What is the typical pattern of Guillain-Barre Syndrome (GBS)?

The Pattern of Guillain-Barré Syndrome

Guillain-Barré syndrome follows a characteristic triphasic pattern: acute progressive ascending weakness reaching maximum disability within 2 weeks, followed by a plateau phase, then gradual recovery over months to years. 1, 2

Classic Clinical Pattern

Temporal Course

• Progression phase: Weakness develops acutely or subacutely, with most patients reaching maximum disability within 2 weeks of symptom onset 1, 2
• Plateau phase: Stabilization occurs, typically after progression stops within 4 weeks (if progression continues beyond 4 weeks, consider alternative diagnoses like A-CIDP) 3, 4
• Recovery phase: Improvement begins and can continue for more than 3 years after onset, with 60-80% of patients able to walk independently by 6 months 1, 2
• The disease course is monophasic in the vast majority of cases, though treatment-related fluctuations and relapses occur in 2-5% of patients 3, 2

Neurological Pattern

• Bilateral ascending weakness is the hallmark, typically starting in the legs and progressing proximally to involve arms and cranial muscles 1, 2
• Areflexia or hyporeflexia develops early, beginning in the lower limbs and eventually affecting most patients at nadir 1, 5
• Distal paresthesias or sensory loss often precede or accompany the motor weakness 1, 2
• Weakness is symmetrical and bilateral, though it can occasionally be asymmetrical or have atypical distribution patterns 3

Associated Features

• Preceding infection: Approximately two-thirds of patients report infectious symptoms within the 6 weeks before GBS onset, most commonly Campylobacter jejuni, cytomegalovirus, hepatitis E virus, Mycoplasma pneumoniae, Epstein-Barr virus, or Zika virus 3, 2
• Pain: Muscular, radicular, or neuropathic pain affects approximately two-thirds of patients and is often an early symptom 1, 2
• Cranial nerve involvement: Bilateral facial palsy is common, and other cranial nerves may be affected 3, 1
• Dysautonomia: Blood pressure/heart rate instability, pupillary dysfunction, and bowel/bladder dysfunction occur frequently 1, 2
• Respiratory failure: Develops in approximately 20% of patients, requiring mechanical ventilation 2, 6

Clinical Variants

While the classic sensorimotor pattern represents 70% of cases in Europe and the Americas, several distinct variants exist 1:

Major Variants

• Pure motor variant (5-70% of cases): Motor weakness without sensory signs; may show normal or exaggerated reflexes in AMAN subtype 3, 1, 7
• Miller Fisher syndrome (5-25% of cases): Ophthalmoplegia, ataxia, and areflexia with minimal limb weakness 3, 1, 7
• Pharyngeal-cervical-brachial weakness: Weakness limited to upper limbs and bulbar muscles 3
• Paraparetic variant: Weakness restricted to lower limbs 3
• Bilateral facial palsy with paresthesias: Cranial nerve-limited presentation 3

Atypical Presentations

• Weakness can start in the arms, legs, or simultaneously in all limbs rather than following strict ascending pattern 3
• Severe diffuse pain or isolated cranial nerve dysfunction may precede weakness 3
• Young children (<6 years) may present with poorly localized pain, refusal to bear weight, irritability, meningism, or unsteady gait 3

Diagnostic Pattern Recognition

CSF Pattern

• Albuminocytologic dissociation (elevated protein with normal cell count) typically appears at the end of the first week and may persist until the third week 1, 7
• Critical caveat: Normal CSF protein in the first week does not exclude GBS 1
• Marked CSF pleocytosis should prompt reconsideration of the diagnosis 1

Electrophysiological Patterns

• AIDP (most common in Europe/North America): Demyelinating features including reduced conduction velocities, temporal dispersion, and conduction blocks 7, 4, 8
• AMAN/AMSAN (more common in Asia): Axonal degeneration pattern with reduced amplitudes but preserved conduction velocities 7, 8
• "Sural sparing pattern": Normal sural sensory nerve action potential with abnormal median/ulnar responses is typical 1

Prognostic Pattern

• Mortality: 3-10% even with optimal medical care 2
• Full recovery: Expected in approximately 90% of cases, though recovery may take up to 2 years 1
• Severe disability: Approximately 20% of patients remain severely disabled 8
• Recurrence: Rare, occurring in only 2-5% of cases 1, 2

Red Flags Suggesting Alternative Diagnosis

• Marked persistent asymmetry of weakness 1
• Bladder dysfunction at onset 1
• Progression beyond 8 weeks (consider A-CIDP) 4
• Marked CSF pleocytosis 1