Motor Neurone Disease (MND): Management Suresh Chhetri MD, FRCP, FHEA Consultant Neurologist and Co-Director Lancashire and South Cumbria MND care and Research centre Royal Preston Hospital
Symptomatic and supportive • Treat symptoms • Maximize function • Promote independence • Maintain/enhance quality of life Kiernan et al. Lancet, 2011;377:942-955 Hardiman et al. Nat Rev Dis Primers. 2017;3:17085 van Es et al. Lancet 2017; 390: 2084–98
p=0.014 at 12 months P=0.046 at 21 months
Non-invasive Ventilation Volume 5, Issue 2, February 2006, Pages 140–147
Multidisciplinary…. • Improves survival • Improves quality of life • Reduces hospital stays Bede et al, BMJ Supportive and Palliative Care 2011. 1:343-8 Van der Berg et al, Neurology 2005;65:1264–7 Traynor et al JNNP, 2003;74:1258–1
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“Multidisciplinary care should be available for people affected by ALS”
Case A 62 year old lady had presented with 4 month history of progressive lower limb weakness. She was diagnosed with MND and commenced on riluzole. Two months later, she reported pain in limbs, neck and back ”I wonder whether we are missing something” Are you happy with the diagnosis? What are your diagnostic considerations? What are the management options?
Pain or no pain?
Pain • Largely neglected symptom until a decade back • Profound effect on quality of life of patient and caregivers Wicks P. Eur J Neurol 2012; 19: 531–32
Pain epidemiology • Frequency varies from 15% to 85% • ? Reliability • Under-recognized • Less common in bulbar form of MND Chiò et al. Lancet Neurol. 2017;16:144-157
Characteristics of pain Chiò et al. Lancet Neurol. 2017;16:144-157
Location of pain • Limbs • Back • Shoulders • Neck • Widespread Chiò et al. Lancet Neurol. 2017;16:144-157
Primary causes • Neuropathic features – no or weak evidence • Cramps • Spasticity Chiò et al. Lancet Neurol. 2017;16:144-157
Secondary causes • Mainly nociceptive • Musculoskeletal • Joint contracture • Shoulder pain • Decubitus ulcers • Interface related problems with mask Chiò et al. Lancet Neurol. 2017;16:144-157
Treatment • Pharmacological • Non-pharmacological Chiò et al. Lancet Neurol. 2017;16:144-157
Pharmacological “There is no evidence from randomised controlled trials about the management of pain in ALS”
Management • WHO analgesic ladder • Treat the pain phenotype Chiò et al. Lancet Neurol. 2017;16:144-157
Non-pharmacological • Regular stretching and exercises to prevent spasticity • Warm and cold compresses • Splints • Mask care Majmudar et al. Muscle Nerve 2014; 50: 4–13
What about rehabilitation “Can you cure ALS?” “I am afraid I can’t.” “What else can you offer then?” “Rehabilitation for ALS? I thought there is nothing you can do for this disease.” Muscle Nerve 50: 4–13, 2014
“Can you cure ALS?” “I am afraid I can’t.” “What else can you offer then?” “Rehabilitation for ALS? I thought there is nothing you can do for this disease.” Muscle Nerve 50: 4–13, 2014
Case • 52 year old teacher with new diagnosis of limb onset MND. • Right upper limb weakness • Right leg weakness with few falls • Struggling with cutting food and doing buttons • Fatigue • Keen to continue employment • How would you formulate your Rx plan?
• Multidisciplinary • Falls assessment • Adaptive equipment for ADL • Assistive devices for ambulation
Majmudar et al. Muscle Nerve 50 : 4–13, 2014
Case 63 year old gentleman arrested by police as was driving erratically and sounded ‘drunk’. However, breath analysis showed no evidence of alcohol intake. He reported no concerns but his wife has noticed some speech difficulties. He has refused to see his GP as he does not think that there is anything wrong with him. Clinical findings are consistent with MND. Any thoughts on his presentation? Will the clinical presentation influence your Rx plans
Cognitive deficits • 50% behavioural or cognitive changes • 10-15% FTD • C9ORF72 repeat expansion • 7% of sporadic and in 39% familial • ALS-FTD Goldstein et al. Lancet Neurol 2013;12:368–380 Phukan et al. JNNP, 2012;83:102–108 Majounie et al. Lancet Neurol. 2012;11:323-30
Cognitive profile J Neurol Neurosurg Psychiatry 2016;87:611–619 Deficits in • Executive functions •Assessment of verbal fluency • Language • Social cognition • Verbal memory
Behavioral changes • Apathy most common (70%) • Behavioural disinhibition • Loss of sympathy/empathy • Perseverative, stereotyped, or compulsive/ritualistic behaviours • Hyperorality and dietary changes Strong et al Amyotroph Lateral Scler Frontotemporal Degener. 2017; 18:153-174
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2017; 18:153-174 • ALS with behavioural impairment (ALSbi) • ALS with cognitive impairment (ALSci) • ALScbi • ALS-FTD
Neurology 2018;91:e1370-e1380
Neurology 2018;91:e1370-e1380
”Phenotypic spectrum of single disease” van Es et al. Lancet 2017; 390: 2084–98
Impact of cognitive impairment • Worse prognosis • Poor quality of life • Increases caregiver burden • Negative impact on carer QOL Beeldman et al. JNNP 2016;87:611–619 Crockford et al. Neurology 2018;91:e1370-e1380
Case 74 year old gentleman with recent diagnosis of bulbar onset MND. He has lost 9 kg within the last 3 months and has just started to notice some difficulties with eating. What are the management options? What factors would you consider in the decision making process?
Malnutrition • Independent prognostic factor for survival in MND • Eight fold increased risk of death
J Neurol Neurosurg Psychiatry 2011;82:628e634 • 30% increased risk of death for a 5% decrease from usual weight at time of diagnosis • 34% increased risks of death associated with each 5% decrease in usual weight • Malnutrition associated with shorter survival
Malnutrition - multifactorial • Dysphagia • Anorexia • Motor weakness • Reduced caloric intake • Anxiety/depression • Respiratory insufficiency • Hypermetabolism Kiernan et al. Lancet, 2011;377:942-955 Hardiman et al. Nat Rev Dis Primers. 2017;3:17085 van Es et al. Lancet 2017; 390: 2084–98
Malnutrition • Proactive approach • Regular nutritional assessment
Strategies • Nutritional supplements e.g. high protein and caloric diets • Adjustments in diet consistency • Feeding techniques, e.g. chin tuck and taking small meals • Enteral feeding
Enteral feeding
Enteral feeding Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease (Review) Katzberg HD, Benatar M 2011, Issue 1. Art. No.: CD004030 No RCT ‘best evidence suggests survival advantage but tentative’
“I'm just so grateful it’s helping me to live, and live more comfortably.” “My quality of life has changed for the better. Improved, I am not hungry or thirsty and don’t have to struggle to swallow.” “Took stress off me eating. It has made my quality of life so much better I would recommend it” “My quality of life has improved it has made travelling better as I can take my own supplies. Putting a feed in by tube is much quicker than the speed I was eating before. I am no longer shy of people seeing me put a feed in so I can go anywhere I want to go.” Chhetri et al. Palliat Med. 2017;31:676-677
Gastrostomy Indications • Dysphagia • Decline in nutritional state • weight loss of more than 10% from pre-morbid weight • body mass index of less than 18.5 kg/m 2 • Forced vital capacity more than 50% of predicted value Kiernan et al. Lancet, 2011;377:942-955 Hardiman et al. Nat Rev Dis Primers. 2017;3:17085 van Es et al. Lancet 2017; 390: 2084–98
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