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Neuro > (2) Motor Neurone Disease (ALS) > Flashcards

(2) Motor Neurone Disease (ALS) Flashcards

1
Q

What is a motor neurone disease?

A

degenerative disorder of upper and lower motor neurones

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2
Q

Where do the pathological changes take place in an MND?

A
  • anterior horn cells of sc
  • cranial nerve
  • medulla
  • corticospinal tract
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3
Q

What is amyotrophy?

A

muscle wasting

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4
Q

What is lateral sclerosis?

A

scarring of uppermost pathways of the SC

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5
Q

Where does the scarring happen in lateral sclerosis?

A

descending upper motor neuron pathways (corticospinal tracts)

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6
Q

What are signs of lateral sclerosis?

A
  • spasticity
  • exaggerated reflexes
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7
Q

What is the epidemiology of ALS?

A
  • male > female
  • 40-70 years
  • life expectancy ~3-10 years
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8
Q

What are the causes of ALS?

A
  • 10-20% dominance one parental gene
  • 90% not inherited
  • environmental triggers
  • defect gene controlling glutamate level in brain
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9
Q

What is primary muscular atrophy?

A
  • primary degeneration in anterior horn cells

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10
Q

What is progressive bulbar palsy?

A

degeneration in cranial nerve nuclei in medulla

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10
Q

What are signs of primary muscular atrophy?

A
  • muscle wasting & fasciculation
  • painful muscle cramps
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11
Q

What are signs of progressive bulbar palsy?

A

paralysis tongue, palate & lower facial muscles

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12
Q

What are signs of ALS?

A
  • UMNL
  • spasticity
  • weakness usually LL
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13
Q

What are signs of an UMNL (5)?

A
  • slurred speech
  • altered emotional control
  • spasticity
  • brisk reflexes
  • decreased dexterity
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14
Q

What are signs of a LMNL (5)?

A
  • weakness
  • muscle wasting
  • fasciculations
  • decreased/absent reflexes
  • cramping
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15
Q

What are ‘bulbar’ signs?

A
  • dysphagia
  • aspiration
  • slurred/loss speech
  • breathing problems
  • weak cough
16
Q

What is the course of ALS?

A
  • Insidious onset (bulbar signs)
  • 50% motor neurones can be lost before weakness evident
  • progressive weakness leads to death ~3-4 years from onset
17
Q

What are investigations carried out for ALS?

A
  • EMG
  • Muscle biopsy
  • nerve conduction studies
  • blood tests
  • lumbar puncture
18
Q

How can EMG detect a MND?

A

can identify LMN involvement or denervation prior to muscle wasting

19
Q

What is a motor unit?

A

nerve fibre and motor fibres it supplies within the muscle

20
Q

What is a fibrillation potential?

A

action potentials that arise from single muscle fibres

21
Q

What do nerve conduction studies do?

A

measures speed conduction of electrical impulse through a nerve

22
Q

What is the El Escorial Criteria?

A
  • Classifies ALS diagnosis as possible, probable, and definite
  • requires presence of LMN degeneration
23
Q

What is definite ALS according to the El Escorial Criteria?

A

UMN & LMN signs 3 regions

24
Q

What is probable ALS according to the El Escorial Criteria?

A
  • UMN & LMN signs in at least 2 regions
  • UMN signs above LMN signs
25
Q

What is possible ALS according to the El Escorial Criteria?

A
  • UMN & LMN signs in at least 1 region
  • UMN signs alone in 2/more regions
  • LMN signs above UMN
26
Q

What is suspected ALS according to the El Escorial Criteria?

A

LMN signs in only 2 or more regions

27
Q

What are problems experienced with MND (6)?

A
  • progressive weakness
  • spasticity
  • pain, cramps
  • Dysphagia, Dysarthria & Dyspnoea
  • tiredness
  • pressure sores
28
Q

What is advice for MND patients?

A
  • maintain activity level
  • low stress, low impact
  • avoid high resistance
  • build exercises slowly & monitor fatigue
  • CV exercise, stretching
  • don’t use all energy on exercise
29
Q

What is the role of the physio with a patient with MND?

A
  • baseline Ax & monitor
  • muscle strength, length & ROM
  • promote independence
  • monitor resp
  • info & educate
  • advice about exercise
30
Q

What is the physiotherapy Tx for MND (7)?

A
  • functional Ax
  • maintain ROM
  • special seating
  • educate carers
  • positioning
  • counselling
  • skin care
31
Q

What is the overall aim of treatment for an individual with MND?

A
  • maintenance independence
  • support during course of disease
32
Q

What are the NICE guidelines for MND equipment & adaptations for ADL (5)?

A
  • ADL’s should all be considered
  • mobility & avoiding falls from loss dexterity
  • adaptations to home environment
  • need for assistive technologies
  • prompt access & Ax for funding home adaptations
33
Q

What is Riluzole?

A
  • disease modifying drug
  • slows progression symptoms
  • increases survival 2-3 months
34
Q

What is a general objective Ax for a MND patient?

A
  • ALSFRS
  • Muscle strength
  • mobility
  • QOL questionnaire
  • fatigue questionnaire
35
Q

What is the ALSFRS used for?

A
  • monitor progression
  • includes dyspnoea, orthopnoea, & ventilatory support
  • graded 4 (normal) to 0 (severe)
  • 12 categories

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