Lecture 1: Guillian Barre and CIDP Flashcards
What type of disease is GBS?
most common form of automimmune inflammatory demyelinating polyradiculoneuropathies
What is most common cause of GBS?
75% preceded by acute infxn 2 weeks prior (URI, CMV, Zika)
25% unknown trigger
What is pathophysiology of GBS?
auto immune attack on Schwann Cells (myelin producing) of peripheral nerves
axon may degenerate as well in severe cases
What results from this autoimmune attack on the Schwann Cells?
leads to lower motor neuron syndrome accompanied by lack of conduction, reduced reflexes, hypotonia, weakness
What is length of demyelination phase?
progressive demylelination phase limited to 4 weeks
remyelination usually begins with in 2-3 weeks
When is GBS most prevelant?
3rd to 5th decade, males affected twice as often as females
What are ways to clinically diagnose GBS?
lumbar puncture (presence of proteins), EMG, NCV
clinical features less than 4 weeks, progressive, symmetrical weakness with areflexia and exclusion of other causes
What are common differential diagnoses?
CIDP, lyme dz, myesthenia gravis (NM junction impaired), neuropathy, cord compression/ caude equine, conversion disorder
In what order does strength return in GBS?
proximal to distal , 80% of pts regain ambulation function
What is most common long term function effect of GBS?
decreased ambulation due to pretibial muscle weakness
What percent of GBS pts with turn into CIDP?
3-6%
What are poor prognostic indicators for GBS?
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
What are clinical features of GBS?
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
What are motor sx of GBS?
bilateral weakness usually symmetrical and distal to proximal, could be mild to total paralysis
20-30% need vents
What are sensory disturbances of GBS?
typically occurs before weakness, distal to proximal in stocking glove pattern, returns in reverse order
hyperesthesia, paraesthesias most common
decreased vibratory sense and proprioception
Why do patients with GBS still experience pain?
C fibers are not myelinated so they will not be affected
What is common form of pain with GBS?
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
How many pts experience autonomic dysfunction?
65-70%
cardiac arrthymias, decreased CO, BP fluctuations, sweating, pupillary dysfunction
greater than 50% have OTN likely due to low muscle tone of LE
Why is it important to test DTR in pts with GBS?
70% of GBS pts will have impaired DTR
intact reflexes suggest other diagnosis
What are two popular forms of medical management for GBS?
- plasma exchange- more expensive, more risk factors
2. IV immunoglobulin- safer, easier to administer
What does plasma exchange do for GBS?
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
What does IV immunoglobulin do for GBS?
50% recovery time reduced if initiated early
neutralizes auto antibodies and decreases levels of cytokines
What are impairment/ body structures and functions to examine for GBS?
strength, cranial nerve integrity, sensation, pain, ROM, skin integ., reflexes, activity tolerance
also examine activity and participation restrictions
What are PT interventions during progressive stage for GBS?
prevention of sequelae, pulm hygiene, skin protection, pain management monitor vitals, may need abdominal corsets or stocking for BP
What are some modalities for pain management?
ROM, TENS, positioning, ice packs, ace wrapping
What are PT interventions during early recovery stage?
gentle stretching- avoid end range for jt protection
activity in gravity minimized position, AAROM, no resistance, few reptitions
avoid overuse damage
What is the cause of overuse damage in GBS?
due to repetitive recruitment of same motor units because other motor units are unavailable
What are sx of overuse damage?
delayed onset of muscle soreness (1-5 days), decreased max isometric force production, decrease in function and strength
What are aerobic training guidelines for GBS?
60% target HR, oe 13/20 on RPE
How long does it take for nerve to regenerate?
1 mm/day, educate pt on this and stress aggressive rehab wont speed this up but will impair it
What are milestones for graduated strength training?
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)
How long should strength program stay the same during rehab?
keep program for one week and advance if no muscle aches or soreness
How does CIDP differ from GBS?
slower progression, weakness, sensory loss, areflexia
atleast 2 months progressive onset of sx required for diagnosis
What is gold standard medical management for CIDP?
IVIG, Plasma exchange, prednisone
also: interferons, rituximab, methotrexate, chemotherapy agents
What are 3 types of clinical course variants?
- monophasic
- relapsing
- progressive
What is monophasic course variant?
one episode of deterioration followed by sustained improvement 12-45% of pts
What is relapsing course variant?
atleast 2 separate deteriorrations and atleast one improvement between episodes, 11-42%
What is progressive course variant?
gradual deterioration with no episode of improvement
12-62% of pts
worst scenario
What are predictors of better outcomes of CIDP?
symmetrical sx, distal nerve abnormalities, favorable response to initial steroid treatment
