guillain -barré syndrome · objectives pathophysiology of guillain barré syndrome (gbs)...
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Seema Yasmin, MD
EIS Field Assignments Branch Arizona Department of Health Services
Guillain-Barré Syndrome
Office of Surveillance, Epidemiology, and Laboratory Services Scientific Education and Professional Development Program Office
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Object ives
Pathophysiology of Guillain Barré Syndrome (GBS) Epidemiology Signs and symptoms Review of GBS subtypes Diagnosis, t reatment, prognosis Outbreaks
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Definit ion
First described in 1916
Acute flaccid paralysis, areflexia, protein in CSF
Flaccid: Voluntary control over muscles has been lost limp, floppy muscles
Commonest cause of acute flaccid paralysis
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Epidemiology
0.9–1.9/100, 000 persons
Incidence differs with age <30 yrs: <1/100,000 >75 yrs: >4/100,000
20% increase with every 10-year rise in age
Male to female rat io – 1.2–1.5:1
Sejvar et al, Neuroepidemiology2011; 36: 123-133
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Pathophysiology
Multiple Sclerosis Trust, UK
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Pathophysiology
Multiple Sclerosis Trust, UK
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Pathophysiology
Multiple Sclerosis Trust, UK
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Nervous System
Morphonix.com
Central nervous system Brain and spinal cord
Peripheral nervous system Somatic - voluntary Autonomic - involuntary
GBS affects the 2 components of the peripheral nervous system
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Neurology Definit ions
Paralysis – loss of voluntary muscle funct ion Partial – affecting one muscle or one limb Total – paralysis of all muscles
Plegia – used to describe paralysis Palsy – not used anymore except for ‘Bell’s palsy’
Paresis – weakness of voluntary movement
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Neurology Definit ions
Hemi – arm, leg, t runk on the same side of the body Hemiplegia – total paralysis on one side of the body Hemiparesis – weakness on one side of the body
Para – either both arms or both legs
Quadri – all 4 limbs and torso
Quadriplegia is the same as tetraplegia
Paresthesia – t ingling, burning, numb sensat ion
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Variants
Autoant ibodies (usually IgG) attack gangliosides Part of the cell membrane involved in signal transduction
N Engl J Med 2012; 366:2294-2304
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Five Variants of GBS
N Engl J Med 2012; 366:2294-2304
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Geographic Distribut ion of GBS Subtypes
Demyelinat ing Autoantibodies attack the myelin sheath
Axonal
Autoantibodies attack nerve axon
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Geographic Distribut ion of GBS Subtypes
Demyelinat ing Autoantibodies attack the myelin sheath 90% of all cases in Europe and North America 22-46% of cases in China, Japan, Bangladesh, Mexico
Axonal
Autoantibodies attack nerve axon
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Geographic Distribut ion of GBS Subtypes
Demyelinat ing Autoantibodies attack the myelin sheath 90% of all cases in Europe and North America 22-46% of cases in China, Japan, Bangladesh, Mexico
Axonal
Autoantibodies attack nerve axon 30-65% of all cases in China, Japan, Bangladesh, Mexico
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Guillain Barré Syndrome
Acute, ascending paralysis Decreased or absent reflexes Elevated protein in the CSF but normal cell count
Autoant ibodies to myelin sheath or axon of nerve cells
5 variants
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Acute Inflammatory Demyelinat ing Polyneuropathy (AIDP)
Commonest variant of GBS in North America, Europe Paresthesias in distal limbs before weakness Ascending paralysis, progressive weakness
Bilateral, symmetrical At presentation, 60% of patients have weakness in all 4 limbs >60% unable to walk independently when maximum weakness is
reached
Respiratory weakness Presenting symptom in 40% of patients
Autonomic dysfunct ion Postural hypotension syncope Sinus tachycardia
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Miller Fisher
Occurs in 5% of GBS cases Classic triad of ophthalmoplegia, ataxia and areflexia Present ing complaint usually diplopia Elevated CSF protein but less than other types of GBS
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Acute Motor Axonal Neuropathy (AMAN)
Acute/subacute paralysis or paresis without any sensory loss
Loss of reflexes Facial/oropharyngeal muscle weakness Associated with Campylobacter jejuni gastric enterit is
>60% seropositive
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Aetiology of GBS: Triggering Event
>60% of cases preceded by upper respiratory tract infect ion or diarrhea 3 days–6 weeks prior to symptoms
Campylobacter jejuni most frequent ly ident ified infect ious agent (30%) Incidence: 0.25–0.65/1000 cases of C.jejuni
Cytomegalovirus (10%)
Epstein-Barr virus, varicella-zoster, Mycoplasma
pneumoniae
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Immunizat ions and GBS
Lit t le evidence to support a causal associat ion with most vaccines
Older formulat ions of rabies vaccine cultured in mammalian brain t issue (Semple)
Swine flu vaccine 1976–77
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Time Course
Pain 2 weeks after t riggering event
Weakness worsens over 2 weeks
Symptoms plateau at 4 weeks
Recovery begins
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Different ial Diagnosis
Acute peripheral neuropathies Toxic: thallium, arsenic, lead, n-hexane, organophosphate Drugs: amiodarone, perhexiline, gold Alcohol Porphyria Systemic vasculitis Poliomyelitis Diphtheria Tick paralysis
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Different ial Diagnosis
Disorders of Neuromuscular Transmission Botulism Myasthenia gravis
Central Nervous System Disorders
Basilar artery occlusion Acute cervical transverse myelitis
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Diagnosis
History
Examinat ion
Lumbar puncture Elevated CSF protein without pleocytosis
Nerve and muscle funct ion tests
Electromyography – is muscle weakness due to the muscle itself? Nerve conduction studies
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Case Definit ion
J Sejvar et al, Vaccine 29 (2011) 599–612
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Nerve Conduct ion Tests
Motor and sensory nerves
Nerve conduct ion velocity is measured Conduction slowing Conduction block
F waves and H reflexes Prolonged or absent
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Treatment
Support ive care Intubation Occupational therapy Physiotherapy Speech and language therapy
Specific therapy
Intravenous immunoglobulins Plasmapheresis
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Prognosis
Recovery starts at ~2–4 weeks after symptom onset Months to years
20% unable to walk 6 months after symptom onset
Time to recovery depends on many factors Age Severity Delay in receiving treatment
Various prognost ic scales Patient’s age, presence/absence of antecedent diarrhea, severity Time between weakness onset and admission, facial weakness
Complicat ions of intubat ion Pneumonia, sepsis, PE in 60%
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Prognosis Depends on Subtype
AIDP 5% mortality >75% of patients have complete/near-complete recovery, no
deficits or with mild residual fatigue and distal weakness
Miller Fisher
Improvement begins at a median of 2 weeks Full recovery takes a median of 1-3 months
AMAN
Recovery time similar to or quicker than AIDP
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GBS Surveillance
1976 influenza 40 million doses of influenza vaccine administered in US Cluster of GBS cases noted Vaccine associated with small, but statistically significant increased
risk of GBS in 6 weeks post-vaccination
CDC Emerging Infect ions Program (EIP) 2009–10 Active, population-based surveillance Connecticut, Maryland, New Mexico, Tennessee, New York,
Minnesota Metropolitan areas in Georgia, Oregon, California, Colorado
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Key Points
GBS characterised by: Muscle weakness or paralysis Loss of reflexes High protein in the CSF but a normal cell count
Not a single disease but a group of immune-mediated
neuropathies
Autoant ibodies against cell membrane gangliosides
Clusters are rare
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Outbreaks
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Outbreaks
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Outbreaks
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Outbreaks
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Office of Surveillance, Epidemiology, and Laboratory Services Scientific Education and Professional Development Program Office
Acknowledgments
The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention
Arizona Department of Health Services
Ken Komatsu Cara Christ
Shoana Anderson Joli Weiss
Maricopa County Department of Public Health Rebecca Sunenshine
NCEZID, CDC
Jim Sejvar
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EXTRA SLIDES
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Risk Factors Older age Male Recent gastrointest inal or respiratory infect ion Recent surgery History of lymphoma, lupus, AIDS