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NEURO 411 > Motor Neuron Disease (Kalra) > Flashcards

Motor Neuron Disease (Kalra) Flashcards

1
Q

Classify weakness, bulk, fasciculations, tone, and reflexes in UMN vs LMN degeneration

A
  • weakness: UMN +, LMN +++
  • bulk: UMN normal, LMN atrophy
  • fasciculations: UMN absent, LMN present
  • tone: UMN increased, LMN decreased
  • reflexes: UMN increased, LMN decreased
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2
Q

TRUE or FALSE: primary lateral sclerosis is purely LMN signs, and progressive muscular atrophy is purely UMN signs

A

FALSE: primary lateral sclerosis = UMN; progressive muscular atrophy = LMN

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3
Q

Does ALS have UMN or LMN signs?

A

both

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4
Q

What does amyotrophy result from?

A

loss of anterior horn cells

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5
Q

What is the most common idiopathic motor neuron disorder?

A

ALS

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6
Q

Who was the first person to describe concurrent upper and lower motor neuron pathology?

A

Charcot

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7
Q

When does onset of ALS occur? What is the male to female ratio?

A
  • anytime in adulthood (around 55 years)
  • male:female, 1.5:1
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8
Q

What are the risk factors for ALS?

A
  • age
  • smoking
  • service in armed forces
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9
Q

What are the clinical features of ALS (i.e. bulbar, limb, respiratory) in general?

A

BULBAR:
- dysarthria
- dysphagia
- dysphonia
- labile affect

LIMB:
- weakness
- cramps
- stiffness

RESPIRATORY:
- resp muscles weaken…major cause of death from ALS

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10
Q

What functions are spared in ALS?

A
  • sensation
  • sphincter
  • (cognitive)
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11
Q

What are the clinical features of upper and lower extremities in ALS?

A

UPPER EXTREMITY:
- hand - fine motor activities
- shoulder - proximal arm weakness; “frozen shoulder”
- axial - stooped posture

LOWER EXTREMITY:
- foot drop
- difficulty with chairs, stairs

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12
Q

What are the respiratory features of ALS?

A
  • dyspnea (shortness of breath)
  • orthopnea (difficulty breathing when lying down)
  • sleep disordered breathing
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13
Q

What are the frontotemporal features of ALS?

A
  • cognitive impairments - executive, language, social cognition
  • behavioural impairments - apathy
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14
Q

In what ways does cognitive impairment affect individuals with ALS?

A
  • impact decision making capacity
  • reduce survival
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15
Q

Is there decreased grey matter or white matter in ALS? In which areas do you see these decreases?

A
  • decreased grey matter
  • bilateral motor cortex
  • left frontal/temporal cortex
  • ACC
  • left lenticular cortex
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16
Q

What gene expansion is associated with ALS?

A

C9ORF72

17
Q

Degeneration of which part of the brain is associated with the extra-motor pathology of ALS?

A
  • frontotemporal lobar degeneration (FTLD)
  • neuronal loss, gliosis
  • superficial spongiform degeneration in layers II/III
18
Q

What is the molecular link between ALS and FTD?

A

TDP-43 protein

19
Q

What is the genetic link between ALS and FTD?

A

C9ORF72

20
Q

What is a marker of neurointegrity that can be quantified in vivo? What does a decrease in this marker indicate?

A
  • NAA
  • decrease NAA = increase disease progression
21
Q

What are the 4 neuropathological stages of pathological TDP-43 distribution in ALS?

A
  • stage 1: motor cortex, bulbar and spinal somatomotor neurons
  • stage 2: reticular formation, precerebellar nuclei, thalamus
  • stage 3: prefrontal neocortex, basal ganglia
  • stage 4: anteromedial temporal lobe, hippocampal formation
22
Q

Describe the ALS-FTD spectrum and the PMA-ALS-PLS spectrum.

A

see slide 24

23
Q

What is the ALS Functional Rating Scale used for? What is the average decline? What percent change in slope is considered clinically meaningful?

A
  • important endpoint in contemporary clinical trials
  • measure of disability; monitor disease progression
  • average decline of 1.02 points/month
  • 20-25%
24
Q

What are challenges resulting from extreme heterogeneity?

A
  • delayed diagnosis
  • delayed treatment
  • unable to predict prognosis
  • unable to predict treatment response
  • clinical trial failures
25
Q

How is ALS largely diagnosed? Describe the work up.

A
  • largely diagnoised by ruling out other diseases
  • NCS/EMG –> rule out neuropathy, subclinical denervation, staging
  • MRI brain or cervical spine
  • blood tests
  • pulmonary function tests
26
Q

What condition mimics ALS?

A

cervical spondylosis

27
Q

What is the WHO definition of palliative care?

A

the active total care of the patient whose disease is not responsive to curative therapy

28
Q

What are the principles of management of ALS?

A
  • patient autonomy
  • holistic and team approach
  • patient participation in clinical trials
  • effective use of home and hospice care
  • close communication between all parties involved
  • PALLIATIVE CARE
29
Q

What are the goals of ALS management?

A

maximize quality of life and quality of death

30
Q

What are some disease modifying treatments for ALS? Describe each.

A

RILUZOLE
- anti-glutamatergic
- prolong survival 3-6 months

EDARAVONE
- anti-oxidant
- slow disability 33%

AMX0035
- phenylbutyrate + taurursodiol
- reduces ER stress and mitochondrial dysfuntion
- slow disability, prolong survival

TOFERSON (miracle drug)
- ASO
- SOD1

31
Q

How do you manage sialorrhea/salivation in ALS?

A

anticholinergics

32
Q

Which drug can be used to manage pseudobulbar symtpoms, anxiety, and insomnia in ALS?

A

amitryptyline

33
Q

What can BiPAP be used for in ALS?

A
  • best intervention for respiratory support
  • provide + pressure
34
Q

What is PEG used for in ALS?

A

feeding tube

NEURO 411 (12 decks)

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