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Guillain-Barré Spectrum Disorders
Surjith Vattoth, MD, FRCR
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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

                                        1. Al Othman B et al: Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 30(6):462-6, 2019
                                        2. Leonhard SE et al: Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 15(11):671-83, 2019
                                        3. 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
                                        4. Cuneo GL et al: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Neuroradiol J. 29(5):396-9, 2016
                                        5. Tyrakowska Z et al: Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. Pol J Radiol. 81:622-628, 2016
                                        6. 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
                                        7. 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
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        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

                                                                              1. Al Othman B et al: Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 30(6):462-6, 2019
                                                                              2. Leonhard SE et al: Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 15(11):671-83, 2019
                                                                              3. 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
                                                                              4. Cuneo GL et al: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Neuroradiol J. 29(5):396-9, 2016
                                                                              5. Tyrakowska Z et al: Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. Pol J Radiol. 81:622-628, 2016
                                                                              6. 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
                                                                              7. 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