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Semester 5 PMNM Exam 7 > Anterior Horn Cell Disorders - ALS > Flashcards

Anterior Horn Cell Disorders - ALS Flashcards

1
Q

ALS is what

A

Amyotrophic Lateral Sclerosis

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2
Q

Meaning of ALS - variant

A

Can refer to several adult onset conditions characterized by progressive degeneration of motor neurons (variants)

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3
Q

Meaning of ALS - Typical ALS

A

A specific form of motor neuron disease, involving both upper and lower motor neuron signs

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4
Q

ALS - Define

A

Motor neuron disease

Adult onset, progressive, motor neuron system degeneration disease (both UMN and LMN)

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5
Q

Amyotrophic means what

A

No muscle nourishment
Muscle atrophy, weakness, fasciculations
LMN signs

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6
Q

Lateral Sclerosis means what

A 
Degeneration and scarring (due to gliosis) of CST in spinal cord 
UMN signs (inc DTR, spasticity)
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7
Q

S/S ALS - UMN

A

Spasticity
Hyperreflexia
Pathological reflexes

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8
Q

S/S ALS - LMN

A 
MM weakness
MM atrophy
Fasciculations
Hyporeflexia
Hypotonicity 
MM cramps
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9
Q

S/S - Bulbar signs

A

Dysarthria
Dysphagia
Sialorrhea (salivation)
Pseudobulbar palsy

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10
Q

S/S Respiratory

A

Nocturnal resp. difficulty
Exertional dyspnea
Accessory mm use
Parodoxical breathing

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11
Q

Other s/s

A 
Fatigue
Weight loss
Cochexia
Tendon shortening
Joint contractures
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12
Q

2 types ALS - Ways to get it

A

Familial ALS

Spontaneous/Sporadic ALS

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13
Q

Familial ALS

A

Less than 10% of all ALS cases

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14
Q

Spontaneous/Sporadic ALS

A

90% of all cases

Etiology is unknown

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15
Q

Prognosis

A

Non curable, is fatal

Median survival is 3 yrs (20% will survive up to 10 yrs)

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16
Q

Better prognosis with what

A

early age

onset of limb weakness initially (as opposed to bulbar initially)

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17
Q

ALS - cause of death is commonly

A

respiratory failure

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18
Q

Main area of research for the cause

A

Glutamate toxicity
Glutamate is primary excitatory NT used by the motor system - it is normally broken down by a protein but people with ALS are deficient in that protein so there is a build up or clogging at the synaptic cleft
Excess amount of glutamate is toxic

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19
Q

Other possible causes

A

SOD1 dysfunction
Microglial mediated neurotoxicity
Mitochondrial dysfunction

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20
Q

SOD1 as a cause

A

Acts as an anti-oxidant in the cell and eliminates free radicals - not working properly in someone with ALS though so leads to oxidative damage and kills neurons

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21
Q

Microglial mediated neurotoxicity as a cause

A

Microglia - main form of active immune defense in the CNS (surrounds the neuroglia) - with ALS there is a proliferation of microglia (too many of them) - and leads to enhanced inflammatory response which can lead to MN degeneration

22
Q

Mitochondrial dysfunction as a cause

A

Mitochondria regulating cell death - there is altered mitochondrial enzyme function though which can lead to oxidative damage and increased apoptosis

23
Q

Diagnostic parameters

A 
Both UMN and LMN signs are present 
Absence of pain or sensory changes 
Intact bowel and bladder
Extraocular mm spared
Diagnostic imaging to rule other things out
24
Q

Diagnositc parameters - why is there an absence of pain and sensory changes

A

Spinocerebellar and sensory systems are spared

25
Q

Diagnostic Parameters - What else besides diagnostic imaging is done

A 
EMG 
Muscle biopsies
NCV
Clinical presentation
Clinical lab
26
Q

Treatment - medications for ALS

A

Riluzole - gluamate antagonist
The only drug approved for tx fo ALS
Effective in inc life 2-3 months

27
Q

Treatment - medicatiosn for people with ALS - for symptoms

A 
Control salivation
Antispasticity
Mucolytics
Anti-anxiety
Anti-depressants
Injectable B12
28
Q

In the initial stages of the disease, most will present with

A

focal weakness, typically in the distal leg, hand, or bulbar mm
Often see foot drop, inability to use hand with ADL, difficulty with speech/swallowing

29
Q

Clinical presentation - Cog

A

Executive function, memory loss in 50% of cases

About 15% have frontotemporal dementia

30
Q

Tx - nutrition

A

Ketogenic diet/supplementation
SLP/nutritionist
PEG tube

31
Q

Tx - communication

A

Speech language pathologist (SLP)

32
Q

Tx - respiratory intervention

A

Need to manage secretions with coughing techniques
When VC decreases to 50% of predicted, may need to assist more with positive airway pressure
Eventually need to decide on ventilatory support with tracheostomy of hospice

33
Q

Goals of PT

A

Optimize remaining function early on
Maintain functional mobility
Maximize QOL

34
Q

Stage 1 description

A

Independent in mobility and ADL, weakness in a specific group of mm (often is a distal limb)

35
Q

What to do in stage 1

A 
Energy conservation
Home modification
Continue normal activities
AROM/Stertching
Strengthening of unaffected 
Aerobic exercise at submax
36
Q

Stage 2 description

A

Moderate weakness in groups of muscles

Usually distally

37
Q

Stage 2 - what to do

A 
Adaptive equipment
AROM/stretching
Strengthening of unaffected 
Aerobic as able 
AAROM/PROM via caregivers to prevent contractures and pain
38
Q

Strengthening in stages 1 and 2

A

Exercise in several brief periods throughout the day - 2 to 3 sessions, 15 to 45 minutes total
Lighter weights and less reps

39
Q

Stage 3 descrition

A

Mild-Moderate limitation of function, severe foot drop, marked hand weakness, neck flexion due to neck extensor weakness, STS deficits

40
Q

Stage 3 what to do

A 
AFOs/splints/soft collar
W/c for longer distances
Lift devices for chairs
PROM/AAROM
Deep breathing, chest expansion/stretching
41
Q

Stage 4 description

A

Severe weakness in LEs

42
Q

Stage 4 what to do

A 
WC - eval for power WC
AAROM/PROM
Isometric contraction
Deep breathing
Skin inspection/pressure relieving strategies
43
Q

Stage 5 description

A

Moderate to sever deterioration or mobility and endurance

44
Q

Stage 5 what to do

A

Power wc
Lift for transfers at home
Frequent position changes
Pain - gentle AAROM/PROM, thermal modalities, splinting
Head neutral position - might need to progress to rigid collar

45
Q

Stage 6 description

A

End stage, dependent

46
Q

Stage 6 what to do

A

Reposition
Pain management
Progressive respiratory distress - chest PT

47
Q

The role of exercise

A

Strengthening and aerobic exercises are effective

Cochrane review

48
Q

Stretching

A

No literature support but we know it can help with weakness that will lead to imbalance, it can help prevent mm shortening and contractures

49
Q

RCTs with exercise - strengthening

A

2 groups - 1 strengthening and stretching and the other was just stretching
Group with strengthening had improved function on outcome measure and improved QOL
Exercised 3/wk for 6 months

50
Q

Studies with aerobic exercise - mouse models

A

Mouse models show moderate intensity can increase survival, but high intensity worsens it

51
Q

Studies with aerobic exercise - RCT that had one group do aerobic program of choice and the other do their normal ADL

A

At 3 months, aerobic group had improved function and less spasticity
At 6 months, there was less of a difference between groups

Semester 5 PMNM Exam 7 (7 decks)

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