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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE)
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN)
Definitions
- Immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections
- Classic GBS: Acute-onset, rapidly progressive, ascending sensorimotor neuropathy; absent or ↓ tendon reflexes
- Initially in legs, progressing to arms and cranial muscles
- Usually reach maximum disability within 2 weeks
- Electrophysiological subtypes: AIDP, AMAN, AMSAN
- Clinical variants of GBS: Do not characteristically progress to classic GBS pattern of sensory loss and weakness
- Usually not "pure"; often partial overlap with classic GBS or other variants
- Pure motor variant: Motor weakness; no sensory signs
- Paraparetic variant: Paresis in lower limbs only
- Bilateral facial palsy with paraesthesias variant: Weakness limited to cranial nerves; ↓ reflexes
- Pharyngeal-cervical-brachial (PCB) variant: PCB muscle weakness, no lower limb weakness
- MFS: Ophthalmoplegia, areflexia, ataxia
- 15% overlap classic GBS
- Incomplete forms: Acute ataxic neuropathy (isolated ataxia); acute ophthalmoplegia
- Other disorders often included in GBS spectrum: Due to similar clinical or pathophysiological features
- Inclusion in GBS spectrum debatable as these do not fulfil GBS diagnostic criteria
- Pure sensory variant: Overlapping clinical features of classic GBS, except motor signs and symptoms
- Pure sensory ataxia: Overlap with MFS
- BBE: Initially ophthalmoplegia, ataxia, areflexia (like MFS), later brainstem dysfunction, e.g., ↓ consciousness and pyramidal tract signs; often overlap classic GBS
IMAGING
General Features
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Al Othman B et al: Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 30(6):462-6, 2019
- Leonhard SE et al: Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 15(11):671-83, 2019
- Malhotra A et al: MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature. J Clin Neurosci. 39:63-7, 2017
- Cuneo GL et al: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Neuroradiol J. 29(5):396-9, 2016
- Tyrakowska Z et al: Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. Pol J Radiol. 81:622-628, 2016
- Zuccoli G et al: Redefining the Guillain-Barré spectrum in children: neuroimaging findings of cranial nerve involvement. AJNR Am J Neuroradiol. 32(4):639-42, 2011
- Inoue N et al: MR imaging findings of spinal posterior column involvement in a case of Miller Fisher syndrome. AJNR Am J Neuroradiol. 25(4):645-8, 2004
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE)
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN)
Definitions
- Immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections
- Classic GBS: Acute-onset, rapidly progressive, ascending sensorimotor neuropathy; absent or ↓ tendon reflexes
- Initially in legs, progressing to arms and cranial muscles
- Usually reach maximum disability within 2 weeks
- Electrophysiological subtypes: AIDP, AMAN, AMSAN
- Clinical variants of GBS: Do not characteristically progress to classic GBS pattern of sensory loss and weakness
- Usually not "pure"; often partial overlap with classic GBS or other variants
- Pure motor variant: Motor weakness; no sensory signs
- Paraparetic variant: Paresis in lower limbs only
- Bilateral facial palsy with paraesthesias variant: Weakness limited to cranial nerves; ↓ reflexes
- Pharyngeal-cervical-brachial (PCB) variant: PCB muscle weakness, no lower limb weakness
- MFS: Ophthalmoplegia, areflexia, ataxia
- 15% overlap classic GBS
- Incomplete forms: Acute ataxic neuropathy (isolated ataxia); acute ophthalmoplegia
- Other disorders often included in GBS spectrum: Due to similar clinical or pathophysiological features
- Inclusion in GBS spectrum debatable as these do not fulfil GBS diagnostic criteria
- Pure sensory variant: Overlapping clinical features of classic GBS, except motor signs and symptoms
- Pure sensory ataxia: Overlap with MFS
- BBE: Initially ophthalmoplegia, ataxia, areflexia (like MFS), later brainstem dysfunction, e.g., ↓ consciousness and pyramidal tract signs; often overlap classic GBS
IMAGING
General Features
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Al Othman B et al: Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 30(6):462-6, 2019
- Leonhard SE et al: Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 15(11):671-83, 2019
- Malhotra A et al: MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature. J Clin Neurosci. 39:63-7, 2017
- Cuneo GL et al: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Neuroradiol J. 29(5):396-9, 2016
- Tyrakowska Z et al: Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. Pol J Radiol. 81:622-628, 2016
- Zuccoli G et al: Redefining the Guillain-Barré spectrum in children: neuroimaging findings of cranial nerve involvement. AJNR Am J Neuroradiol. 32(4):639-42, 2011
- Inoue N et al: MR imaging findings of spinal posterior column involvement in a case of Miller Fisher syndrome. AJNR Am J Neuroradiol. 25(4):645-8, 2004
