ALS Flashcards

(36 cards)

1
Q

What is ALS

A

A fatal, progressive neurodegenerative disorder

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2
Q

What does ALS stand for and broken up meaning

A

Muscle atrophy

Degeneration and eventual death of motor neurons in the cortex, brainstem, anterior horn cells and pyramidal tracts

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3
Q

Two forms

A

Familial

sporadic (90%)

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4
Q

Risk factors for sporadic ALS

A
Smoking
Exposure to certain metals
Electrical work
Military 
Environment*
Head trauma*
Excessive physical activity*
Genetics*
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5
Q

Pathophys of ALS

A

Progressive deterioration of motor nerve cells in brain and spinal cord

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6
Q

What’s the protein found misfolded in the cytoplasm

A

TDP 43

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7
Q

Links ALS to FTD

A

TDP 43

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8
Q

General clinical manifestations of ALS

A
UMN
LMN
Limb and spine
Bulbar
Pseudobulbar
Respiratory
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9
Q

UMN

A

Spasticity
HyPER reflexia and clonus
Pseudobulbar effect

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10
Q

LMN

A

Muscle weak and atrophy
Fasciculations
Cramps
HYPOreflexia

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11
Q

Limb and spine

A

Asymmetric limb weak most common

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12
Q

What’s affected first in limb issues

A

Finger extensors and ankle DF affected first

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13
Q

Cervical

A

Weak extensors- head flop

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14
Q

Bulbar from brainstem

A

Dysphagia
Dysarthria
Sialorrhea

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15
Q

Pseudobulbar

A

Inappropriate crying and laughter

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16
Q

Respiratory issues (6)

A
DOE or with lying flat
Weak cough
Use aux muscles more
Daytime sleepy
Less concentration
Headaches
17
Q

Signs and Sx of FTD (5)

A
Emotional blunting
Loss of insight
Decreased social conduct
Personality changes 
Decreased word generation
18
Q

ALS does NOT affect? (5)

A
Bowel and bladder
Internal organs
Sexual function
Eye muscles 
Sensation
19
Q

One of the most common Sx with ALS

20
Q

Weakness with specific patterns

A

Doesn’t improve or get worse with exercise
Distal limbs affected first
Spine extensors most affected

21
Q

Presence of both

A

UMN and LMN Sx

22
Q

3 other common themes in ppl with ALS

A

Fatigue
Difficulty with lying flat
Personality changes

23
Q

Cranial nerves involved

A

PBP = progressive bulbar palsy

24
Q

UMN

A

Primary lateral sclerosis (PLS)

25
LMN
Progressive muscle atrophy (PMA)
26
Which one? | Pt complains of fatigue, muscle weak and cramping
PMA b/c theyre LMN
27
Which one? | Pt complains of fatigue, stiffness and crying inappropriately
PLS b/c UMN
28
Which one? | Pt complains of fatigue, weak, stiff and fasciculations
ALS b/c both UMN and LMN
29
3 meds as treatment
No cure but Riluzole Nuedexta Radicava
30
Can increase survival by 2-3 months
Riluzole
31
May help bulbar Sx med
Nuedexta
32
New treatment May decrease slope of decline Very expensive No long term results
Radicava
33
Exercise guidelines
Avoid eccentric contraction | Avoid high reps and intensity
34
Important exercise for ALS
STRETCH | 30 sec, 2-4 x/day
35
Managing respiratory issues
NIPPV (biPAP) | NOT OXYGEN
36
Psychosocial factors of ALS
Depression Insomnia Difficult decisions