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Flashcards in GBS Deck (42):
1

Axonopathy: definition, chronic examples

Wallerian degeneration of the axon
Acute- regrowth = 3-4mm/day
Cronic- no regrowth: DM, toxins, hereditary (CMT)

2

Myelinopathy: definition, examples

degeneration of myelin producing cells (Schwann- peripheral, oligodendrocytes- central)
example = GBS

3

Neuronopathy: definition, examples

damage to the cell bodies (no regrowth possible)
examples = ALS, SMA, Polio

4

Neuropathic Weakness

weaker distal > proximal
example = polyneuropathy (GBS)

5

Myopathic Weakness

weaker proximal > distal

6

Cause of Mortality in GBS

inability to breath; complications from respiratory or autonomic issues

7

Etiology/Pathology of GBS

Autoimmune illness
inflammatory process --> T cells create antibodies that target myelin and the axons --> macrophages destroy schwann cell or axon

8

Clinical symptoms of GBS used in diagnosis

Flaccid paralysis, symmetrically ascending (no spasticity)
Respiratory failure and ANS dysfunction
diminished or absent DTRs
slowed nerve conduction velocity
CSF with elevated proteins
nonspecific lab tests

9

3 clinical subtypes of GBS

AIDP- acute inflammatory demyelinating polyneuropathy
AMAN- acute motor axonal neuropathy
AMSN- acute motor-sensory axonal neuropathy

10

AIDP

only myelin affected (demyelination)
Motor neurons > sensory (but could be reversed)
most often in western hemisphere (80%)
EMG = prolonged motor response
*best prognosis

11

AMAN

20% (2nd most common type in western hem)
axonal damage (degeneration)
EMG = decreased conduction velocity, + spike waves

12

AMSAN

Rare!
Axon affected (degeneration)
EMG = decreased conduction velocity, + spike waves

13

Miller-Fisher Syndrome

Allied with GBS
more brainstem involvement- cranial nerves > axial muscles
issues with eye movements, swallowing, facial movements

14

Only way to know type of GBS

EMG analysis!
AIDP- increased motor latencies
AMAN/AMSAN- decreased conduction velocity, (+) spike waves

15

Recovery period for GBS

up to 2 years

16

Poorer outcomes associated with:

1. Quadriplegia
2. respiratory dependency > 1m
3. failure to show improvement within 3 weeks of plateau
4. AMAN/AMSAN v AIDP

17

3 temporal phases of GBS

Acute/Before stabilization --> deterioration
Nadir/Plateau phase --> stabilization
Recovery phase --> acute care - rehab/homecare/OP

18

Acute/Before stabilization stage
- location/what's happening
- pattern of weakness
- peak weakness

deterioration- in ICU
symmetrical, ascending weakness
Peak of weakness: 3-21 days

19

Nadir/Plateau Phase
- location/what's happening
- peak weakness

stabilization/quiet period- in ICU (ventilation) or ward (no ventilation)
lowest the pt will get (functionally)

20

Recovery Phase
- location/what's happening
- duration
- pattern of return

location depends on severity (could be in the ICU if weaning off ventilator)
up to 2 yrs
Return in reverse order: proximal before distal

21

5 phases of Pt's Perspective of Recovery

1. Experiencing dependency (unable to do anything)
2. Encountering helplessness
3. Wanting to know more about GBS (most at rehab level)
4. Discovering inner strength
5. Regaining independence

22

Hughe's Disability Scale:
Purpose
Study that followed pts in the UK findings

Grades GBS based on functionality/presence of temporal summation
UK study found that:
1. more acutely, more impaired, many on ventilator
2. by 2 years, no one on ventilation, some in w/c, all becoming less impaired

23

Hughe's Disability scale: Ordinal scale

0 = healthy, all functional activities as before
1 = minor signs or symptoms, able to run
2 = able to walk > 10m without symptoms, unable to run
3 = able to walk more than 10m with assistance
4 = requires wheel chair
5 = requiring assisted ventilation for at least part of the day
6 = dead due to GBS

24

Why is running a good criteria for ability to recruit more and have temporal summation

Running requires speed, which requires at least 1 of 2 things:
1. increase firing rate: temporal summation (rate coding)
- requires myelin to send down AP
2. increase # of muscles firing: engaging more alpha motor neurons (recruitment)

25

Plasmaphoresis: how long is it administered, what is done?

administered for 5 days to eliminate items in plasma causing inflammation
- all blood withdrawn
- replace plasma with saline and electrolytes

26

Intravenous Immunoglobulin: how long is it administered, why?

3-5 days, to prevent immune system from attacking

27

Standard of acute care in GBS (medically)

Plasmaphoresis and Intravenous immunoglobulin

28

Is neuropathic pain a potential issue for GBS pts?

yes! inflammatory process is occurring along the nerves

29

What is the distribution of pain?

Stocking glove distribution

30

Which types of muscles are particularly painful to stretch?

2 joint muscles (HS)

31

Specific areas of interest in integumentary system

EARS
heels
no ER of feet - fibular head

32

Autonomic dysfunction: symptoms to be aware of

decreased temperature regulation
impaired HR response (may be racing)
Orthostasis (unstable BP)

33

3 settings where pt might enter respiratory distress when exercising on the ventilator

1. routine exercises when weaning off ventilator
2. new exercise or activity performed at usual setting
3. progressing difficulty at same setting

34

Rehab goals: Compensation or restoration?

Both! ideally you want restoration, but if you want them to be functional they may need to compensate for muscles that haven't returned yet

35

Strengthening issues

1. Core is critical for all movements (limbs overworked)
2. Train fast-twitch fibers for force production (speed and force training)
3. No Eccentric work until anti-gravity strength! (avoid DOMS- results in dec. strength)
4. submaximal exercise with small reps + sets

36

Definition of submaximal exercise:
in LE and UE
appropriate reps and sets

LE: 30% or 40% max
UE: 10%, 15%, or 20% max
Reps: 4, 6, 8 with small sets

37

When to begin training in anti-gravity positions:

Muscle must be at least a 3/5 - needs to handle resistance of the limb

38

3 residual syndroms in patients without full recovery

1. severe parathesias, dyesthesias
2. sensory ataxia
3. foot drop + intrinsic hand weakness

39

Long term studies: what does recovery look like?

Full recovery with subclinical signs:
- speed/force issues
- "full", but activities not as enjoyable/performed at same level
- recovery highly related to fast twitch fibers

40

Long term studies: what do pts with good recovery still report?

1. Fatigue: can do everything, but get tired
2. Pain: residual

41

Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP)

Recurrent form of GBS, acute and insidious
Looks like GBS at first, progression is much slower
steady decline in functioning: small recovery followed by another episode

42

CIDP PT intervention

requires more compensatory strategies
HEP of daily strengthening+ cycle/walking for 6 weeks --> shown to increase strength and diminish disabilty