Lecture 1: Guillian Barre and CIDP

Question 1

What type of disease is GBS?

Answer 1

most common form of automimmune inflammatory demyelinating polyradiculoneuropathies

Question 2

What is most common cause of GBS?

Answer 2

75% preceded by acute infxn 2 weeks prior (URI, CMV, Zika)

25% unknown trigger

Question 3

What is pathophysiology of GBS?

Answer 3

auto immune attack on Schwann Cells (myelin producing) of peripheral nerves

axon may degenerate as well in severe cases

Question 4

What results from this autoimmune attack on the Schwann Cells?

Answer 4

leads to lower motor neuron syndrome accompanied by lack of conduction, reduced reflexes, hypotonia, weakness

Question 5

What is length of demyelination phase?

Answer 5

progressive demylelination phase limited to 4 weeks

remyelination usually begins with in 2-3 weeks

Question 6

When is GBS most prevelant?

Answer 6

3rd to 5th decade, males affected twice as often as females

Question 7

What are ways to clinically diagnose GBS?

Answer 7

lumbar puncture (presence of proteins), EMG, NCV

clinical features less than 4 weeks, progressive, symmetrical weakness with areflexia and exclusion of other causes

Question 8

What are common differential diagnoses?

Answer 8

CIDP, lyme dz, myesthenia gravis (NM junction impaired), neuropathy, cord compression/ caude equine, conversion disorder

Question 9

In what order does strength return in GBS?

Answer 9

proximal to distal , 80% of pts regain ambulation function

Question 10

What is most common long term function effect of GBS?

Answer 10

decreased ambulation due to pretibial muscle weakness

Question 11

What percent of GBS pts with turn into CIDP?

Answer 11

3-6%

Question 12

What are poor prognostic indicators for GBS?

Answer 12

need for vent support, cranial nerve involvement with difficulty swallowing, axonal damage, advanced age, preceding GI or CMV infection, rapid progression to quadriplegia within 1 week of onset

Question 13

What are clinical features of GBS?

Answer 13

rapid progression of symmetrical weakness, diminshed or absent DTR (70%), stocking glove pattern sensory distribution, autonomic dysregulation, recovery typically begins 2-4 weeks after plateau

Question 14

What are motor sx of GBS?

Answer 14

bilateral weakness usually symmetrical and distal to proximal, could be mild to total paralysis

20-30% need vents

Question 15

What are sensory disturbances of GBS?

Answer 15

typically occurs before weakness, distal to proximal in stocking glove pattern, returns in reverse order

hyperesthesia, paraesthesias most common

decreased vibratory sense and proprioception

Question 16

Why do patients with GBS still experience pain?

Answer 16

C fibers are not myelinated so they will not be affected

Question 17

What is common form of pain with GBS?

Answer 17

associated with pressure areas, stretching axons

worse at night, radicular pain usually in large muscles groups, hammys quads glutes

meds are shown to be non effective however

Question 18

How many pts experience autonomic dysfunction?

Answer 18

65-70%

cardiac arrthymias, decreased CO, BP fluctuations, sweating, pupillary dysfunction

greater than 50% have OTN likely due to low muscle tone of LE

Question 19

Why is it important to test DTR in pts with GBS?

Answer 19

70% of GBS pts will have impaired DTR

intact reflexes suggest other diagnosis

Question 20

What are two popular forms of medical management for GBS?

Answer 20

1. plasma exchange- more expensive, more risk factors

2. IV immunoglobulin- safer, easier to administer

Question 21

What does plasma exchange do for GBS?

Answer 21

if initiated with 1 week of diagnosis can shorten recovery time by 50% for indpt respiratory function and ambulation

removes cytokines and immunoglobulin and replaces with more neutral protein albumin

Question 22

What does IV immunoglobulin do for GBS?

Answer 22

50% recovery time reduced if initiated early

neutralizes auto antibodies and decreases levels of cytokines

Question 23

What are impairment/ body structures and functions to examine for GBS?

Answer 23

strength, cranial nerve integrity, sensation, pain, ROM, skin integ., reflexes, activity tolerance

also examine activity and participation restrictions

Question 24

What are PT interventions during progressive stage for GBS?

Answer 24

prevention of sequelae, pulm hygiene, skin protection, pain management monitor vitals, may need abdominal corsets or stocking for BP

Question 25

What are some modalities for pain management?

Answer 25

ROM, TENS, positioning, ice packs, ace wrapping

Question 26

What are PT interventions during early recovery stage?

Answer 26

gentle stretching- avoid end range for jt protection

activity in gravity minimized position, AAROM, no resistance, few reptitions

avoid overuse damage

Question 27

What is the cause of overuse damage in GBS?

Answer 27

due to repetitive recruitment of same motor units because other motor units are unavailable

Question 28

What are sx of overuse damage?

Answer 28

delayed onset of muscle soreness (1-5 days), decreased max isometric force production, decrease in function and strength

Question 29

What are aerobic training guidelines for GBS?

Answer 29

60% target HR, oe 13/20 on RPE

Question 30

How long does it take for nerve to regenerate?

Answer 30

1 mm/day, educate pt on this and stress aggressive rehab wont speed this up but will impair it

Question 31

What are milestones for graduated strength training?

Answer 31

must have greater than 2/5 strength before anti gravity work

must have greater than 3/5 for eccentric work (overuse damage likely to occur with eccentric work)

Question 32

How long should strength program stay the same during rehab?

Answer 32

keep program for one week and advance if no muscle aches or soreness

Question 33

How does CIDP differ from GBS?

Answer 33

slower progression, weakness, sensory loss, areflexia

atleast 2 months progressive onset of sx required for diagnosis

Question 34

What is gold standard medical management for CIDP?

Answer 34

IVIG, Plasma exchange, prednisone

also: interferons, rituximab, methotrexate, chemotherapy agents

Question 35

What are 3 types of clinical course variants?

Answer 35

1. monophasic
2. relapsing
3. progressive

Question 36

What is monophasic course variant?

Answer 36

one episode of deterioration followed by sustained improvement 12-45% of pts

Question 37

What is relapsing course variant?

Answer 37

atleast 2 separate deteriorrations and atleast one improvement between episodes, 11-42%

Question 38

What is progressive course variant?

Answer 38

gradual deterioration with no episode of improvement
12-62% of pts

worst scenario

Question 39

What are predictors of better outcomes of CIDP?

Answer 39

symmetrical sx, distal nerve abnormalities, favorable response to initial steroid treatment