GBS is an autoimmune disorder that is thought to be a postinfectious polyneuropathy, involving mainly motor but also sensory and sometimes autonomic nerves.
This syndrome affects people of all ages and is not hereditary.
Most patients in the U.S and Europe have a demyelinating neuropathy, but primarily axonal degeneration is apparent in some forms of GBS, seen mainly in China, Mexico, Bangladesh, and Japan.
Clinical Manifestations
The onset of weakness usually follows a nonspecific GI or respiratory infection by ~ 10 days.
The original infection might have caused only GI (especially C. jejuni, but also H.pylori ), respiratory tract (especially M.pneumoniae ), or systemic (Zika virus) symptoms.
Consumption of undercooked poultry , unpasteurized milk, and contaminated water are the main sources of GI infections.
West Nile virus also can mimic GBS, but more often causes a motor neuron disease similar to poliomyelitis.
GBS may follow administration of vaccines against rabies , influenza, and conjugated meningococcal vaccine, particularly serogroup C.
Other infectious precursors of GBS include mononucleosis, Lyme disease, CMV, and the Zika virus
Initial symptoms include numbness and paresthesia, followed by weakness.
Radicular back pain and myalgia are common in the initial stages ; affected children can be very irritable.
Weakness usually begins in the lower extremities and progressively involves the trunk, the upper limbs, and finally the bulbar muscles, but weakness is sometimes proximally prominent.
Extraocular muscle involvement is rare, but many patients develop facial weakness.
In most patients, weakness is essentially symmetric.
Weakness progresses over days or weeks, the clinical nadir occurring in < 4 wk.
~ 60% of children lose the ability to walk at some point in their illness; a small proportion progress to flaccid tetraplegia.
The maximal severity of weakness is reached by 4 wk after onset.
GBS and MFS and their subtypes form a continuum of discrete and overlapping syndromes.
The pattern of weakness for each subtype is as follows:
Classic GBS, tetraparesis with or without motor cranial nerve involvement;
Paraparetic GBS, lower limbs;
pharyngeal-cervical-brachial weakness, bulbar, neck, and upper limbs; bifacial weakness with paresthesias,
Facial; MFS, external ophthalmoplegia
Bickerstaff brainstem encephalitis, external ophthalmoplegia.
Facial weakness and motor cranial nerve involvement are more frequent in demyelinating-type classic GBS (AIDP) than in axonal-type GBS (acute motor axonal neuropathy).
In MFS , there is ataxia, and in its CNS subtype, Bickerstaff brainstem encephalitis, there is additional hypersomnolence
DDX
SPINAL CORD LESIONS
Acute transverse myelitis
Epidural abscess
Tumors
Poliomyelitis
Enteroviruses
Acute flaccid myelitis
Hopkins syndrome
Vascular malformations
2. • GBS is an autoimmune disorder that is thought to be a
postinfectious polyneuropathy, involving mainly motor
but also sensory and sometimes autonomic nerves.
• This syndrome affects people of all ages and is not
hereditary.
• Most patients in the U.S and Europe have a
demyelinating neuropathy, but primarily axonal
degeneration is apparent in some forms of GBS, seen
mainly in China, Mexico, Bangladesh, and Japan.
3. Clinical Manifestations
• The onset of weakness usually follows a nonspecific GI
or
respiratory infection by ~ 10 days.
• The original infection might have caused only GI
(especially C. jejuni, but also H.pylori ), respiratory tract
(especially M.pneumoniae ), or systemic (Zika virus)
symptoms.
• Consumption of undercooked poultry , unpasteurized milk,
and contaminated water are the main sources of GI
infections.
4. • West Nile virus also can mimic GBS, but more often causes a
motor neuron disease similar to poliomyelitis.
• GBS may follow administration of vaccines against rabies ,
influenza, and conjugated meningococcal vaccine, particularly
serogroup C.
• Other infectious precursors of GBS include mononucleosis,
Lyme disease, CMV, and the Zika virus
5. • Initial symptoms include numbness and paresthesia, followed
by weakness.
• Radicular back pain and myalgia are common in the initial
stages ; affected children can be very irritable.
• Weakness usually begins in the lower extremities and
progressively involves the trunk, the upper limbs, and finally
the bulbar muscles, but weakness is sometimes proximally
prominent.
• Extraocular muscle involvement is rare, but many patients
develop facial weakness.
• In most patients, weakness is essentially symmetric.
• Weakness progresses over days or weeks, the clinical nadir
occurring in < 4 wk.
6. • ~ 60% of children lose the ability to walk at some point
in their illness; a small proportion progress to flaccid
tetraplegia.
• The maximal severity of weakness is reached by 4 wk
after onset.
7.
8. • GBS and MFS and their subtypes form a continuum of discrete
and overlapping syndromes.
• The pattern of weakness for each subtype is as follows:
• Classic GBS, tetraparesis with or without motor cranial nerve
involvement;
• Paraparetic GBS, lower limbs;
• pharyngeal-cervical-brachial weakness, bulbar, neck, and upper
limbs; bifacial weakness with paresthesias,
9. • Facial; MFS, external ophthalmoplegia
• Bickerstaff brainstem encephalitis, external ophthalmoplegia.
• Facial weakness and motor cranial nerve involvement are more
frequent in demyelinating-type classic GBS (AIDP) than in
axonal-type GBS (acute motor axonal neuropathy).
• In MFS , there is ataxia, and in its CNS subtype, Bickerstaff
brainstem encephalitis, there is additional hypersomnolence
13. • Bulbar involvement occurs in about 50% of cases and can result in
respiratory insufficiency.
• Dysphagia and facial weakness can be signs of impending respiratory failure,
interfere with saliva control and swallowing, and increase the risk of aspiration.
• Vocal cord paralysis may cause dyspnea or a hoarse voice.
• Severe bulbar and respiratory muscle involvement can lead to death if GBS is not
recognized and treated.
• The autonomic nervous system is also involved in some cases.
• Lability of BP and HR, postural hypotension, episodes of profound
bradycardia or tachycardia, and occasional asystole occur, more commonly in
younger patients or those with severe weakness.
• Cardiovascular monitoring is important, especially early in the disease course,
when rapid progression of weakness, respiratory insufficiency, and autonomic
instability can be life threatening.
• The tendon reflexes are lost in GBS, usually early in the course, but are sometimes
preserved until later; areflexia is more common but hyporeflexia may be seen.
• Of affected children, 10% retain their reflexes throughout.
• This variability can cause diagnostic confusion.
14. • Subtypes of GBS include an acute inflammatory demyelinating
polyneuropathy and an acute motor axonal neuropathy; these are
distinguished by findings on nerve conduction studies and an associated
pattern of antiganglioside antibodies.
• Localized forms of GBS also occur and include a pattern of facial diplegia
with paresthesias and a pattern of pharyngeal-cervical-brachial weakness.
• MFS is an uncommon GBS variant associated with acute external (and
occasionally internal) ophthalmoplegia, ataxia, and areflexia.
• The 6th cranial nerve is most often involved in MFS.
• Although areflexia is seen in MFS, patients have no or only very mild lower
extremity weakness, compared with GBS.
• Distal paresthesias are common in MFS.
• Urinary incontinence or retention is a complication in approximately 20% of
cases but is usually transient.
• MFS overlaps clinically with Bickerstaff brainstem encephalitis
15.
16. • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP,
sometimes called chronic inflammatory relapsing polyneuritis ) is a more
chronic, slowly progressive, acquired inflammatory neuropathy with some
clinical overlap with GBS.
• Symptoms such as weakness and paresthesias develop over more than 4-6
wk, recur intermittently (relapsing), or progress slowly over periods of months
to years.
• Weakness is generally both proximal and distal, and variably severe.
Hyporeflexia or areflexia is almost universal.
• Motor deficits occur in 94% of cases and sensory paresthesias in 64%, but
cranial nerve and autonomic involvement is uncommon.
• The cerebrospinal fluid (CSF) shows no pleocytosis, but the CSF protein is
almost always elevated.
• Nerve conduction studies show variable slowing of nerve conduction; where
required, sural nerve biopsy shows patchy myelin loss and focal
inflammatory changes.
17. • Acute-onset CIDP may be difficult to distinguish from GBS;
CIDP may also be difficult to distinguish from GBS with
treatment-related symptom fluctuations.
• Congenital GBS is very rare, manifesting as generalized
hypotonia , weakness, and areflexia in an affected neonate,
fulfilling all electrophysiologic and CSF criteria and in the
absence of maternal neuromuscular disease.
• Treatment is not always required.
18. Laboratory Findings and Diagnosis
• CSF studies are helpful in diagnosing GBS.
• The CSF protein is usually elevated to more than twice the upper
limit of normal, the glucose level is normal, and there is no
pleocytosis; there should be fewer than 10 white blood cells/mm3
• Bacterial cultures are negative, whereas viral studies rarely isolate
specific viruses.
• The dissociation between high CSF protein and a lack of cellular
response (cytoalbuminologic dissociation) in a patient with an acute
or subacute polyneuropathy is essentially diagnostic of GBS.
• These findings may not be apparent in the first week after the onset
of symptoms
19. Diagnostic Criteria for Guillain-Barré Syndrome*
FEATURES NEEDED FOR DIAGNOSIS OF GBS IN CLINICAL
PRACTICE
• Progressive weakness in legs and arms (sometimes initially only in
legs).
• Areflexia (or decreased tendon reflexes) in weak limbs.
• ADDITIONAL SYMPTOMS
• Progressive phase lasts days to 4 wk (often 2 wk).
• Relative symmetry.
• Mild sensory symptoms or signs (not present in acute motor axonal
neuropathy).
• Cranial nerve involvement, especially bilateral weakness of facial
muscles.
• Autonomic dysfunction.
• Pain (common)
20. FEATURES THAT SHOULD RAISE DOUBT ABOUT THE
DIAGNOSIS OF GUILLAIN-BARRÉ SYNDROME
• CSF: increased number of mononuclear cells or PMN cells (>50
cells/µL).
• Severe pulmonary dysfunction with little or no limb weakness at
onset.
• Severe sensory signs with little or no weakness at onset.
• Bladder or bowel dysfunction at onset.
• Fever at onset.
• Sharp spinal cord sensory level.
• Marked, persistent asymmetry of weakness.
• Persistent bladder or bowel dysfunction
• Slow progression of weakness and without respiratory involvement
(consider subacute inflammatory demyelinating polyneuropathy or
acute-onset chronic inflammatory demyelinating polyneuropathy).
21. NERVE CONDUCTION STUDIES
• Can be helpful in clinical practice but are generally not required to
diagnose Guillain-Barré syndrome.
• Essential for classification of Guillain-Barré syndrome as acute
inflammatory demyelinating polyneuropathy or acute motor axonal
neuropathy.
• Acute inflammatory demyelinating polyneuropathy: features of
demyelination (decreased motor nerve conduction velocity,
prolonged distal motor latency, increased F-wave latency, conduction
blocks, and temporal dispersion).
• Acute motor axonal neuropathy: no features of demyelination (one
demyelinating feature in one nerve, if distal
• CMAP amplitude is less than 10% LLN, can be found; distal CMAP
amplitude less than 80% LLN in at least two nerves.
• Transient motor nerve conduction block might be present
22. • MRI of the spinal cord in GBS, typical findings include
thickening of the cauda equina and intrathecal nerve roots with
gadolinium enhancement
• Atypical findings should prompt consideration of the alternative
diagnoses.
• Imaging in CIDP is similar but demonstrates greater
enhancement of spinal nerve roots
23. • Nerve conduction studies and electromyography are sensitive to
early signs of peripheral nerve inflammation in GBS.
• Motor and sensory nerve conduction velocities are reduced to a
variable extent, reflecting the patchy nature of nerve involvement in
this disorder, which is also reflected in the presence of focal
conduction block and dispersed responses.
• Electromyography may show acute denervation of muscle.
• Serum creatine kinase levels may be mildly elevated or normal.
• Serum antiganglioside antibodies against GM1 and GD1 are
sometimes elevated in GBS, particularly in cases with primarily
axonal rather than demyelinating neuropathy, suggesting that they
might play a role in disease propagation and/or recovery in some
cases.
24. • Sural nerve biopsy shows segmental demyelination, focal
inflammation, and Wallerian degeneration, but is almost never
required for diagnosis
• Serologic testing for Campylobacter and Helicobacter infections
helps establish causation if results are positive but does not
alter treatment.
• Stool cultures are rarely positive because the infection is self-
limited and only occurs for about 3 days, and the neuropathy
follows the acute gastroenteritis
25. Treatment
• Patients in early stages of this acute disease should be admitted to the hospital
for observation because the ascending paralysis can rapidly involve respiratory
muscles and cause respiratory failure and autonomic instability.
• The respiratory effort (measured by bedside testing or spirometry) must be
monitored for changes predicting an onset of hypoventilation and respiratory
failure.
• Patients with milder weakness and slow progression may be treated
expectantly, with observation for stabilization and spontaneous remission.
• Severe or rapidly progressive muscle weakness is treated with intravenous
immunoglobulin (IVIG); common protocols include IVIG 0.4 g/kg/day for 5
consecutive days or 1g/kg/day for 2 days.
• Plasmapheresis and/or immunosuppressive drugs are alternatives if IVIG is
ineffective.
• Steroids are not effective for weakness but may help with pain.
• Supportive care, such as respiratory support, prevention of pressure sores,
nutritional support, pain management, prevention of deep vein thrombosis, and
treatment of secondary bacterial infections is important.
26.
27. • Neuropathic pain in GBS should be treated aggressively, with
narcotic analgesics where necessary, and with medications
such as gabapentin.
• CIDP can be treated with either oral or pulsed steroids or IVIG,
with refractory cases often requiring use of other
immunosuppressive medications.
• Children with relapsing or slowly progressive weakness often
need months to years of therapy, but most eventually achieve a
sustained remission.
• The outcome is generally good, but some children have
permanent deficits
28. Prognosis
• GBS is usually a monophasic illness; spontaneous recovery begins within 2-3
wk but can take months.
• Therapy with IVIG hastens recovery but not does alter the long-term outcome.
• As many as 60% become nonambulant during their illness, but most eventually
regain full strength.
• A minority has some residual weakness, most often of the ankle dorsiflexors.
• Clinical features predicting a severe course and slow (possibly incomplete)
recovery include cranial nerve involvement, the need for ventilatory support, and
maximum disability at the time of presentation.
• Neurophysiologic studies do not necessarily predict the long-term outcome, but
children with demyelinating forms of GBS generally recover more quickly than
those with axonal forms.
• The tendon reflexes are usually the last function to recover.
• Improvement usually follows a gradient opposite the direction of involvement, with
bulbar function recovering first and lower extremity weakness resolving last.
29. • Bulbar and respiratory muscle involvement can lead to death if
the syndrome is not recognized and treated.
• Fatigue is the most common long-term residuum of GBS.
• Relapses occur in about 4% of children with GBS and are
generally responsive to immunomodulatory treatment.
