Guillian Barre syndrome clinical scores

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"Guillain-Barre syndrome"[MeSH Terms] AND "clinical score" AND prognosis

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"Guillain-Barre syndrome"[MeSH Terms] AND prognosis AND score

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PMID: 37814552

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Guillain Barre syndrome clinical scoring systems GBS disability scale Brighton criteria EGOS mEGOS EGRIS 2023 2024

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https://gbs.tghn.org/clinical-guideline/prognosis-tools

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Guillain-Barré Syndrome: Clinical Scoring Systems

GBS uses several distinct scoring systems, grouped by purpose: diagnostic/classification, disability/functional outcome, and prognostic (respiratory risk and functional outcome).

1. Diagnostic Criteria

Asbury-Cornblath Criteria (Clinical Diagnosis)

The classic diagnostic framework used in clinical practice:
Features required:
  • Progressive weakness of both legs and arms
  • Areflexia or hyporeflexia
Supportive clinical features:
  • Progression over days to 4 weeks
  • Relative symmetry of symptoms and signs
  • Mild sensory symptoms or signs
  • Bifacial palsies
  • Autonomic dysfunction
  • Absence of fever at onset
  • Recovery beginning 2-4 weeks after progression ceases
Supportive laboratory features:
  • Elevated CSF protein with <10 cells/µL (albuminocytological dissociation)
  • Electrodiagnostic features of nerve conduction slowing or block
(Bradley and Daroff's Neurology in Clinical Practice)

Brighton Criteria (Surveillance/Epidemiological Certainty Levels)

Used for post-vaccination surveillance and epidemiological studies; assigns diagnostic certainty levels 1-4:
LevelCriteria Met
1 (Highest certainty)Bilateral flaccid limb weakness + decreased/absent DTRs in weak limbs + monophasic course + albuminocytological dissociation + EMG consistent with GBS
2Bilateral flaccid limb weakness + decreased/absent DTRs in weak limbs + monophasic course + either CSF or EMG consistent
3Bilateral flaccid limb weakness + decreased/absent DTRs in weak limbs + monophasic course (without CSF or EMG)
4Reported as GBS but not meeting levels 1-3
Used alongside NINDS criteria in clinical studies to stratify diagnostic reliability (e.g., a 2024 Egyptian cohort study classified patients as level 2 or 3 without CSF data).

2. Disability / Functional Status Scores

Hughes GBS Disability Scale (GBS-DS)

Originally described by Hughes et al. (1978), this is the most widely used functional outcome score in GBS trials. The version adapted by van Koningsveld et al. (2007) for Lancet Neurology is the standard:
ScoreDescription
0Healthy state
1Minor symptoms, capable of running
2Able to walk 10m or more without assistance, but unable to run
3Able to walk 10m across open space with help
4Bedridden or chairbound
5Requiring assisted ventilation for at least part of the day
6Dead
This score is the primary endpoint in most GBS clinical trials (IVIg vs plasma exchange studies). The 2023 EAN/PNS guideline uses inability to walk unaided (score ≥3) as the threshold for treatment.

MRC Sum Score

Ranges from 0 (no muscle power) to 60 (full muscle power). It sums the strength of 6 muscle groups bilaterally (shoulder abduction, elbow flexion, wrist extension, hip flexion, knee extension, ankle dorsiflexion), each scored 0-5. Used as a component in EGRIS and mEGOS. A score of ≤20 carries the highest risk of requiring intubation.

Overall Neuropathy Limitation Scale (ONLS)

A broader measure of upper and lower limb activity limitation, used alongside HDS in some recent studies to capture functional recovery.

3. Prognostic Scores: Respiratory Failure

EGRIS - Erasmus GBS Respiratory Insufficiency Score

Developed by Walgaard et al. (2010, Ann Neurol). Predicts risk of requiring mechanical ventilation within the first 7 days of hospitalization.
MeasureCategoryScore
Days between onset of weakness and hospitalization>7 days0
4-7 days1
≤3 days2
Facial and/or bulbar weakness at hospitalizationAbsent0
Present1
MRC sum score at hospitalization60-510
50-411
40-312
30-213
≤204
Interpretation:
EGRISRisk CategoryRisk of Intubation
0-2Low~4%
3-4Intermediate~24%
5-7High~65%
  • Patients with EGRIS 5-7 should be admitted to ICU for close observation.
  • Internationally validated in 2022 (Doets et al., Ann Neurol).
(Fishman's Pulmonary Diseases and Disorders)

mEGRIS - Modified Erasmus GBS Respiratory Insufficiency Score

Published by Luijten et al. (2023, J Neurol Neurosurg Psychiatry). A simplified version of EGRIS designed to be more clinically practical, with simplified MRC scoring categories. The 2023 EAN/PNS guideline (van Doorn et al., Eur J Neurol) specifically recommends the mEGRIS over the original EGRIS for assessing risk of requiring artificial ventilation.
Rule of 20-30-40 (from Bradley and Daroff): Intubation risk is high when any of the following are present:
  • Vital capacity <20 mL/kg or declining 30% from baseline
  • Maximal inspiratory pressure < -30 cm H₂O
  • Maximal expiratory pressure <40 cm H₂O
Criteria for intubation (Fishman's):
Major (1 criterion = intubate)Minor (2 criteria = intubate)
PaCO₂ ≥48 mmHgIneffective cough
PaO₂ ≤56 mmHgImpaired swallowing
VC <15 cc/kgAtelectasis

4. Prognostic Scores: Functional Outcome

EGOS - Erasmus GBS Outcome Score

Validated prognostic tool used at 14 days of admission (van Koningsveld et al., Lancet Neurol 2007). Predicts inability to walk independently at 6 months. Three components:
VariableOptionsScore
Age at onset<40 years0
≥40 years1
Preceding diarrheal illness (past 4 weeks)No0
Yes1
GBS Disability Score at 2 weeks0-20
33
46
59
Higher EGOS = greater risk of inability to walk at 6 months.
(Rosen's Emergency Medicine)

mEGOS - Modified Erasmus GBS Outcome Score

Developed by Walgaard et al. (2011, Neurology). Can be applied earlier - at 1 week of admission (unlike EGOS which requires 2 weeks). Replaces the GBS Disability Score with the MRC sum score, allowing earlier prognostication:
  • Uses: age, preceding diarrheal illness, and MRC sum score (instead of GBS disability score)
  • Validated internationally including in South Asian populations (Papri et al., 2022; Doets et al., Neurology 2022)
  • The 2023 EAN/PNS guideline specifically recommends mEGOS for assessing functional outcome prognosis

Summary Table

ScorePurposeTimingKey Variables
Hughes GBS-DSDisability/functional statusAny timeAmbulation, ventilation need
MRC Sum ScoreMuscle strengthAny time6 muscle groups, bilateral, 0-60
Brighton CriteriaDiagnostic certaintyAt diagnosisCSF, EMG, clinical features
EGRISRespiratory failure riskAt admissionDays to admission, bulbar weakness, MRC
mEGRISRespiratory failure risk (simplified)At admissionSimplified EGRIS - preferred by 2023 EAN/PNS guideline
EGOSFunctional outcome at 6 monthsDay 14 of admissionAge, diarrhea, GBS-DS at 2 weeks
mEGOSFunctional outcome at 6 months (earlier)Day 7 of admissionAge, diarrhea, MRC sum score

2023 EAN/PNS Guideline Recommendations

The current EAN/Peripheral Nerve Society guideline (van Doorn et al., 2023, PMID 37814552) recommends:
  • mEGOS to assess functional outcome prognosis
  • mEGRIS to assess risk of requiring mechanical ventilation
  • IVIg 0.4 g/kg for 5 days or plasma exchange for patients unable to walk unaided within 4 weeks of onset
  • Gabapentinoids, TCAs, or carbamazepine for pain management

How to calculate MRC sum score in guillian Barre syndrome

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MRC Medical Research Council muscle strength grading scale table

This composite clinical photograph demonstrates manual muscle testing (MMT) of the foot and ankle, typically performed to assess neurological or musculoskeletal impairment, such as in post-polio sequelae. Image (a) depicts the assessment of ankle dorsiflexor power. The clinician's hand applies downward resistance against the dorsal aspect of the forefoot and toes while the patient attempts to lift the foot, testing the tibialis anterior and extensor muscles. The foot shows a slight cavus arch. Image (b) demonstrates the testing of the foot invertors (primarily the tibialis posterior). The examiner applies lateral and eversional force against the medial border of the forefoot while the patient attempts to pull the foot inward (inversion). These clinical maneuvers are essential for grading muscle strength according to the Medical Research Council (MRC) scale, particularly when planning surgical interventions like tendon transfers or stabilizing arthrodesis to correct deformities such as equinovarus or calcaneocavovalgus.

This composite clinical photograph demonstrates manual muscle testing (MMT) of the foot and ankle, typically performed to assess neurological or musculoskeletal impairment, such as in post-polio sequelae. Image (a) depicts the assessment of ankle dorsiflexor power. The clinician's hand applies downward resistance against the dorsal aspect of the forefoot and toes while the patient attempts to lift the foot, testing the tibialis anterior and extensor muscles. The foot shows a slight cavus arch. Image (b) demonstrates the testing of the foot invertors (primarily the tibialis posterior). The examiner applies lateral and eversional force against the medial border of the forefoot while the patient attempts to pull the foot inward (inversion). These clinical maneuvers are essential for grading muscle strength according to the Medical Research Council (MRC) scale, particularly when planning surgical interventions like tendon transfers or stabilizing arthrodesis to correct deformities such as equinovarus or calcaneocavovalgus.

A multi-panel medical graphic illustrating clinical course and diagnostic imaging of a spinal cord infarction. Panel A is a line graph and bar chart summarizing clinical events over 20 days post-symptom onset, tracking platelet count, D-dimer, Fibrinogen Degradation Products (FDP), and Medical Research Council (MRC) muscle strength scores. Panel B shows a coronal maximum intensity projection (MIP) reconstruction of a CT angiography (CTA) image of the thoracolumbar region. An arrow points to tandem narrowing of the artery of Adamkiewicz (Aka) at the T10-11 level. An inset curved multiplanar reformatted image displays the artery's continuity from the left T9-10 lumbar artery and aorta around the vertebral arch pedicle. Panel C presents a T2-weighted axial MRI of the spinal cord at the T9-11 level, demonstrating focal hyperintensity (edema) in the central gray matter. Red arrows highlight bilateral high signal intensity in the ventral horns, forming the characteristic 'owl's eyes sign,' which is highly suggestive of anterior spinal artery syndrome or spinal cord ischemia.

A multi-panel medical graphic illustrating clinical course and diagnostic imaging of a spinal cord infarction. Panel A is a line graph and bar chart summarizing clinical events over 20 days post-symptom onset, tracking platelet count, D-dimer, Fibrinogen Degradation Products (FDP), and Medical Research Council (MRC) muscle strength scores. Panel B shows a coronal maximum intensity projection (MIP) reconstruction of a CT angiography (CTA) image of the thoracolumbar region. An arrow points to tandem narrowing of the artery of Adamkiewicz (Aka) at the T10-11 level. An inset curved multiplanar reformatted image displays the artery's continuity from the left T9-10 lumbar artery and aorta around the vertebral arch pedicle. Panel C presents a T2-weighted axial MRI of the spinal cord at the T9-11 level, demonstrating focal hyperintensity (edema) in the central gray matter. Red arrows highlight bilateral high signal intensity in the ventral horns, forming the characteristic 'owl's eyes sign,' which is highly suggestive of anterior spinal artery syndrome or spinal cord ischemia.

This clinical photograph demonstrates a standardized procedure for measuring muscle strength using hand-held dynamometry (HHD), specifically focusing on hip extension. The patient is positioned in a side-lying posture on a medical examination table. A large, grey rectangular support block is placed between the patient’s legs to maintain neutral alignment and provide stability for the upper limb during the assessment. The examiner, wearing a white uniform, is seen applying a hand-held dynamometer to the posterior aspect of the patient's lower leg, just proximal to the ankle joint. A notable technical feature shown is the use of a wide stabilization belt worn by the examiner around their waist and anchored to the table. This belt serves as a mechanical brace to counteract the force exerted by a strong patient, ensuring that the measurement reflects the patient's actual peak force rather than being limited by the physical strength of the tester. This procedural setup is indicative of clinical research or advanced physiotherapy protocols aimed at improving the reliability and validity of objective muscle strength testing.

This clinical photograph demonstrates a standardized procedure for measuring muscle strength using hand-held dynamometry (HHD), specifically focusing on hip extension. The patient is positioned in a side-lying posture on a medical examination table. A large, grey rectangular support block is placed between the patient’s legs to maintain neutral alignment and provide stability for the upper limb during the assessment. The examiner, wearing a white uniform, is seen applying a hand-held dynamometer to the posterior aspect of the patient's lower leg, just proximal to the ankle joint. A notable technical feature shown is the use of a wide stabilization belt worn by the examiner around their waist and anchored to the table. This belt serves as a mechanical brace to counteract the force exerted by a strong patient, ensuring that the measurement reflects the patient's actual peak force rather than being limited by the physical strength of the tester. This procedural setup is indicative of clinical research or advanced physiotherapy protocols aimed at improving the reliability and validity of objective muscle strength testing.

A clinical photograph depicting a participant undergoing static quadriceps muscle strength testing in a clinical or research setting. The individual is seated upright on a testing table with the back firmly supported against a vertical backrest. Safety and stabilization straps are visible across the chest and thighs to prevent compensatory movements. The participant's knees are positioned at the edge of the table, flexed at approximately 60 degrees. A cable tensiometer is attached to the participant's distal lower leg using a padded ankle cuff. This device measures isometric force (kg or Newtons) as the participant attempts to extend the knee against the resistance of the fixed cable. A clinician in a white lab coat is shown monitoring the tensiometer scale. This setup is typical for physical therapy assessments and rehabilitation research to evaluate quadriceps peak force and muscle function, particularly in the management of knee osteoarthritis or post-surgical recovery.

A clinical photograph depicting a participant undergoing static quadriceps muscle strength testing in a clinical or research setting. The individual is seated upright on a testing table with the back firmly supported against a vertical backrest. Safety and stabilization straps are visible across the chest and thighs to prevent compensatory movements. The participant's knees are positioned at the edge of the table, flexed at approximately 60 degrees. A cable tensiometer is attached to the participant's distal lower leg using a padded ankle cuff. This device measures isometric force (kg or Newtons) as the participant attempts to extend the knee against the resistance of the fixed cable. A clinician in a white lab coat is shown monitoring the tensiometer scale. This setup is typical for physical therapy assessments and rehabilitation research to evaluate quadriceps peak force and muscle function, particularly in the management of knee osteoarthritis or post-surgical recovery.

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MRC Sum Score in Guillain-Barré Syndrome

The MRC Grading Scale (Per Individual Muscle)

Each muscle group is graded from 0 to 5 using the standard Medical Research Council scale:
GradeDescription
0No contraction at all
1Flicker or trace of contraction, but no movement
2Active movement possible, but only with gravity eliminated (limb can move sideways on flat surface)
3Active movement against gravity, but not against added resistance
4Active movement against gravity AND against some resistance (weaker than normal)
5Normal strength - movement against gravity and full resistance
Some clinicians further subdivide Grade 4 into 4-, 4, and 4+ to detect subtle changes, but for the GBS MRC sum score, whole numbers 0-5 are used.

The 6 Muscle Groups Tested (Each Side)

In GBS, the MRC sum score tests 6 specific muscle groups bilaterally - the same set on the left and right side:
#Muscle GroupMovement TestedNerve Root
1Shoulder abductors (deltoid)Raise arm out to the side to 90°C5
2Elbow flexors (biceps)Bend the elbow against resistanceC5-C6
3Wrist extensors (extensor carpi radialis)Cock the wrist back against resistanceC6-C7
4Hip flexors (iliopsoas)Lift the thigh off the bed against resistanceL2-L3
5Knee extensors (quadriceps)Straighten the knee against resistanceL3-L4
6Ankle dorsiflexors (tibialis anterior)Pull the foot up against resistanceL4-L5
These 6 groups are chosen because they span the upper and lower limb, proximal and distal, and reflect the ascending/descending weakness pattern of GBS.

How to Calculate the Total Score

Score each of the 6 muscle groups on the LEFT side (0-5) = subtotal (max 30) Score each of the 6 muscle groups on the RIGHT side (0-5) = subtotal (max 30) Add both sides together = MRC Sum Score (range: 0 to 60)
MRC Sum Score = Σ (6 muscles × left side) + Σ (6 muscles × right side)
              = max 30 + max 30 = 60 total
ScoreMeaning
60Normal, full strength bilaterally
51-59Mild weakness
41-50Moderate weakness
31-40Significant weakness
21-30Severe weakness
≤20Very severe - near complete paralysis

How MRC Sum Score Is Used in GBS Clinical Scoring

In EGRIS (Respiratory Failure Prediction)

The MRC sum score at admission is one of three variables:
MRC Sum ScoreEGRIS Points
60-510
50-411
40-312
30-213
≤204
A score of ≤20 contributes the maximum 4 points, indicating the greatest risk of needing mechanical ventilation.

In mEGOS (Functional Outcome Prediction at 1 Week)

The mEGOS uses MRC sum score at Day 7 of admission (instead of the Hughes Disability Scale used in EGOS) to predict walking ability at 6 months - higher MRC score (stronger) = better prognosis.

Practical Tips for Bedside Testing

  1. Position matters - test in standard positions: sitting for upper limbs, supine for lower limbs.
  2. Gravity elimination - for grade 2 testing, position the limb in the horizontal plane (e.g., hip flexion tested with the patient on their side).
  3. Serial testing - repeat every 4-6 hours during the progressive phase to track deterioration.
  4. Symmetric assessment - always score both sides separately; GBS is usually symmetric but asymmetric forms exist.
  5. Pain limitation - GBS commonly causes severe radicular/limb pain (~70% of patients); this can confound testing - document if effort is pain-limited.
  6. Bulbar muscles are NOT included in the MRC sum score - they are assessed separately (facial/bulbar weakness is the second EGRIS variable).
(Fishman's Pulmonary Diseases and Disorders; Bradley and Daroff's Neurology in Clinical Practice)
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