Important and useful topic

1. • 44-year-old female came with history of paraesthesia of both lower limbs
since 10 days, noticed buckling of both knee since nine days, started having
difficulty to get up from squatting since one week, unable to lift upper limb
since five days and difficulty in swallowing since five days.
• How will you proceed??

2. GUILLAIN-BARRE SYNDROME
M.L.N SANDEEP
2nd YEAR POST GRADUATE
UNIT 2

3. 1) HEREDITARY : Charcot Marie Tooth disorder,fabrys
2) INFLAMMATORY : GUILLAIN BARRE SYNDROME,CIDP
3) METABOLIC AND NUTRITIONAL : T2dm,porphyria’s,vitamin deficiencies
4) TOXINS : drugs,alcohol,heavy metals
5) VASCULAR : Ischemic neuropathies
6) COMPRESSIVE

4. • GBS IS AN ACUTE,
• FREQUENTLY SEVERE,
• FULMINANT POLY RADICULO NEUROPATHY,
• AUTO IMMUNE IN NATURE.
• MALES > FEMALES
• ADULTS > CHILDREN
• LEADING CAUSE OF ACUTE PARALYTIC DISEASE IN WESTERN COUNTRIES

5. 70 % OF GBS OCCUR IN 1 - 3 WEEKS AFTER AN
ACUTE INFECTIOUS PROCESS
1) MOSTLY - GASTRO INTESTINAL INFECTIONS.
(CAMPYLOBACTER JEJUNI)
- RESPIRATORY INFECTIONS
LESS COMMON - CMV,EBV,HIV,HEPATITIS E
2) VACCINE ASSOCIATED - 1976 H1N1 VACCINE / OLD
RABIES VACCINE
3) POST TRAUMA
4) SURGERY

6. PROXIMAL SYMMETRICAL WEAKNESS - DIFFICULTY IN SQUATTING
- BUCKLING OF KNEES
- DIFFICULTY IN LIFTING ARMS ABOVE SHOULDERS
ASCENDING PARALYSIS (FLACCID PARALYSIS)
DESCENDING PARALYSIS SEEN IN TETANUS,BOTULISM,DIPHTHERIA
TINGLING DYSESTHESIA - PINS AND NEEDLE SENSATIONS IN FINGERS , TOES , ANKLES OR WRISTS
WEAKNESS EVOLVES OVER HOURS TO FEW DAYS
UPPER NERVES ARE MORE INVOLVED

7. 50% WILL HAVE FACIAL DIPARESIS - DROOPING OF MOUTH
INCOMPLETE EYE LID CLOSURE
LOSS OF FOREHEAD WRINKLING
LOWER CRANIAL NERVES ARE ALSO AFFECTED(9,10,11,12) CAUSING BULBAR WEAKNESS :
DIFFICULTY IN SWALLOWING
DIFFICULTY IN CHEWING
NASAL REGURGITATION
AIRWAY OBSTRUCTION
DYSPHONIA : UNABLE TO PRODUCE SOUND DUE TO LARYNGEAL WEAKNESS
ASPIRATION OF LIQUIDS

8. NECK,SHOULDER AND BACK PAIN ARE MORE COMMON
ABSENT DEEP TENDON REFLEX
PROPRIOCEPTION IS AFFECTED - DIFFICULTY IN MAINTAINING BALANCE
-WASH BASIN PHENOMENA
- UNCORDINATED MOVEMENTS
- NOT ABLE TO WALK IN STRAIGHT LINE
- FREQUENT FALLS WHILE WALKING OR SITTING

9. AUTONOMIC INVOLVEMENT IS MORE COMMON
• Loss of vasomotor control -Profuse sweating
• Wide fluctuations in Blood pressure
• Postural hypotension
• Cardiac Dysarrythimias - Bradycardia/ Tachycardia
BOWEL AND BLADDER INVOLVEMENTS ARE RARE

10. COMMON SUBTYPES :
• ACUTE INFLAMMATORY DEMYLENATING POLY RADICULO NEUROPATHY(AIDP) (m/c)
• ACUTE MOTOR AXONAL NEUROPATHY(AMAN)
• ACUTE MOTOR SENSORY AXONAL NEUROPATHY(AMSAN)
RARE VARIANTS :
• MILLER FISCHER SYNDROME
• PHARYNGEAL-CERVICAL-BRACHIAL SYNDROME
• FACIAL DIPLEGIA WITH PARASTHESIAS
• PARAPARETIC VARIANT

13. • TYPE 2 HYPERSENSITIVITY REACTION
• CELLULAR AND HUMORAL IMMUNITY INVOLVED
• MOLECULAR MIMCRY MECHANISM
• IMMUNE RESPONSE ACTIVATES COMPLEMENT SYSTEM,WHICH ACTIVATES MEMBRANE
ATTACKING COMPLEX.
• MAC STRIPS MYELIN SHEATH
• ION CHANNELS PRESENT IN AXON GET EXPOSED,LEADING TO ALTERED CURRENT FLOW

14. • ACUTE INFLAMMATORY DEMYELINATING POLY NEUROPATHY

15. • DEMYLENATING TYPE
• ADULTS > CHILDREN
• RAPID RECOVERY
• ANTI GM1 ANTIBODIES < 50%
• BASIS FOR FLACCID PARALYSIS AND SENSORY DISTURBANCE IS CONDUCTION BLOCK.
• AXONAL CONNECTIONS REMAIN INTACT
• SECONDARY AXONAL DEGENERATION IS ASSOCIATED WITH SEVERE GBS,DELAYS
RECOVERY

17. • AXONAL TYPE
• CHILDREN AND YOUNG ADULTS ARE AFFECTED
• PREVALENT IN CHINA AND MEXICO
• SEASONAL
• RAPID RECOVERY
• ANTI GD1a ANTIBODIES PRESENT
• AXONS ARE DISCONNECTED FROM TARGETS

18. • AXONAL TYPE
• UNCOMMON
• RESEMBLES ACUTE MOTOR AXONAL NEUROPATHY
• ALSO AFFECTS SENSORY NERVES
• SLOW AND INCOMPLETE RECOVERY

19. 90 % ASSOCIATED WITH GQ1b antibodies
1) OPHTHALMOPLEGIA
2) ATAXIA
3) AREFLEXIA
CAN BE AXONAL OR DEMYLENATING
WEAKNESS IS NOT A CLASSICAL FEATURE
SHOULD NOT HAVE LOSS OF CONSIOUNESS AND CORTOCAL SPINAL TRACT INVOLVEMENT
IF LOC/CST INVOLVEMENT PRESENT - SUGGESTS DX OF BICKERSTAFF BRAINSTEM ENCEPHALITIS

20. 1) CSF ANALYSIS :
ELEVATED PROTEINS WITHOUT PLEOCYTOSIS
ALBUMINO CYTOLOGICAL DISSOCIATION PRESENT
• WBC <10 : CLASSICAL
• WBC 10-50 : ACCEPTABLE
• WBC > 50 : EVALUATE FOR OTHER CONDITIONS LIKE HIV,LYMES DISEASE
CSF SHOULD BE SCREENED FOR PRESENCE OF ANTI GLYCOLPID AND ANTI
GANGLIOSIDE ANTIBODIES

21. INVESTIGATIONS
2) NERVE CONDUCTION STUDIES :
DECREASED CONDUCTION VELOCITY
INCREASED DURATION
TEMPORAL DISPERSION PRESENT
PROLONGED LATENCY
SURAL NERVE SPARING : CLASSIC OF GBS
SURAL SNAP + RADIAL SNAP / RADIAL AND ULNAR SNAP(SENSORY RATIO) : > 1 IN CASE
OF GBS

22. 3) EXCLUSION : ABSENCE OF ALTERNATIVE DIAGNOSIS.
• ALL INVESTIGATIONS CAN BE NORMAL IN FIRST WEEK
• CSF AND NERVE MAY BE NORMAL IN FIRST WEEK
• SO ,THEY SHOULD BE REPEATED AGAIN IN SECOND WEEK

26. LEVEL 1 EVIDENCE :
CLINICAL CRITERIA
+
EXCLUSION CRITERIA
+
BOTH INVESTIGATIONS POSITIVE

27. LEVEL 2 EVIDENCE :
CLINICAL CRITERIA
+
EXCLUSION CRITERIA
+
ONE OF THE INVESTIGATION
IS POSITIVE

28. LEVEL 3 EVIDENCE :
CLINICAL CRITERIA
+
EXCLUSION CRITERIA

29. IF PLATEAU IS REACHED + MINIMAL DEFICITS : CAN WAIT AND MONITOR
TREATMENT IS REQUIRED IF:
• FAST PROGRESSION (<7 DAYS)
• RESPIRATORY MUSCLE INVOLVEMENT
• NECK MUSCLES INVOLVEMENT
• BULBAR INVOLVEMENT

30. ICU CARE NEEDED IF :
• SEVERE LIMB WEAKNESS
• RAPID PROGRESSION (< 7 days)
• AUTONOMIC FLUCTUATION
• BULBAR INVOLVEMENT
• ABG SHOULD BE TAKEN 6th HOURLY
• SINGLE BREATH COUNT SHOULD BE ASSESED 6th HOURLY
• VITAL CAPACITY TESTING SHOULD BE DONE Q6th HOURLY

31. 20-30-40 RULE :
1) VITAL CAPACITY <20 ML/KG
2) MAXIMUM INSPIRATORY PRESSURE < 30 ml H20
3) MAXIMUM EXPIRATORY PRESSURE > 40 ml H2O
IF VITAL CAPACITY < 12 ML/KG - REQUIRES EMERGENCY INTUBATION

32. IV IMMUNOGLOBULINS : 2 GRAM /KG OVER 5 DAYS Or 0.4 mg/kg/day
x5D
• BENEFICIAL IF GIVEN <2 weeks of onset
PLASMA EXCHANGE:250ml/kg total volume to be filtered
• BENEFICIAL IF GIVEN > 2 weeks of onset
• DUAL THERAPY NOT ADVISED - may be useful,if patient is not responding to initial
treatment
• IMMUNOMODULATORS CAN BE CONSIDERED (new studies)
• STEROIDS ARE NOT BENEFICIAL IN TREATMENT OF GBS

33. 1) PREVENT INFECTIONS
2) RESPIRATORY AND VITAL MONITORING
3) DVT PROPHYLAXIS
4) PHYSIOTHERAPY
5) NUTRITION
6) CHEST PHYSIOTHERAPY
7) EARLY CONSIDERATION OF TRACHEOSTOMY,IF INTUBATION LONGER
THAN 2 WEEKS

35. • HEAD ACHE,CHILLS AND MYALGIA
• CHEST DISCOMFORT
• ASEPTIC MENINGITIS (48-72 hrs of first dose)
• VENOUS OR ARTERIAL THROMBO EMBOLIC EVENTS
• TRANSIENT RENAL FAILURE
• ANAPHYLAXIS IN IGA DEFICIENT INDUVIDUAL

37. • 1-5 % : MORTALITY
• AROUND 30 % RESPIRATORY FAILURE
• AROUND 70 % : IMPROVES IN ONE YEAR
• AROUND 80 % : RECOVERY IN 2 YEARS
• AROUND 20 % : RESIDUAL WEAKNESS PRESENT

38. • AGE > 60 YEARS
• SEVERE WEAKNESS
• AUTONOMIC FLUCTUATIONS
• RESPIRATORY FAILURE
• AMSAN VARIANT

39. AIDP CIDP
< 4 WEEKS
> 8 WEEKS
INFLAMMATORY INFLAMMATORY
ALBUMINO CYTOLOGICAL DISASSOCIATION ALBUMINO CYTOLOGICAL DISASSOCIATION
>50 % FACIAL NERVE INVOLVEMENT <5 % FACIAL NERVE INVOLVEMENT
STEROIDS ARE NOT BENEFICIAL STEROIDS ARE USED IN THE TREATMENT

43. • HARRISON’S PRINCIPLES OF INTERNAL MEDICINE
• BRADLEY AND DAROFF’S NEUROLOGY

44. Progress in Guillain–Barré
syndrome immunotherapy—
A narrative review of new
strategies in recent years
Jiajia Yao, Rumeng Zhou,
Yue Liu & Zuneng Lu
Article: 2215153 | Received
12 Mar 2023, Accepted 15
May 2023, Published online:
06 Jun 2023
• Cite this article
https://doi.org/10.1080/2
1645515.2023.2215153