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Neuroscience and Rehabilitation 5MPT2015 (Y2) > Motor Neurone Disease therapy & management > Flashcards

Motor Neurone Disease therapy & management Flashcards

1
Q

What is Motor Neurone Disease?

A 
A group of related Neuromuscular Diseases
including:
- Amyotrophic lateral sclerosis (ALS)
- Primary lateral sclerosis
- Progressive muscular atrophy
- Progressive bulbar palsy
- Spinal muscular atrophy

Affects the motor nerves in the CNS, PNS or both.

Nerves become damaged & muscles they control
become weaken & waste.

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2
Q

Is MND a progressive degenerative disease?

A

yes

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3
Q

What are the risk factors associated with MND?

A

• 1 in 300 risk of developing MND
• Occurs in adults or children, depending on the type.
• More likely to affect men than women.
• Inherited forms of the condition may be present (1 in 10 people with
MND, there is evidence of a family history with the disease).

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4
Q

What is the cause of MND?

A

• 5-10% of MND cases are familial, 90-95% occur sporadic
• Some genes for familial and sporadic MND are known.
• The C9orf72 gene now thought to be:
• the most common genetic cause of front temporal dementia (FTD)
• It also counts for 24% of genetic defects of familial MND and 4% in sporadic
MND
• Environmental contribution?

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5
Q

What occurs in terms of anatomy in MND?

A 
• In motor neurone disease
connections from the brain to
the spinal cord (UMN) and from
the spinal cord to the muscle
(LMN) may die.

• This results in a loss of muscle
control and atrophy

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6
Q

What motor pathways are Upper motor neurones?

A
  • Corticospinal tract

* Corticobulbar tract

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7
Q

What motor pathways are Lower motor neurones?

A
  • Anterior horn cell
  • Efferent peripheral
    pathways
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8
Q

What are the symptoms of MND?

A
  • muscle weakness
  • muscle cramps and spasms
  • stiff joints
  • pain or discomfort
  • speech and communication problems
  • swallowing difficulties
  • saliva problems
  • weakened coughing
  • breathing problems
  • emotional lability
  • changes to thinking and behaviour
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9
Q

What are the signs for upper MND/ UMN?

A
  • Weakness
  • Hyper-reflexia
  • Hypertonia
  • Clonus
  • Babinski
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10
Q

What are the signs for lower MND/ LMN?

A
  • Weakness
  • Fasciculations
  • Hypo-reflexia
  • Hypotonia
  • Atrophy
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11
Q

How do you diagnose MND?

A

• Exclude other etiologies
• History of onset
• Clinical presence of motor neuron signs:
• UMN signs
• LMN signs
• Limb onset e.g. muscle weakness, cramps, twitching.
• Bulbar onset e.g. swallow (dysphagia) or speech(dysarthria)
difficulties.
• Resp onset e.g. SOB, weak cough, shallow breaths

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12
Q

What is the progression of MND like?

A

• TDP-43 protein a core component of MND pathology but unclear of
role.
• No clearly defined stages
• Patients do not follow a set pattern
• Variable distribution of weakness + clinical course
• Eventually general weakness so profound patient confined to bed or
wheelchair
• Many need admission to special units

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13
Q

What are good/poor prognosis

A 
Better prognosis:
• The rate of change in the first
year
• Pure UMN or pure LMN
syndromes
• Regional syndromes (upper
or lower limb)

Poor Prognosis
• Elderly
• Bulbar onset
• Early respiratory involvement

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14
Q

What’s the most common phenotype?

A

ALS

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15
Q

What’s the life expectancy of a person with ALS?

A
  • Most people die 2-3 years
  • 25% alive at 5 years
  • 5-10% alive at 10 years
  • Stephen Hawking – ALS
  • lived for decades so there are exceptions.
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16
Q

What are the phenotypes and life expectancy for each?

There’s 4

A
  • Amyotrophic Lateral Sclerosis (Lou Gehrig’s)
  • Prognosis 3-5 yrs
  • Progressive bulbar palsy
  • Prognosis 6m-3yrs
  • Progressive muscle atrophy
  • Prognosis 5yrs +
  • Primary lateral sclerosis
  • Possible normal life span
17
Q

What’s the management for MND (overview)

A
  • No cure
  • MDT approach
  • Maintain functional ability (use ICF framework)
  • Enabling to live life as full as possible
  • Early diagnosis
  • Provision of medication and aids
  • Communication
  • Advanced care planning
18
Q

What are the common cognitive impairments for MND patients?

A
  • Dysphagia

- Dysarthria

19
Q

What are the common impairments for MND patients?

A

Respiratory impairment
Muscle weakness, Muscle
atrophy, Muscle cramps,
Spasticity, Loss of range, pain

20
Q

Take home points about cognition of MND patients.

A 
• Almost all have capacity
• May have difficulty with
learning new technologies
• May be less engaged with
health professionals / family
• May be less able to
understand carers emotional
needs / exhaustion
21
Q

What is Dysarthria?

A 
• When speech becomes slurred (‘drunk’)
• Making it difficult for others to understand them
• Complex and distressing
• Common towards end of life
• Exacerbated by respiratory weakness
• Social isolation
- avoidance from both patient and others
• Difficulty expressing own wishes
- Medical management, end of life
22
Q

What Augmentative and Alternative

communication - AAC, is out there?

A 
• Text to speech apps
• Voice banking / phrase banking
• To start early
• Eye Gaze technology
• Scanning devices – switch operated
• E-tran frame
• Alphabet boards
but all the above need literacy, fluency and
common language
23
Q

How help is there for Dysphagia and Nutrition?

A
  • Speech and language therapy, dietetic input
  • Common for most patients towards end of life
  • Problem of 3 parts:
  • Oral care
  • Sialorrhea: hypersalivation → drooling
  • Swallowing
  • Management:
  • Diet and fluid modification,
  • Safety strategies: posture, adapted cutlery, feeding techniques
  • Risk feeding and/ or enteral feeding options
  • timing of interventions : clinical safety and patient choice
  • PEGS
  • RIGS
  • Taste for pleasure
24
Q

What are the Dysphagia – red flags?

A 
Extended mealtimes
• Dietary modification
• Loss of appetite / interest in
meals
• Weight loss of > 5% in 3 months
- Malnutrition Universal
Screening Tool
- dietetic referral
• Chest infections
25
Q

What is Dyspnoea and how prevalent is it in MND patients?

A

• Dyspnoea – difficulty breathing - is a problem for
up to 85% of people with MND
• Quality of life correlates with respiratory
symptoms
• Often described as the terminal event in MND

26
Q

What are the Red flags - Dyspnoea?

A
  • Orthopnoea - sob when lying flat
  • Unable to speak in sentences
  • Can’t sing / cough / blow their nose
  • Can’t eat and breathe
  • Nocturnal hypoventilation
  • FVC 70 - 50 % of predicted
27
Q

What is the role of exercise in MND?

A

• Maintain/ improve muscles NOT affected by MND
• Maintain flexibility of muscles that are affected
• Prevent fatigue and disuse atrophy
• Cochrane Review 2013 (Dal Bello-Has & Florence JM):
• Number of included studies small
• Unable to determine if strengthening exercises are
beneficial or harmful.
• No reported adverse events due to exercise
• Resistance, Endurance, or Stretching/Range of Motion
exercise safe (RCT Clawson LL et al., 2018)

28
Q

What type of exercise dosage is recommended for MND patients?

A
  • Select a mode of exercise with minimal risk
  • recumbent stationary bike as opposed to treadmill
  • Moderate, submaximal level
  • Consider carer support
  • Supply orthotics as required
  • Avoid high-resistance exercise
  • Progression to heavier loads depends on disease stage
  • Don’t exercise muscles < grade 3/5
  • Avoid eccentric exercise
29
Q

What can be done to ease cramps in MND patients?

A

• A cramp is a sudden, involuntary painful
contraction
• Many people with MND experience cramps
- Ranging from mild cramps to very severe, painful cramps.

• To consider quinine as first-line treatment
- If not effective, tolerated or contraindicated,
consider baclofen or if this is not possible
consider tizanidine, dantrolene or gabapentin

30
Q

What can be done to ease spacisticty in MND patients?

A

• Consider baclofen, tizanidine, dantrolene or gabapentin to treat
muscle stiffness, spasticity or increased tone
• Consider referral to specialist service for treatment of severe
spasticity (NICE GUIDELINES, 2016)
• Nabiximols, a cannabis-based oral spray, significantly improved
spasticity symptoms in combination with antispasticity drugs in
patients with MND
• in a randomized phase 2 clinical trial
• Findings need to be confirmed in phase 3 trials

31
Q

What can be done to ease fatigue in MND patients?

A

• No firm conclusions can be drawn about the effectiveness of
interventions to improve fatigue for people with MND
• Few randomised studies
• Quality of available evidence is very low

32
Q

What can be done to help mobility in MND patients?

A 
• Maintain standing /walking as long as
possible
• Ensure equipment meet changing needs
• Refer to wheelchair services without
delay
• Manual and/or powered wheelchair
• Walking aids
• Rollator frame, 4WW, crutches, stick
33
Q

What can be done to help dexterity in MND patients?

A

Thickened handles on cutlery, pens, toothbrushes

to assist with weak grip

Neuroscience and Rehabilitation 5MPT2015 (Y2) (20 decks)

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