Guillain-Barré Syndrome
A rare autoimmune disorder causing rapid-onset muscle weakness and paralysis
Quick Facts
- Type: Autoimmune Disorder
- ICD-10: G61.0
- Prevalence: 1-2 per 100,000 annually
- Onset: Rapid (days to weeks)
Overview
Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nervous system - the network of nerves outside the brain and spinal cord. This attack damages the protective covering (myelin sheath) of nerves and can also damage the nerve fibers themselves, leading to muscle weakness, loss of sensation, and in severe cases, paralysis.
GBS affects approximately 1-2 people per 100,000 each year worldwide. While it can occur at any age, it most commonly affects adults and older adults. The condition typically develops rapidly over days to weeks, often starting with tingling and weakness in the legs that spreads upward through the body. In most cases, people with GBS experience their worst symptoms within the first two weeks after symptoms begin.
The exact cause of GBS is unknown, but it often follows an infection, surgery, or vaccination. About two-thirds of people with GBS report having had an infection in the six weeks before their symptoms began. Despite its potentially serious nature, most people with GBS recover completely, though recovery can take months to years. Early diagnosis and treatment can significantly improve outcomes and reduce the risk of long-term complications.
Symptoms
GBS symptoms typically develop rapidly and can progress from mild to severe within days or weeks. The progression usually follows a specific pattern, starting in the extremities and moving toward the center of the body.
Early Symptoms
Progressive Symptoms
- Muscle weakness: Begins in legs and spreads upward to arms and face
- Loss of reflexes: Absent or diminished deep tendon reflexes
- Paralysis: In severe cases, can affect entire body
- Difficulty walking: Unsteady gait progressing to inability to walk
- Difficulty with eye movements: Double vision or inability to move eyes
- Swallowing difficulties: Problems eating or drinking
- Speech problems: Slurred speech or difficulty speaking
Serious Complications
In severe cases, GBS can affect vital functions:
- Breathing difficulties: Respiratory muscle weakness requiring ventilation
- Blood pressure fluctuations: Unstable cardiovascular function
- Heart rhythm abnormalities: Dangerous arrhythmias
- Bowel and bladder dysfunction: Loss of control
- Blood clots: Due to immobility
- Severe pain: Neuropathic pain that can be debilitating
Types of GBS and Their Symptoms
- Acute Inflammatory Demyelinating Polyneuropathy (AIDP):
- Most common form in North America and Europe
- Weakness spreads from legs upward
- Affects myelin sheath primarily
- Acute Motor Axonal Neuropathy (AMAN):
- More common in Asia and Central/South America
- Affects motor nerves primarily
- May have more rapid recovery
- Miller Fisher Syndrome:
- Rare variant affecting eye muscles
- Double vision, loss of reflexes, coordination problems
- Usually doesn't affect limbs severely
Timeline of Symptoms
- Days 1-7: Initial tingling, pain, and mild weakness
- Days 7-14: Progressive weakness, loss of reflexes
- Weeks 2-4: Peak disability, potential respiratory involvement
- Weeks 4-12: Stabilization and beginning of recovery
- Months to years: Gradual improvement and rehabilitation
Causes
The exact cause of Guillain-Barré Syndrome is unknown, but it's believed to be an autoimmune condition triggered by an infection or other immune system stimulus. The immune system mistakenly attacks the body's own nerves.
Autoimmune Mechanism
In GBS, the immune system produces antibodies that attack components of the peripheral nervous system:
- Molecular mimicry: Immune system confuses nerve proteins with foreign antigens
- Myelin attack: Antibodies destroy the protective myelin sheath around nerves
- Axonal damage: In severe cases, the nerve fibers themselves are damaged
- Inflammatory response: White blood cells invade peripheral nerves
Triggering Infections
About two-thirds of GBS cases follow an infection within 2-6 weeks:
Bacterial Infections
- Campylobacter jejuni: Most common bacterial trigger (25-50% of cases)
- Mycoplasma pneumoniae: Respiratory tract infections
- Haemophilus influenzae: Respiratory infections
- Salmonella: Gastrointestinal infections
Viral Infections
- Epstein-Barr virus (EBV): Mononucleosis
- Cytomegalovirus (CMV): Various systemic symptoms
- Influenza viruses: Seasonal flu
- Zika virus: Associated with GBS outbreaks
- COVID-19: Rare but reported association
- Hepatitis viruses: A, B, C, and E
Other Triggers
- Surgery: Major surgical procedures
- Vaccinations: Very rare trigger (1-2 cases per million doses)
- Benefits of vaccination far outweigh risks
- Historical association with 1976 swine flu vaccine
- Modern vaccines have much lower risk
- Trauma: Physical injury or emotional stress
- Pregnancy: Rare trigger during pregnancy or postpartum
- Cancer: Hodgkin's lymphoma and other malignancies
- Bone marrow transplant: Following stem cell transplantation
Genetic Factors
- HLA associations: Certain genetic markers increase susceptibility
- Not directly inherited: Family clustering is extremely rare
- Population differences: Genetic factors may explain geographic variations
Environmental Factors
- Geographic variations: Different subtypes more common in different regions
- Seasonal patterns: Slight increase in respiratory infection seasons
- Age-related factors: Different triggers at different ages
Unknown Triggers
In about one-third of cases, no clear trigger can be identified. These cases may involve:
- Subclinical infections that went unnoticed
- Environmental toxins or chemicals
- Genetic susceptibility factors
- Unknown viral or bacterial agents
Risk Factors
While anyone can develop GBS, certain factors may increase the risk:
Age Factors
- Adults and older adults: Most common in people over 50
- All ages affected: Can occur from infancy to elderly
- Age-related severity: Older patients may have more severe disease
- Recovery differences: Younger patients typically recover better
Gender Factors
- Male predominance: Men slightly more affected than women (1.5:1 ratio)
- Age-related differences: Gender ratios vary by age group
- Pregnancy considerations: Slight increased risk during pregnancy
Geographic and Environmental Factors
- Worldwide distribution: Occurs in all countries and climates
- Regional variations: Different subtypes more common in different areas
- Developing countries: Higher rates of Campylobacter-associated GBS
- Seasonal patterns: Slight increase during respiratory infection seasons
Medical History Factors
- Recent infections: Especially gastrointestinal or respiratory
- Autoimmune conditions: May have increased susceptibility
- Cancer treatment: Chemotherapy and radiation may increase risk
- Bone marrow transplant: Higher risk in transplant recipients
- Surgery: Major surgical procedures may trigger GBS
Genetic Susceptibility
- HLA markers: Certain genetic markers associated with increased risk
- Population genetics: Some ethnic groups have different risk patterns
- Family history: Extremely rare but reported familial cases
Factors NOT Associated with Increased Risk
It's important to note that the following do NOT increase GBS risk:
- Routine vaccinations (risk is extremely low)
- Minor illnesses or injuries
- Stress alone (without other triggers)
- Diet or lifestyle factors
- Environmental exposures in general
Recurrence Risk
- Very rare: Less than 2-5% of people have recurrent GBS
- Chronic forms: Some people develop chronic inflammatory demyelinating polyneuropathy (CIDP)
- Monitoring needed: People with one episode should be aware of symptoms
Diagnosis
Diagnosing GBS requires a combination of clinical assessment, laboratory tests, and specialized studies. Early diagnosis is crucial for optimal treatment outcomes.
Clinical Assessment
- Medical history: Recent infections, surgeries, or vaccinations
- Symptom progression: Pattern and timeline of weakness development
- Neurological examination: Assessment of muscle strength, reflexes, and sensation
- Functional assessment: Ability to walk, swallow, and breathe
Diagnostic Criteria
The diagnosis of GBS is based on clinical features and supporting tests:
Required Features
- Progressive weakness in more than one limb
- Absent or diminished deep tendon reflexes
Supportive Features
- Progression over days to 4 weeks
- Relative symmetry of symptoms
- Mild sensory symptoms or signs
- Cranial nerve involvement
- Recovery beginning 2-4 weeks after progression stops
Laboratory Tests
Cerebrospinal Fluid (CSF) Analysis
- Lumbar puncture: Sampling fluid from around spinal cord
- Protein elevation: Usually >0.45 g/L (normal: 0.15-0.45 g/L)
- Cell count: Usually normal (<10 cells/μL)
- Albuminocytologic dissociation: High protein with normal cell count
Blood Tests
- Complete blood count: Rule out other conditions
- Inflammatory markers: ESR, CRP may be elevated
- Antibody testing: GM1, GD1a, GQ1b antibodies in some cases
- Infection markers: Testing for recent Campylobacter, CMV, EBV
Nerve Conduction Studies
- Electrodiagnostic testing: Measures nerve function
- Conduction velocity: Slowed in demyelinating forms
- Conduction blocks: Partial or complete nerve blocks
- F-wave studies: Tests nerve root function
- Electromyography (EMG): Tests muscle response to nerve stimulation
Imaging Studies
- MRI of spine: May show nerve root enhancement
- Chest X-ray: Assess respiratory function
- CT scans: Rule out other conditions if needed
Specialized Testing
- Pulmonary function tests: Monitor breathing capacity
- Cardiac monitoring: Watch for rhythm abnormalities
- Autonomic function tests: Assess blood pressure and heart rate control
Differential Diagnosis
Other conditions to consider and rule out:
- Myasthenia gravis
- Acute transverse myelitis
- Botulism
- Tick paralysis
- Poliomyelitis
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
- Spinal cord compression
- Metabolic disorders (porphyria, critical illness polyneuropathy)
- Toxic neuropathies
Monitoring and Assessment Tools
- GBS Disability Scale: 0 (normal) to 6 (death)
- Medical Research Council (MRC) Sum Score: Muscle strength assessment
- Hughes Functional Scale: Functional disability assessment
- Respiratory monitoring: Vital capacity measurements
Treatment Options
Treatment for GBS focuses on reducing the immune system's attack on the nervous system, managing symptoms, and preventing complications. Early treatment is crucial for the best outcomes.
Acute Treatment
Immunotherapy
Two main treatments are equally effective for acute GBS:
- Intravenous Immunoglobulin (IVIG):
- Dose: 0.4 g/kg daily for 5 days
- Pooled antibodies from healthy donors
- Works by modulating immune response
- Preferred in many centers due to easier administration
- Fewer side effects than plasma exchange
- Plasma Exchange (Plasmapheresis):
- Removes harmful antibodies from blood
- Typically 4-6 sessions over 8-10 days
- Requires central venous access
- May be preferred in severe cases
- Cannot be combined with IVIG
Corticosteroids
- Not recommended: Studies show no benefit and may delay recovery
- May worsen outcomes: Can be harmful in GBS
- Exception: May be used for chronic forms like CIDP
Supportive Care
Respiratory Support
- Monitoring: Regular vital capacity measurements
- Criteria for intubation:
- Vital capacity <20 mL/kg
- Maximal inspiratory pressure <30 cmH2O
- Rapid decline in respiratory function
- Inability to clear secretions
- Mechanical ventilation: May be needed for weeks to months
- Tracheostomy: For prolonged ventilation (>2-3 weeks)
Cardiovascular Monitoring
- Blood pressure management: Treat hypertension and hypotension
- Cardiac monitoring: Watch for arrhythmias
- Avoid sudden position changes: Prevent orthostatic hypotension
- Fluid management: Maintain adequate hydration
Pain Management
- Neuropathic pain medications:
- Gabapentin: 300-3600 mg daily in divided doses
- Pregabalin: 150-600 mg daily in divided doses
- Tricyclic antidepressants: Amitriptyline 25-100 mg at bedtime
- Opioids: May be needed for severe pain (short-term use)
- Non-pharmacological:
- Physical therapy
- Positioning and support
- Heat/cold therapy
- Relaxation techniques
Rehabilitation
Physical Therapy
- Early mobilization: Prevent contractures and maintain range of motion
- Strengthening exercises: Gradual muscle strengthening as recovery occurs
- Gait training: Relearn walking skills
- Balance training: Improve stability and prevent falls
Occupational Therapy
- Activities of daily living: Relearn basic self-care skills
- Fine motor skills: Hand and finger coordination
- Adaptive equipment: Tools to assist with daily tasks
- Work rehabilitation: Return to employment when appropriate
Speech Therapy
- Swallowing assessment: Evaluate and treat dysphagia
- Communication aids: Alternative communication methods
- Breathing exercises: Strengthen respiratory muscles
Prevention of Complications
- Deep vein thrombosis:
- Compression stockings
- Anticoagulation when appropriate
- Early mobilization
- Pressure ulcers:
- Regular position changes
- Specialized mattresses
- Skin care protocols
- Infections:
- Respiratory hygiene
- Urinary catheter care
- Wound care
Psychological Support
- Counseling: Help cope with sudden disability
- Family support: Education and support for caregivers
- Antidepressants: If depression develops
- Support groups: Connect with other GBS survivors
Long-term Management
- Regular follow-up: Monitor recovery progress
- Ongoing rehabilitation: Continue therapy as needed
- Fatigue management: Address persistent fatigue
- Return to activities: Gradual resumption of normal activities
- Vaccination considerations: Discuss future vaccination risks/benefits
Prevention
Since the exact cause of GBS is unknown and it's often triggered by common infections, complete prevention isn't possible. However, some strategies may help reduce risk:
General Health Measures
- Good hygiene: Regular handwashing to prevent infections
- Food safety: Proper food handling to prevent Campylobacter infections
- Cook poultry thoroughly (165°F/74°C internal temperature)
- Avoid cross-contamination between raw and cooked foods
- Wash hands after handling raw meat
- Use separate cutting boards for meat and vegetables
- Safe drinking water: Avoid contaminated water sources
- Wound care: Proper cleaning and care of cuts and injuries
Infection Prevention
- Respiratory infections:
- Annual flu vaccination
- Pneumococcal vaccination as recommended
- Avoid close contact with sick individuals
- Practice respiratory etiquette (cover coughs and sneezes)
- Gastrointestinal infections:
- Practice food safety
- Avoid contaminated water
- Wash fruits and vegetables thoroughly
Vaccination Considerations
- Continue routine vaccinations: Benefits far outweigh minimal GBS risk
- Risk is extremely low: 1-2 cases per million vaccine doses
- Infections carry higher GBS risk: Than vaccines preventing those infections
- Special considerations: For people with previous GBS
- Discuss with healthcare provider
- Individual risk-benefit assessment
- May still receive vaccines with careful monitoring
For High-Risk Individuals
- Immunocompromised patients:
- Extra precautions against infections
- Avoid live vaccines when contraindicated
- Prompt treatment of infections
- Elderly individuals:
- Up-to-date vaccinations
- Good nutrition and exercise
- Regular medical care
Lifestyle Factors
- Healthy immune system:
- Adequate sleep (7-9 hours nightly)
- Regular exercise
- Balanced nutrition
- Stress management
- Avoid smoking
- Limit alcohol consumption
- Medical care:
- Regular check-ups
- Prompt treatment of infections
- Manage chronic conditions properly
Secondary Prevention
For people who have had GBS:
- Recurrence is rare: <5% chance of developing GBS again
- Monitor for symptoms: Be aware of early warning signs
- Prompt medical attention: Seek care if symptoms return
- Vaccination decisions: Discuss with healthcare provider
- Infection prevention: Extra care to avoid triggering infections
Travel Considerations
- Travel vaccinations: Get appropriate vaccines for destination
- Food and water safety: Extra precautions in developing countries
- Insect protection: Prevent vector-borne diseases
- Travel insurance: Ensure coverage for medical emergencies
Public Health Measures
- Surveillance: Monitoring for GBS outbreaks
- Food safety regulations: Preventing foodborne illnesses
- Vaccination programs: Preventing infections that can trigger GBS
- Research: Better understanding of GBS causes and prevention
When to See a Doctor
GBS is a medical emergency that requires immediate attention. Early recognition and treatment can significantly improve outcomes:
Call Emergency Services (911) Immediately
- Breathing difficulties: Shortness of breath, unable to take deep breaths
- Severe weakness: Inability to walk or stand
- Swallowing problems: Choking, drooling, unable to swallow
- Speech difficulties: Severe slurring or inability to speak
- Rapid progression: Weakness spreading quickly over hours
- Double vision: Sudden onset of seeing double
- Loss of consciousness: Fainting or altered mental status
Seek Immediate Medical Care (Emergency Room)
- Progressive weakness: Weakness that gets worse over days
- Starting in legs and moving upward
- Affecting both sides of body
- Difficulty walking or climbing stairs
- Numbness and tingling: In hands, feet, legs, or arms
- Pins-and-needles sensation
- Loss of feeling in extremities
- Spreading pattern of symptoms
- Loss of reflexes: Doctor unable to elicit normal reflexes
- Coordination problems: Sudden onset of clumsiness or unsteadiness
- Severe pain: Especially nerve pain or muscle aches
Schedule Urgent Medical Appointment
- Following recent illness: New neurological symptoms after infection
- Gastrointestinal illness in past 6 weeks
- Respiratory infection recently
- Any infection followed by weakness
- Mild but concerning symptoms:
- Persistent tingling in hands or feet
- Mild weakness that's worsening
- Balance problems
- Unusual fatigue with other symptoms
Red Flag Symptoms
These symptoms require immediate emergency evaluation:
- Any breathing difficulty or shortness of breath
- Weakness that prevents walking
- Inability to move arms or legs
- Severe pain that's getting worse
- Problems swallowing food or saliva
- Slurred speech or difficulty speaking
- Double vision or other vision changes
- Rapid progression of any neurological symptoms
For People with Previous GBS
- Any return of symptoms: Even mild neurological symptoms
- New weakness: Different from baseline function
- After infections: Monitor closely for symptom recurrence
- Before vaccinations: Discuss with healthcare provider
What to Tell Healthcare Providers
- Symptom timeline: When symptoms started and how they've progressed
- Recent illnesses: Any infections in past 6 weeks
- Recent procedures: Surgeries, vaccinations, or medical procedures
- Current symptoms: Detailed description of weakness, numbness, pain
- Functional impact: What activities you can and cannot do
- Family history: Any neurological conditions in family
- Medications: All current medications and supplements
Preparation for Emergency Visit
- Bring someone with you: For support and to help communicate
- Medical records: Recent test results if available
- Medication list: All current prescriptions and over-the-counter drugs
- Insurance information: Healthcare coverage details
- Emergency contacts: Family members to notify
Don't Wait if Symptoms Are Worsening
GBS can progress rapidly, and early treatment is crucial. It's better to seek medical care early and be evaluated than to wait for symptoms to worsen. Healthcare providers would rather evaluate someone with early symptoms than see someone after serious complications have developed.
Frequently Asked Questions
Most people with GBS recover completely or nearly completely. About 80% of people are able to walk independently within 6 months, and many recover fully within a year. However, some people may have residual weakness, fatigue, or numbness. The extent of recovery depends on the severity of the initial illness and how quickly treatment was started.
Recovery time varies greatly between individuals. The acute phase typically lasts 2-4 weeks, followed by a recovery phase that can take months to years. Most improvement occurs in the first year, with some people continuing to improve for up to 2 years. Younger patients and those with less severe disease generally recover faster and more completely.
Recurrence of GBS is rare, occurring in less than 2-5% of people. However, some people may develop chronic inflammatory demyelinating polyneuropathy (CIDP), which is a chronic form of the condition. Anyone who has had GBS should be aware of symptoms and seek medical attention if neurological symptoms return.
No, GBS itself is not contagious. You cannot catch GBS from someone who has it. However, the infections that sometimes trigger GBS (like stomach flu or respiratory infections) can be contagious. GBS is an autoimmune reaction that occurs in susceptible individuals following certain triggers.
This is a complex question that should be discussed with your healthcare provider. While there is a very small increased risk of GBS after certain vaccinations, the risk of GBS from the actual infections that vaccines prevent is often higher. Your doctor will help you weigh the risks and benefits based on your individual situation and medical history.
While both are autoimmune conditions affecting the nervous system, they are quite different. GBS affects the peripheral nervous system (nerves outside the brain and spinal cord) and typically occurs as a single episode with recovery. Multiple sclerosis affects the central nervous system (brain and spinal cord) and is typically a chronic, progressive condition with relapses and remissions.
References
- Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2023.
- Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2023.
- van den Berg B, Walgaard C, Drenthen J, et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2023.
- Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2023.
- Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2023.