Motor Neurone Disease (MND): Management Suresh Chhetri MD, FRCP, - - PowerPoint PPT Presentation

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Motor Neurone Disease (MND): Management Suresh Chhetri MD, FRCP, - - PowerPoint PPT Presentation

May 19, 2023 481 likes •1.13k views

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

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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

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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

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p=0.014 at 12 months P=0.046 at 21 months

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Non-invasive Ventilation

Volume 5, Issue 2, February 2006, Pages 140–147

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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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MDT

Hospice or hospital-at-home service Gastrostomy services Wheelchair services Continence team Assistive technology, orthotics Augmentive and alternative communication services Neuropsychology/psychiatry Social worker/ benefits advisor Neurologist Physiotherapy Specialist nurse or care coordinator Palliative care specialist Speech therapy Dietician Respiratory specialist Occupational therapy Charities and health

  • rganizations, such as:
  • MND Association (UK)
  • ALS Association (USA)
  • Hobson et al. Amyotroph. Lateral Scler. Frontotemporal
  • Degener. 2013; 14, 521–527
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“Multidisciplinary care should be available for people affected by ALS”

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Case

A 62 year old lady had presented with 4 month history of progressive lower limb weakness. She was diagnosed with MND and commenced

  • n 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?

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Pain or no pain?

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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

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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

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Characteristics of pain

Chiò et al. Lancet Neurol. 2017;16:144-157

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Location of pain

  • Limbs
  • Back
  • Shoulders
  • Neck
  • Widespread

Chiò et al. Lancet Neurol. 2017;16:144-157

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Primary causes

  • Neuropathic features – no or weak evidence
  • Cramps
  • Spasticity

Chiò et al. Lancet Neurol. 2017;16:144-157

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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

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Treatment

  • Pharmacological
  • Non-pharmacological

Chiò et al. Lancet Neurol. 2017;16:144-157

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Pharmacological

“There is no evidence from randomised controlled trials about the management of pain in ALS”

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Management

  • WHO analgesic ladder
  • Treat the pain phenotype

Chiò et al. Lancet Neurol. 2017;16:144-157

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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

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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

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“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

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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?
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  • Multidisciplinary
  • Falls assessment
  • Adaptive equipment for ADL
  • Assistive devices for ambulation
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Majmudar et al. Muscle Nerve 50 : 4–13, 2014

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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

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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

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Cognitive profile

Deficits in

  • Executive functions
  • Assessment of verbal fluency
  • Language
  • Social cognition
  • Verbal memory

J Neurol Neurosurg Psychiatry 2016;87:611–619

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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
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  • ALS with behavioural impairment (ALSbi)
  • ALS with cognitive impairment (ALSci)
  • ALScbi
  • ALS-FTD

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2017; 18:153-174

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Neurology 2018;91:e1370-e1380

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Neurology 2018;91:e1370-e1380

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”Phenotypic spectrum of single disease”

van Es et al. Lancet 2017; 390: 2084–98

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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

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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?

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Malnutrition

  • Independent prognostic factor for survival in

MND

  • Eight fold increased risk of death
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  • 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

J Neurol Neurosurg Psychiatry 2011;82:628e634

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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

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Malnutrition

  • Proactive approach
  • Regular nutritional assessment
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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
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Enteral feeding

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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’

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Chhetri et al. Palliat Med. 2017;31:676-677

“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.”

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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/m2
  • 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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25 50 75 100 Overall survival (%) 124 99 107 66 82 48 58 28 Number at risk Up to 10% weight loss More than 10% weight loss 100 200 300 400 Time from gastrostomy insertion (days) Up to 10% weight loss More than 10% weight loss

Lancet Neurol 2015; 14: 702–09

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Gastrostomy – Timing

  • Delay exposes to increased procedural risk and

minimal meaningful benefit

  • Roughly 5% weight loss
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Cramps

Treatment for cramps in amyotrophic lateral sclerosis/motor neuron disease (Review) Baldinger R, Katzberg HD, Weber M 2012, Issue 4. Art. No.: CD004157

“There is no evidence to support the use of any intervention for muscle cramps in ALS/MND”

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Quinine

  • Commonly used
  • Adverse event dose dependent
  • Restricted in USA
  • First choice (NICE)
  • Last resort (AAN)
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Cramps

  • Levatiracetam (EFNS)
  • Mexiletine

Bedlack et al. Amyotroph. Lateral Scler. 2009: 10, 210–215 Weiss et al. Neurology 2016: 86, 1474–1481

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Spasticity – Rx aims

  • Reduce its impact and prevent complications
  • Pain/discomfort
  • Pressure ulcers
  • Contracture
  • Impact on physical activity including personal hygiene
  • Emotional impact

Thompson et al JNNP, 2005;76:459-463

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Principles of management

  • Does it require treatment?
  • Manage triggering/aggravating factors e.g. pain,

constipation

  • Pharmacological
  • Non-pharmacological
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Pharmacological

Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease (Review)

Ashworth NL, Satkunam LE, Deforge D 2012, Issue 2. Art. No.: CD004156

“…moderate intensity endurance type exercises …..No

  • ther medical, surgical or alternative treatment and

therapy has been evaluated in a randomized fashion in this patient population”

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Drug therapies

  • Baclofen
  • Tizanidine
  • Dantrolene
  • Benzodiazepines

Start slow go slow

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Failed orals?

  • Intrathecal baclofen
  • Can improve symptoms
  • Can improve quality of life

Marquardt et al, JNNP 2000, 72, 275–276 McClelland et al. Muscle Nerve 2008: 37, 396–398

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Fatigue

  • Common and persistent
  • Distinct from sleepiness
  • Can occur in up to 83% of patients
  • 44% clinically significant (FSS >40)
  • Worsens slowly and higher rate in later stages

Ramirez et al. Amyotroph Lateral Scler 2008;9:75–80 McElhiney et al. JNNP, 2009;80:1146–9

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Fatigue

  • Persistent
  • Impacts negatively on quality of life
  • “Pure fatigue” an independent entity
  • Most patients (65%)with fatigue are not depressed
  • But most depressed patients (68%) experience fatigue

Ramirez et al. Amyotroph Lateral Scler 2008;9:75–80 McElhiney et al. JNNP, 2009;80:1146–9 Lou et al 2003 Neurology 2003;60:122–3

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Aetiology

  • Multifactorial
  • Respiratory issues
  • Malnutrition
  • Drugs
  • Depression

Ramirez et al. Amyotroph Lateral Scler 2008;9:75–80 McElhiney et al. JNNP, 2009;80:1146–9 Lou et al 2003 Neurology 2003;60:122–3

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Fatigue treatment

Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease (Review)

Gibbons C, Pagnini F, Friede T, Young CA 2018, Issue 1. Art. No.: CD011005

“Impossible to draw firm conclusions about the effectiveness of interventions to improve fatigue for people with ALS/MND as there are few randomised studies, and the quality of available evidence is very low”

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Drug treatments

  • Modafinil

Rabkin et al. Muscle Nerve 2009: 39, 297–303

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Mood disturbances

  • Depression in 11-75%
  • Anxiety in up to 30%
  • Affects quality of life of patients and carers

Kurt et al , CNS Drugs 2007: 21, 279–291 Andersen et al. Eur J Neurol. 2012;19:360-75

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Treatment

  • Depression
  • TCA
  • SSRI
  • Mirtazapine
  • Depression
  • Benzodiazepines
  • Bupropion

Kurt et al , CNS Drugs 2007: 21, 279–291 Andersen et al. Eur J Neurol. 2012;19:360-75

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Emotional lability

  • Roughly 50% exhibit pseudobulbar affect
  • Pathological crying
  • Pathological laughter
  • More prevalent in bulbar illness
  • Can affect quality of life
  • Does not corelate with cognitive impairment

Gallagher JP. Acta Neurol Scand 1989;80:114–117 Brooks et al. Neurology 2004: 63,1364–1370

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Emotional lability

  • Dextromethrophan- quinidine
  • SSRI
  • Amitriptyline

Brooks et al. Neurology 2004: 63,1364–1370 Andersen et al. Eur J Neurol. 2012;19:360-75

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Thank you

Suresh.chhetri@lthtr.nhs.uk