A short discussion on GBS and its variants.

1. GUILLAIN-BARRÉ
SYNDROME
Presented by:
Dr. A.S.M. Saleh
Dr. Harendra Singh
Intern Doctors
MARMCH, Dinajpur.

2. CONTENTS
1. INTRODUCTION
2. EPIDEMIOLOGY
3. ANTECEDENT EVENTS
4. PATHOGENESIS
5. CLINICAL FEATURES
6. CLINICAL COURSE
7. VARIANTS OF GBS
8. INVESTIGATIONS
9. DIFFERENTIAL DIAGNOSIS??
10. THE BRIGHTON CRITERIA
11. TREATMENT
12. PROGNOSIS

3. INTRODUCTION
• Guillain-Barre syndrome(GBS) is an acute, frequently
severe, and fulminant polyradiculoneuropathy that is
autoimmune in nature.
• In 1916, three French Military doctors Georges
Guillain, Jean-Alexandre Barre & Andre Strohl
described two soldiers with areflexic paralysis
followed by recovery, referred to as Guillain-Barre
Syndrome.

5. EPIDEMIOLOGY
• It occurs year round
• Males affected more than females & adults affected more
than children. ( M:F = 1.1-1.7 : 1)
• Incidence of 1-4 cases in 1,00,000 in North America.
• Most common acute Neuromuscular disorder seen in ICU
• It is the most common cause of AFP in children
• Childhood GBS: average age = 4-8 years

6. ANTECEDENT EVENTS:
• Approximately 70% cases of GBS occur 1-3 weeks after an acute
infectious process, usually respiratory or gastrointestinal.
• 20-30% cases preceded by infection or reinfection with
Campylobacter jejuni
• Similar proportion preceded by infection with human herpes virus,
often CMV or EBV.
• HIV, Hepatitis E virus, Zika virus & Mycoplasma pneumoniae have
also been identified as antecedent infections.
• Recent immunizations : influenza vaccine, older type rabies vaccine
etc.

7. PATHOGENESIS:
• Several lines of evidence support autoimmune basis for GBS.
• Both cellular and humoral immune mechanisms involved.
• Cellular component involves the activation of T-Cells
• All forms of GBS involve the formation of autoantibodies against non-
self antigens that exhibit molecular mimicry with gangliosides,
present abundantly in human nervous system.
• Autoantibodies commonly seen are
1. AIDP : anti GM-1 antibodies
2. AMAN : anti GD1a antibodies
3. MFS: anti GQ1b antibodies

9. CLINICAL FEATURES:

10. CLINICAL FEATURES
GBS manifests as rapidly evolving areflexic motor
paralysis with/without sensory disturbances.
1. Ascending paralysis:
*That may first be noticed as “ rubbery legs”
*Weakness evolves over few hours to days
*Legs involved more than arms
*Facial diparesis in about 50% cases
2. Tingling dysesthesias in extremities
3. Deep tendon reflexes(DTR) weaken/disappear within first
few days of onset.

11. 4. Involvement of lower cranial nerves causing
*Bulbar weakness,
*Difficulty in handling secretions
*Difficulty in maintaining airway
Nerves commonly involved:
Adults: 7th CN
Pediatrics: 9th & 10th CN
5.Pain is a common feature
*A deep aching pain in the weakened muscles
*Pain in the shoulder, back or over spine in early stages
*Pain is self limiting and often responds to analgesics.

12. 6. Autonomic involvement is common causing,
*Loss of vasomotor control
*Fluctuation of BP
*Postural hypotension
*Cardiac dysrhythmias
7. Cutaneous sensory deficits are relatively mild.
8. Respiratory weakness requiring ventilatory support
occurs in 30% cases.

13. CLINICAL COURSE
• Once clinical worsening stops and the patient reaches a
plateau, almost within 4 weeks of onset, further
progression is unlikely.
• First two weeks of the illness is the dangerous period.
• Recovery can take place as soon as re-myelination occurs,
which is usually over a period of several months.
• The rate of regeneration depends upon the extent of axonal
damage. The greater the damage the longer the recovery
takes.

15. VARIANTS

16. INVESTIGATIONS
1. CSF Study:
elevated CSF protein( 100-1000 mg/dl) without accompanying
pleocytosis (albumino-cytological dissociation)
The CSF findings maybe normal in the first week of illness
A transient increase in CSF white cell count( 10-100/µl) may
occur, however sustained rise suggests alternative diagnosis,
like
a. Viral myelitis
b. Unrecognized HIV
c. Leukemia
d. Lymphoma
e. Neuro-sarcoidosis

17. 2. Electrodiagnosis(Edx):
Edx features are mild/absent in early stages of GBS
Earliest features: prolonged F-wave latencies, prolonged distal
latencies & reduced compound muscle action potential(CMAP)
Later slowing of conduction velocity, conduction block &
temporal dispersion maybe seen
Occasionally sensory nerve action potentials(SNAP) maybe
normal in the feet when abnormal in the arms. ( sural sparing )

20. D/D??
1. Acute myelopathies
2. Diphtheria
3. Lyme polyradiculitis & other tick borne paralyses
4. Porphyria
5. Vasculitic Neuropathy
6. Poliomyelitis
7. West Nile fever
8. CMV polyradiculitis
9. Myasthenia Gravis
10.Botulism
11.Poisoning with: OPC, Thallium, Arsenic, Shellfish
12.Severe hypophosphatemia.

22. When to consider alternate diagnosis?
 Fever at onset
 Early, persistent bowel/bladder dysfunction.
 Marked asymmetry of weakness
 A definite sensory level, significant sensory signs
 Severe pulmonary dysfunction at onset
 CSF pleocytosis > 50
 Slowly progressive/non-monophasic illness, without respiratory
involvement.

23. DIAGNOSIS
GBS is a clinical diagnosis.
• The diagnosis of GBS is made by recognizing the
pattern of rapidly evolving paralysis with areflexia,
absence of fever or other systemic symptoms, and
characteristic antecedent events.
• In 2011, THE BRIGHTON COLLABORATION developed
a new set of case definitions for GBS which have
subsequently been validated.

25. GBS

28. MFS

31. TREATMENT:
• Treatment should be started as early as possible after
diagnosis to halt progression as each day counts.
• After 2 weeks of first motor symptoms, it is not known
whether immunotherapy is still effective.
• Treatment is no longer indicated once the patient
reaches plateau phase unless,
a. severe motor weakness
b. we can’t exclude the possibility that an immunologic attack is
still ongoing.

32. SUPPORTIVE TREATMENT
Important specially in the worsening phase
Monitoring in the critical care setting: it involves monitoring of
1. Vital capacity
2. Heart rhythm
3. Cardiovascular status
4. BP
5. Nutrition
6. DVT prophylaxis
7. Early consideration of tracheostomy(after 2 weeks)
8. Chest physiotherapy
Skin care
Daily range of motion exercises
Daily reassurance

33. SPECIFIC TREATMENT
It involves either of the following two:
A. Intravenous Immunoglobulin(IVIG):
 IVIG is the usually chosen therapy for GBS
 It is easy to administer & has good safety profile
 It has been considered superior to plasmapheresis in case of
AMAN & MFS
 Five daily infusions for a total dose of 2 g/kg BW
 Mechanism of action: the GBS autoantibodies are neutralized by
anti idiotypic-antibodies present in IVIG preparations.

34. B. Plasmapheresis:
 Dose : 40-50 ml/KG BW plasma exchange 4-5 times over the course
over a week
 PE decreases the need for ventilation by nearly half( from 27% to
14%)
 It has also shown increased likelihood of full recovery at 1 year
from 55% to 68%
 Functionally significant improvement by the end of 1st week or
maybe delayed for several weeks.

35. TREATMENT( cont.)
• Either of the above two therapies can be used with both
showing similar effectiveness
• A combination of two therapies is not significantly better
than either alone.
• Glucocorticoids have not been found to be effective in GBS.
• Some cases show relapse within a month after showing
improvement following treatment early in the course of
disease. These cases improve after brief re-treatment.

36. PROGNOSIS
85% show full functional recovery over a period that varies from
several months to a year. Although, areflexia and some other
symptoms like fatigue may persist longer
Mortality rate < 5%. Death occurs from secondary pulmonary
complications.
Remaining 10% suffer residual disability.
The outlook is worst in severe proximal motor & sensory axonal
damage
5-10% cases have one/more late relapses; such cases are
classified as CIDP.

37. BAD PROGNOSTIC FACTORS:
1. Advanced age
2. Fulminant/severe attack
3. Delay in onset of treatment.
4. Rapid deterioration to ventilation
5. Evidence of axonal loss on EMG

38. TAKE HOME MESSAGE
Besides the classical GBS, other variants are known.
GBS is fully a clinical diagnosis.
Treatment shouldn’t be delayed even if Edx is non-
confirmatory.
All patients should be treated with IVIG/PIEx even in mild
cases. Therapy should be initiated within 2 weeks .
No justification to use recurrent IVIG/PIEx unless recurrent
disease.
In the setting of developing countries proper supportive
care, monitoring & early intervention forms the core of the
treatment.

39. RESOURCES USED:
1. Davidson’s Principles and Practice of
Medicine, 23rd edition.
2. Harrison’s Principles of Internal Medicine,
20th edition.
3. https://emedicine.medscape.com/article/315
632-overview