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title, docid, authors, breadcrumbs, category, documentVersionId, imageCount, lastUpdated, pageDescription, pageKeywords, pageTitle, enhancedTitle, type, references, breadcrumbs
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| Guillain-Barré Spectrum Disorders | c1f52a65-920e-4e28-8a75-07dfa208f290 |
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Brain | 52016b28-7710-43a4-8cca-e659ab8227cf | 5 | 06/12/20 | Guillain-Barré Spectrum Disorders | Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Guillain-Barré Spectrum Disorders | Guillain-Barré Spectrum Disorders | STATdx | Guillain-Barré Spectrum Disorders | DX | true |
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title: "Guillain-Barr\u00e9 Spectrum Disorders" docid: "c1f52a65-920e-4e28-8a75-07dfa208f290" authors:
- key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" value: "Surjith Vattoth, MD, FRCR" breadcrumbs:
- name: "Brain" slug: "brain" treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
- name: "Diagnosis" slug: "diagnosis" treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
- name: "Pathology-Based Diagnoses" slug: "pathology-based-diagnoses" treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77"
- name: "Infectious, Inflammatory, and Demyelinating Disease" slug: "infectious-inflammatory-and-demyel-" treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607"
- name: "Inflammatory and Demyelinating Disease" slug: "inflammatory-and-demyelinating-dis-" treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc"
- name: "Guillain-Barr\u00e9 Spectrum Disorders" slug: "guillain-barr-spectrum-disorders" treeNodeId: null category: "Brain" documentVersionId: "52016b28-7710-43a4-8cca-e659ab8227cf" imageCount: 5 lastUpdated: "06/12/20" pageDescription: "Guillain-Barr\u00e9 Spectrum Disorders" pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Guillain-Barr\u00e9 Spectrum Disorders" pageTitle: "Guillain-Barr\u00e9 Spectrum Disorders | STATdx" enhancedTitle: "Guillain-Barr\u00e9 Spectrum Disorders" type: "DX" references: true breadcrumbs:
- "Brain"
- "Diagnosis"
- "Pathology-Based Diagnoses"
- "Infectious, Inflammatory, and Demyelinating Disease"
- "Inflammatory and Demyelinating Disease"
- "Guillain-Barr\u00e9 Spectrum Disorders"
KEY FACTS
-
Terminology
- Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE)
- Classic GBS: Acute-onset, rapidly progressive, ascending sensorimotor neuropathy; absent or ↓ tendon reflexes
- Clinical variants of GBS: Pure motor, paraparetic, bilateral facial palsy with paraesthesias, pharyngeal-cervical-brachial (PCB) variants and MFS
- Other disorders often included in GBS spectrum (controversial): Pure sensory variant, pure sensory ataxia, BBE
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Imaging
- Cauda equina (CE) nerve root enhancement in > 80%
- Isolated ventral CE in 17%, both ventral and dorsal in 65%
- Enhancement intensity of ventral CE ≥ dorsal CE
- Pial surface of distal cord/conus variable enhancement
- Pediatric GBS and MFS: CN enhancement in > 80% - CNIII, CNVI, CNVII most common; others like CNII (and optic chiasm), CNV, CNXI, CNXII
- BBE: Brainstem T2 hyperintensities, may involve cerebellar peduncles and cerebellum
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Top Differential Diagnoses
- Subacute or chronic demyelinating polyneuropathies (SIDP/CIDP)
- CSF metastatic seeding, infections, arachnoiditis
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Pathology
- 6 temporally associated pathogens: Campylobacter jejuni, Cytomegalovirus (CMV), Hepatitis E virus, Mycoplasma pneumoniae, Epstein Barr virus (EBV), and Zika virus
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Clinical Issues
- Motor weakness and sensory signs in legs progressing to upper limbs and cranial muscles
- GBS spectrum/clinical variants with various findings
- Serum anti-GQ1b antibodies in up to 90% of MFS, 70% of BBE, and 8% of classic GBS
- Anti-GM1 antibodies more characteristic of classic GBS
- Intravenous immunoglobulins and plasma exchange therapy
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Diagnostic Checklist
- Consider GBS, variants or overlap syndromes in MR with ventral > dorsal cauda equina nerve root enhancement
- Look for cranial nerve root enhancement in MFS
- Look for brainstem signal abnormalities in BBE
TERMINOLOGY
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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
-
Best diagnostic clue
- Cauda equina (CE) nerve root enhancement > 80% - Isolated ventral CE 17%, both ventral and dorsal 65% - Enhancement intensity of ventral CE ≥ dorsal CE - Pial surface of distal cord/conus variable enhancement - Pediatric GBS and MFS: Cranial nerve enhancement > 80% - CNIII, CNVI, CNVII most common; others like CNII (and optic chiasm), CNV, CNXI, CNXII - Entire spinal cord T2-hyperintense posterior column described as delayed-onset finding in MFS case - BBE: Brainstem T2 hyperintensities, may involve cerebellar peduncles and cerebellum - Enhance only rarely; extremely rare, patchy spinal cord involvement described
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-
MR Findings
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T2WI
- May see slight nerve root thickening - Brainstem hyperintensities in BBE -
T1WI C+
- Ventral > dorsal nerve root enhancement - Cranial nerve enhancement
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-
Ultrasonographic Findings
- Enlarged nerves/roots during first 3 weeks, improve later
-
Imaging Recommendations
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Best imaging tool
- MR of lumbar spine with contrast - MR of brain with contrast if MFS or BBE suspected
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DIFFERENTIAL DIAGNOSIS
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Infections
- Bacterial or granulomatous meningitis
- Nerve root enhancement in minority of enterovirus D68 acute flaccid myelitis
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CSF Metastatic Seeding
- Nodular nerve root thickening and enhancement
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Subacute or Chronic Demyelinating Polyneuropathies
- Slower onset, protracted course
- CIDP: Nadir 2 months; SIDP: 4-8 weeks
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Arachnoiditis
- Secondary to hemorrhage, surgery, chemicals, chemotherapy (like vincristine), radiation
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Vasculitic Neuropathy
- Polyarteritis nodosa, Churg-Strauss syndrome
PATHOLOGY
-
General Features
- Infection in 6 weeks prior to onset in 2/3; triggering immune response causing GBS
- 6 temporally associated pathogens: Campylobacter jejuni, Cytomegalovirus (CMV), Hepatitis E virus, Mycoplasma pneumoniae, Epstein Barr virus (EBV), and Zika virus
- Absence of preceding illness does not exclude GBS - Infections and other immunological stimuli (immunobiologicals like tumour necrosis factor antagonists, immune checkpoint inhibitors or type I interferons, surgery, amlgnancy) may be subclinical
- Serum antibodies against gangliosides in axolemma and other peripheral nerve components
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Gross Pathologic & Surgical Features
- Peripheral nerve and nerve root complement activation, macrophage infiltration, and edema
CLINICAL ISSUES
-
Presentation
-
Most common signs/symptoms
- Motor weakness and sensory signs in legs progressing to upper limbs and cranial muscles - Low back pain common symptom - GBS spectrum/clinical variants with various findings - Respiratory failure and mechanical ventilation in 20% - Autonomic nervous system involvement with cardiac arrhythmias and unstable blood pressure -
Other signs/symptoms
- Serum **anti-GQ1b** antibodies in up to 90% of MFS, 70% of BBE, and 8% of classic GBS - **Anti-GM1** antibodies more characteristic of classic GBS - Should not wait for antibody test results to start treatment when GBS is suspected - Albumino-cytological dissociation: Classic GBS finding - ↑ CSF protein and normal CSF cell count - Protein levels normal in 30-50% during 1st week and 10-30% during 2nd week - Mild CSF pleocytosis (10-50 cells/μl) can be seen in GBS, but should exclude infectious polyradiculitis - Electrodiagnostic studies not required to diagnose GBS; might be normal during 1st week, mild or variant disease, initial proximal weakness, or slow progression - GBS: Sensorimotor polyradiculoneuropathy or polyneuropathy: ↓ conduction velocities, sensory and motor evoked amplitudes, abnormal temporal dispersion &/or partial motor conduction blocks - GBS: Typical "sural sparing" pattern: Normal sural sensory nerve action potential, but abnormal or absent median and ulnar sensory nerve action potentials - MFS: Normal or only ↓ amplitude of sensory nerve action potentials
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-
Demographics
- 1-2 per 100,000 person-years; M > F; ↑ with age
-
Natural History & Prognosis
- Rapid progression, nadir within 2 weeks; mortality 3-10%
- GBS plateau phase (days to weeks or months); then recovery
- 60-80% walk independently after 6 months
- GBS typically monophasic illness, but relapses in 2-5%
- Treatment-related fluctuation (TRF): Deterioration after intial stability/improvement on therapy
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Treatment
- Intravenous immunoglobulin for 5 days (0.4 g/kg daily)
- Plasma exchange for 5 sessions (200-250 mL/kg)
- If TRF, repeat same treatment
- If no initial response or incomplete recovery, currently no evidence to support repeat treatment
DIAGNOSTIC CHECKLIST
-
Consider
- GBS, variants or overlap syndromes in MR showing ventral > dorsal cauda equina nerve root enhancement
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Image Interpretation Pearls
- Look for cranial nerve root enhancement in MFS
- Look for brainstem signal abnormalities in BBE
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References
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
Images
Selected Images
Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement 
and slight thickening.
Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.
Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.
Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal 
CE. Also note enhancement of the pial surface of the distal cord/conus 
. GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.
Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal CE. Also note enhancement of the pial surface of the distal cord/conus . GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.
Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.
Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.
Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.
Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.
Additional Images
Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).
Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).