GBS Flashcards by Carly Mueller

Axonopathy: definition, chronic examples

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

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Myelinopathy: definition, examples

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

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Neuronopathy: definition, examples

damage to the cell bodies (no regrowth possible)

examples = ALS, SMA, Polio

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Neuropathic Weakness

weaker distal > proximal

example = polyneuropathy (GBS)

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Myopathic Weakness

weaker proximal > distal

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Cause of Mortality in GBS

inability to breath; complications from respiratory or autonomic issues

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Etiology/Pathology of GBS

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

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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

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3 clinical subtypes of GBS

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

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AIDP

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

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AMAN

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

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AMSAN

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

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Miller-Fisher Syndrome

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

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Only way to know type of GBS

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

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Recovery period for GBS

up to 2 years

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Poorer outcomes associated with:

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

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3 temporal phases of GBS

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

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

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)

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

5 phases of Pt’s Perspective of Recovery

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

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

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

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:

increase firing rate: temporal summation (rate coding)
- requires myelin to send down AP
increase # of muscles firing: engaging more alpha motor neurons (recruitment)

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

Intravenous Immunoglobulin: how long is it administered, why?

3-5 days, to prevent immune system from attacking

Standard of acute care in GBS (medically)

Plasmaphoresis and Intravenous immunoglobulin

Is neuropathic pain a potential issue for GBS pts?

yes! inflammatory process is occurring along the nerves

What is the distribution of pain?

Stocking glove distribution

Which types of muscles are particularly painful to stretch?

2 joint muscles (HS)

Specific areas of interest in integumentary system

EARS heels no ER of feet - fibular head

Autonomic dysfunction: symptoms to be aware of

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

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

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

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

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

When to begin training in anti-gravity positions:

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

3 residual syndroms in patients without full recovery

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

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

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

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

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

CIDP PT intervention

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