Guillain-Barre syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction | *Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction | ||
*Associated with viral or febrile illness, campylobacter infection, or vaccination | *Although there is often a motor component, patients can also present with sensory deficits. | ||
*Associated with [[viral syndrome|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 | *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== | ==Clinical Features== | ||
*Viral illness | *[[Viral syndrome|Viral illness]] → ASCENDING, symmetric [[weakness]] or paralysis and loss of DTRs | ||
*May progress to diaphragm resulting in need for mechanical ventilation (33% of | *Little or no sensory involvement | ||
*Autonomic dysfunction occurs in 50% of | *May progress to diaphragm resulting in need for mechanical ventilation (33% of patients) | ||
*Autonomic dysfunction occurs in 50% of patients | |||
===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 | |||
===Variants and Subtypes=== | |||
*Multiple variants<ref>Ho TW et al. | |||
Guillain-Barrésyndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995;118 ( Pt 3):597. </ref><ref>Sumner AJ et al. The physiological basis for symptoms in Guillain-Barrésyndrome. Ann Neurol. 1981;9 Suppl:28. </ref> | |||
*Acute inflammatory demyelinating polyneuropathy | |||
**most common type | |||
**progressive symmetric muscle [[weakness]] | |||
**often with decreased deep tendon reflexes due to peripheral nerve involvement | |||
*Acute motor axonal neuropathy | |||
**often associated with [[campylobacter]] infections | |||
**only motor involvement | |||
*Acute motor and sensory axonal neuropathy | |||
**presence both motor and sensory involement | |||
*Miller-Fisher Syndrome | *Miller-Fisher Syndrome | ||
**Associated | **Associated with [[campylobacter]] infection | ||
**More likely to be preceded by diarrhea than viral prodrome | **More likely to be preceded by [[diarrhea]] than viral prodrome | ||
** | **Presence of ''ophthalmoplegia'' with [[ataxia]] and areflexia | ||
**Weakness is less severe but DESCENDING; disease course milder than classic GBS | **Weakness is less severe but DESCENDING; disease course milder than classic GBS | ||
**May present similarly to [[botulism]], which is also descending paralysis | |||
==Diagnosis== | ==Differential Diagnosis== | ||
{{Weakness DDX}} | |||
== | ==Evaluation== | ||
*[[ | *[[LP]] | ||
**'''Albumin-cytological dissociation of [[CSF]] (high protein (>45) and low WBC count (<10)) is most common | |||
**Patients with HIV can have a pleocytosis<ref name="Rosen">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.</ref><ref>Brannagan TH et al. HIV-associated Guillain-Barré syndrome. J Neurol Sci. 2003;208(1-2):39. </ref>''' | |||
*Typical findings on electromyogram and nerve conduction studies | |||
*MRI: Selective enhancement of the anterior spinal nerve roots is suggestive<ref name="Rosen" /> | |||
==Management== | |||
===[[IVIG]]=== | |||
*Treat non-ambulatory 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 | |||
*''Avoid'' [[succinylcholine]] during intubation, as this may cause severe hyperkalemia | |||
==Disposition== | ==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== | ==See Also== | ||
*[[Weakness]] | *[[Weakness]] | ||
== | ==References== | ||
<references/> | |||
[[Category: | [[Category:Neurology]] |
Revision as of 15:51, 14 September 2019
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
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
Variants and Subtypes
- Multiple variants[1][2]
- Acute inflammatory demyelinating polyneuropathy
- most common type
- progressive symmetric muscle weakness
- often with decreased deep tendon reflexes due to peripheral nerve involvement
- Acute motor axonal neuropathy
- often associated with campylobacter infections
- only motor involvement
- Acute motor and sensory axonal neuropathy
- presence both motor and sensory involement
- Miller-Fisher Syndrome
- Associated with campylobacter infection
- More likely to be preceded by diarrhea than viral prodrome
- Presence of ophthalmoplegia with ataxia and areflexia
- 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
- Upper motor neuron:
- CVA
- Hemorrhagic stroke
- Multiple sclerosis
- Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
- Lower motor neuron:
- Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
- Infection (Epidural abscess)
- Infarction/ischemia
- Trauma (Spinal Cord Syndromes)
- Inflammation (Transverse Myelitis)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Neuromuscular junction disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Upper motor neuron:
- 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
- Aortic disease - occlusion, stenosis, dissection
- Other causes of weakness and paralysis
- Acute intermittent porphyria (ascending weakness)
- Can't miss diagnoses:
Evaluation
- LP
- Typical findings on electromyogram and nerve conduction studies
- MRI: Selective enhancement of the anterior spinal nerve roots is suggestive[3]
Management
IVIG
- Treat non-ambulatory 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
- Avoid succinylcholine during intubation, as this may cause severe hyperkalemia
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.