MND intro Flashcards

1
Q

Motor Neuron Disease?

A
  • Devastating condition causing progressive weakness and eventually death, usually as a result of respiratory failure or aspiration
  • Untreatable and rapidly progressive neurodegenerative condition
  • Mainly clinical diagnosis-clinical examination rather than lab or scan diagnosis
  • Muscle weakness and potentially problems with speech, swallow & breathing
  • Upper and/or Lower motor neurone signs without sensory problems
  • Focal onset and continuous spread, finally generalized paresis-one part and then entire body
  • MND exhibits variable clinical phenotypes!-it’s not a single pheno, MND variable hastesh, fk nakon ba ye pattern khasi dare
  • symptom/presentation
  • Amyotrophic lateral sclerosis is the most common subtype of MND
  • Cognitive impairment (behavioural changes) is a feature of MND
  • 90 % ‘sporadic-no strong family history’ and 10% ‘familial
  • both female and male
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2
Q

——is the most common motor neuron disease (MND) phenotype in adults which is the mixture of both

A

ALS

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3
Q

Clinical presentation?

A
  • Classically, there is a combination ofupper motor neuron and lower motor neuron signs.
  • Upper motor neuron signs include spasticity, hyperreflexia and upgoing plantars (though they are often down going in MND)
  • Lower motor neuron signs include fasciculations, and later atrophy
  • Generally, the eye and sphincter muscles are spared until late in the disease course and sensory disturbance is NOT seen
  • Focal onset and continuous spread, finally generalised paresis
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4
Q

Clinical patterns?

A

The four clinical groups are:

  • Spinal ALS
  • Bulbar ALS
  • Pure lower motor neurone presentation with weakness, muscle wasting and fasciculations, usually starting in one limb and gradually spreading to involve other adjacent spinal segments
  • Primary lateral sclerosis - only upper motor neuron features (1-2% - rare)
    • Confined to upper motor neurones, causing a slowly progressive tetraparesis and pseudobulbar palsy
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5
Q

Spinal ALS representation?

A
  • the classic MND syndrome
  • Simultaneous involvement of upper and lower motor neurones, usually in one limb, spreading gradually to other limbs and trunk muscles
  • muscle wasting
  • Loss of tone or contractures
  • Split hand syndrome - preferential wasting of thenar group is a typical pattern of atrophy seen in ALS
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6
Q

Bulbar dysfunction/ALS?

A
  • early tongue and bulbar involvement
  • Dysarthria, dysphagia, nasal regurgitation of fluids and choking are the presenting symptoms
  • leads to ALS
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7
Q

Progressive muscular atrophy?

A
  • only lower motor neurone features
  • Pure lower motor neurone presentation with weakness, muscle wasting and fasciculations, usually starting in one limb and gradually spreading to involve other adjacent spinal segments
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8
Q

Primary lateral sclerosis?

A
  • only upper motor neuron features (1-2% - rare)
  • Confined to upper motor neurones, causing a slowly progressive tetraparesis and pseudobulbar palsy
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9
Q
A
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10
Q

Upper and lower motor neuron signs in MND?

A
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11
Q

Investigations?

A
  • Diagnosis is largely clinical
  • Investigations allow exclusion of other disorders and may confirm subclinical involvement of muscle groups, such as paraspinal muscles
  • Denervation of muscles due to degeneration of lower motor neurones is confirmed by EMG
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12
Q

On-going management

A

-regular rapid ongoing assessments – patients seen 4-6 weekly if needed
- Key worker assessing needs and coordination of care like MND nurses
- Communication needs - speech therapy, AAC
- Nutritional needs - dieticians, gastrostomy
- Metabolic rate in MND is doubled - weight loss of >10% at diagnosis is a poor survival indicator
- Respiratory needs - assessment, home ventilation
- Riluzole is a sodium-channel blocker that inhibits glutamate release - slows progression slightly, increasing life expectancy by 3–4 months on average

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13
Q

Symptomatic treatments

A
  • Sialorrhoea - hyoscine/buscopan, glycopyrronium, botox, suction
  • Nutrition - supplements/thickeners, liquid drug preparations
  • Bulbar dysfunction - early communicator, nutritional support, care for upper respiratory tract
  • Limb dysfunction - washing, dressing, feeding, turning in bed, mobility aids/hoists often necessary
  • Muscle cramps - quinine, baclofen
  • Muscle spasms - baclofen, tizanidine, dantroline, gabapentin
  • Emotional lability - sometimes treated with antidepressants
  • Cognitive impairment - always be aware of capacity
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14
Q

Red flag signs of respiratory failure?

A

Breathlessness
Orthopnea
Recurrent chest infection
Disturbed sleep
Non-refreshed sleep
Nightmares
Daytime sleepiness
Poor concentration

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