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Guillain-Barre syndrome
From WikEM
(Redirected from Guillain-Barré Syndrome)
Contents
Background
- Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction. Although there is often a motor component, patients can also present with sensory deficits.
- Associated with viral or febrile illness, campylobacter infection, or vaccination
- Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months
- Associated with Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae
Clinical Features
- Viral illness → ASCENDING, symmetric weakness or paralysis and loss of DTRs
- Little or no sensory involvement
- May progress to diaphragm resulting in need for mechanical ventilation (33% of patients)
- Autonomic dysfunction occurs in 50% of patients
Variants and Subtypes
There are multiple Guillain Barre variants with differing presentaions[1][2]
- Acute inflammatory demyelinating polyneuropathy - most common type with progressive symmetric muscle weakness often with decreased deep tendon reflexes due to peripheral nerve involvment
- Acute motor axonal neuropathy - often associated with campylobacter infections with only motor involvement
- Acute motor and sensory axonal neuropathy - presence both motor and sensory involement
- Miller-Fisher Syndrome - presence of ophthalmoplegia with ataxia and areflexia
Miller-Fisher Syndrome
- Associated with campylobacter infection
- More likely to be preceded by diarrhea than viral prodrome
- Consists of ophthalmoplegia and ataxia
- Weakness is less severe but DESCENDING; disease course milder than classic GBS
- May present similarly to botulism, which is also descending paralysis
Differential Diagnosis
Weakness
- Neuromuscular weakness
- UMN:
- Spinal cord disease:
- Infection (Epidural abscess)
- Infarction/ischemia
- Trauma (Spinal Cord Syndromes)
- Inflammation (Transverse Myelitis)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Guillain-Barre syndrome
- Toxins (Ciguatera)
- Tick paralysis
- DM neuropathy (non-emergent)
- NMJ disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Non-neuromuscular weakness
- Can't miss diagnoses:
- ACS
- Arrhythmia/Syncope
- severe infection/Sepsis
- Hypoglycemia
- Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
- Respiratory failure
- Emergent Diagnoses:
- Symptomatic Anemia
- Severe dehydration
- Hypothyroidism
- Polypharmacy
- Malignancy
- Other causes of weakness and paralysis
- Acute intermittent porphyria (ascending weakness)
- Can't miss diagnoses:
Evaluation
Required
- Progressive weakness of more than one limb
- Areflexia
Suggestive
- Progression over days to weeks
- Recovery beginning 2–4 wk after cessation of progression
- Relative symmetry of symptoms
- Mild sensory signs and symptoms
- CN involvement (Bell's Palsy, dysphagia, dysarthria, ophthalmoplegia)
- Autonomic dysfunction
- Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
- Urinary Retention
- Constipation
- Facial flushing
- Absence of fever at onset
- Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count (<10)) is most common. However patients with HIV can have a pleocytosis[3][4]
- Typical findings on electromyogram and nerve conduction studies
- MRI: Selective enhancement of the anterior spinal nerve roots is suggestive[3]
Management
IVIG
- Treat nonambulatory patients within 2 weeks of symptom onset
IVIG vs Plasmapheresis
- IVIG associated with thromboembolism and aseptic meningitis
- Plasmapheresis associated with greater hemodynamic instability, lower rate of relapse
- Combined IVIG and plasmapheresis no better than single therapy (IVIG or plasmapheresis)
- IVIG preferred due to convenience and availability
Intubation indications
- Vital capacity <15mL/kg
- Negative Inspiratory Force < 30 cm H2O
- PaO2 <70 mm Hg on room air
- Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
- Aspiration
Disposition
Indications for admission to ICU
- Autonomic dysfunction
- Bulbar dysfunction
- Initial vital capacity <20 mL/kg
- Initial negative inspiratory force <–30 cm of water
- Decrease of >30% of vital capacity or negative inspiratory force
- Inability to ambulate
- Treatment with plasmapheresis
- Anticipated clinical course requiring mechanical ventilation
See Also
References
- ↑ Ho TW et al. Guillain-Barrésyndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995;118 ( Pt 3):597.
- ↑ Sumner AJ et al. The physiological basis for symptoms in Guillain-Barrésyndrome. Ann Neurol. 1981;9 Suppl:28.
- ↑ 3.0 3.1 Bunney EB, Gallagher EJ: Peripheral Nerve Disorders, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 105:p 1400-1401.
- ↑ Brannagan TH et al. HIV-associated Guillain-Barré syndrome. J Neurol Sci. 2003;208(1-2):39.
