GUILLAIN-BARRE SYNDROME

Acute Inflammatory Demyelinating Polyradi

culoneuropathy (AIDP)
◼ GBS is a heterogenous grouping of immun
e-mediated processes generally characteri
zed by motor, sensory & autonomic dysfun
ction.
◼ Characterized by progressive symmetric a
scending muscle weakness, paralysis and
hyporeflexia with or without sensory or aut
onomic symptoms.
◼ It affects the nerve roots and peripheral ne
rves leading to motor neuropathy & flaccid
paralysis.
 Pathophysiology
◼ Both humeral & cell mediated autoimmune response to a recent i
nfection.
◼ rarely related to toxic & metabolic causes like diabetes, alcohol ab
use, chronic exposure to heavy metals or industrial toxins.
◼ Autoantibodies against myelin constituents & gangliosides and gl
ycolipids of axonal & myelin membranes
↓
Lymphocytic infiltration & macrophage mediated demyelination of pe
ripheral nerves & spinal roots (cranial nerves)
↓
Recovery occurs with remyelination
Primarily myelin-sparing axonal damage resulting from direct cellu
lar attack on the axon itself.
◼ Incidence - 1-3 per 1,00,000 persons
◼ Age group
- all ages
- common in young adulthood (15-35 yrs) &
elderly persons (50-75 yrs)
- rare in infants
◼ Progression
- maximum paralysis in 1-2 days
- 50% reach greatest severity within 1 week
- 70% by 2 weeks
- in some cases it continues for 1-2 months
◼ Recovery
- gradual recovery of muscle strength occurs 2-4 we
eks after progression has stopped or the condition has pla
teaued.
◼ Morbidity/Mortality
- most patients(80%) achieve full & functional r
ecovery within 6-12 months.
- recovery is maximal by 18 months past onset.
- 7-15% of patients have permanent neurologic
al sequelae. Muscle weakness (pretibial, intrinsic h
and & foot muscles, quadriceps & gluteals), sensor
y ataxia & dysesthesia.
- mortality rate < 5% (ARDS, sepsis, pneumoni
a, pulmonary emboli & cardiac arrest).
- despite intensive care 3-8% die.
 Prognosis
◼ Poor prognosis is associated with
- rapid progression of symptoms to quadriplegia,
- advanced age,
- prolonged ventilation (>1month),
- failure to show improvement within 3 weeks of
plateau &
- severe reduction of action potentials on neuro
muscular testing.
◼ Patients with primary axonal degeneration show
a prolonged recovery period with persistent disab
ility.
 Variants
1. Acute motor axonal neuropathy (AMAN)
- purely motor subtype
- more prevalent in pediatric age group
- rapid progressive weakness with respiratory failure
- good recovery
2. Acute motor-sensory axonal neuropathy (AMSAN)
- affects both sensory & motor nerves and roots
- more prevalent in adults
- motor & sensory dysfunction with marked muscle w
asting
- poor recovery
3. Miller-Fisher syndrome (MFS)
- rare variant
- classic triad of ataxia, areflexia & ophthalmoplegia
- ataxia → sensory loss
- also have mild limb weakness, ptosis, facial palsy or bul
bar palsy
- ↓ed or absent SNAP or absent H-reflex
- recovery occurs in 1-3 months
4. Acute pan autonomic neuropathy
- rarest of all variants
- involves both sympathetic & parasympathetic systems
- cardiovascular involvement is common → mortality due
to dysrhythmias
- also have sensory symptoms
- recovery is gradual & often complete
 Clinical Presentation
1. Motor Dysfunction:
a. progressive signs & symptoms of motor weakness t
hat develop rapidly
- relative symmetry of motor involvement
- progression of weakness from distal to proximal (
distal weakness – quadriplegia with respiratory muscl
e weakness)
- cranial nerve involvement (III-VII, IX-XII) with dys
phagia, dysarthria, ophthalmoplegia & pupillary distur
bances
b. areflexia of at least distal tendon responses
Clinical Presentation cont…

2. Sensory Dysfunction:
a. distal hyperesthesia, paresthesia (tingling, burnin
g) & numbness are common but not progressive
or persistent. (stocking & glove pattern)
b. Decreased vibration & proprioception
c. Pain usually symmetrical & in large-bulk muscles
(gluteals, quadriceps & hamstrings). Less often in
leg and upper limb muscles.
 Clinical Presentation cont…
3. Autonomic Dysfunction:
a. Cardiovascular signs
- low cardiac output,
- cardiac dysrhythmias,
- marked fluctuation in blood pressure,
- peripheral pooling of blood &
- poor venous return.
b. Urinary retention due to urinary sphincter disturbance
s
c. Constipation – bowel paresis & gastric dysmotility
d. Hyper salivation & anhydrosis
◼ Differential Diagnosis:
- cauda eqina syndrome
- spinal cord infections/injuries
- hyperkalemia/hypokalemia/hypophosphatemia
- myasthenia gravis
- polymyositis
- basilar artery occlusion
◼ Diagnosis:
- clinical features
- LP & spinal fluid analysis
- Biochemical screening & Imaging studies – rule o
ut other conditions
- EMG – amplitude of CMAP ↓ed
- NCV – delay in F waves, MNAP & SNAP is ↓ed
◼ Medical management:
- Cardio respiratory support in ICU if vital capacity falls b
elow 20ml/kg or O2 saturation falls below 75%.
- Intubated if VC < 12ml/kg
- Plasmapheresis
- Intravenous immunoglobulin (IVIG)
◼ Complications:
- persistent paralysis
- respiratory failure – mechanical ventilation
- postural hypotension
- thromboembolism/ pneumonia/ skin breakdown
- aspiration
- cardiac dysrhythmia
- urinary retention
- psychiatric problems