristics Demographics Enteral nutrition Motor neurone disease abstract Motor neurone disease (MND) is a fatal neurodegenerative disease of unknown aetiology. Malnutrition is a common occurrence and an independent risk factor for worse prognosis. However, it remains unclear whether provision of enteral nutrition (EN) through a gastrostomy tube offers any survival advantage. Our aim was to describe the demographic and clinical characteristics of MND in Lancashire and South Cumbria in North West England and the impact of EN on survival in the 8 year period of 2005–2012. Four hundred and seven patients with MND were identified through the Preston MND care and research centre registry giving a crude incidence rate of 3.15/100,000. Three hundred and forty patients with ade- quate information were included in the final analysis of whom 53.2% were male. The presentation was limb/spinal in 62.1% and bulbar in 37.9% of patients, bulbar onset being more common in elderly females. Mean age of onset was 67.28 years (standard deviation 11.06; range 22.78–93.06). Median survival was 1.98 years (range 1.18–3.05). Ninety-one patients received EN of whom 67% had bulbar onset disease. EN was not associated with a statistically significant survival advantage except for the subgroup who received EN more than 500 days after symptom onset. In conclusion, the early requirement for EN may indicate a prognostically less favourable subgroup. Ó 2015 Elsevier Ltd. All rights reserved. 1. Introduction Motor neurone disease (MND)/Amyotrophic lateral sclerosis is a relentlessly progressive neurodegenerative disease of unknown aetiology characterised by the degeneration of motor neurons in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. The annual incidence varies between 1.5–3.2/100,000 population [1–3]. Few studies suggest an increasing incidence of the illness [4–6], but this is not supported by longitudinal assess- ments [7–9]. Mean survival varies between 2 and 3 years [2,4,7]. In the absence of a cure, management is mainly supportive and palliative focussed on preserving independence and quality of life [10]. Dysphagia due to bulbar involvement develops in most patients during the course of illness with consequent aspiration pneumonia, weight loss and malnutrition [11]. Malnutrition is an independent adverse prognostic factor for survival with an eight-fold increased risk of death [12,13]. Body mass index has a prognostic value for survival at various stages of the illness, includ- ing at the time of diagnosis, at the time of gastrostomy placement or during the course of the illness [13,14]. Patients may not be able to meet their nutritional needs for a number of reasons including motor weakness interfering with the ability to feed oneself, reduced caloric intake, dysphagia, hypermetabolism and increased resting energy expenditure [14–16]. With inevitable disease pro- gression, enteral nutrition (EN) through a feeding tube may be needed and provides a reliable route for nutrition and hydration for patients with dysphagia [10,17,18]. The importance of nutri- tional management is being increasingly recognised but the evi- dence for a conferred survival advantage after EN is conflicting. Practice guidelines recommend considering EN in patients with dysphagia, weight loss of more than 10% over the baseline and before the forced vital capacity falls below 50% of the predicted value [10,18]. The aim of our study was to describe the demographics and clinical characteristics of patients with MND in Lancashire and South Cumbria in North West England and the impact of EN on survival. http://dx.doi.org/10.1016/j.jocn.2015.07.007 0967-5868/Ó 2015 Elsevier Ltd. All rights reserved. ⇑ Corresponding author. Tel.: +44 17 7252 2256; fax: +44 17 7252 3165. E-mail address: chhetri@doctors.org.uk (S.K. Chhetri). Journal of Clinical Neuroscience 24 (2016) 47–51 Contents lists available at ScienceDirect Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn