gbs

1. Guillain-Barré syndrome
Dr.Manoj Aryal
MBBS,MD(KU)
Physician/Internist
Department of Internal medicine
LMCTH Palpa

2. INTRODUCTION
• An acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in
nature.
• Rapid progressive ascending paralysis.
• An acute monophasic paralyzing illness, usually provoked by a preceding infection.
• Heterogeneous condition with several variant forms.

3. PATHOGENESIS

4. EPIDEMIOLOGY
• Occurs world-wide with an overall incidence of 1 to 2 cases per 100,000 per year.
• All age groups are affected but is slightly greater in males than in females.
• In Western countries, adults are more frequently affected than children.

5. ANTECEDENT EVENTS
• Approximately 70% of cases occurs after1–3 weeks of an acute infectious process,
usually respiratory or gastrointestinal.
• 20–30% of all cases are preceded by infection or reinfection with Campylobacter
jejuni.
• Other human herpes virus infection, often CMV or Epstein-Barr virus. Other
viruses (e.g., HIV, hepatitis E, Zika) and also Mycoplasma pneumonia.
• C. jejuni has also been implicated in summer outbreaks of AMAN among children
and young adults exposed to chickens in rural China.
• Other triggering events, develop after immunization, surgery, trauma, and bone-
marrow transplantation, linked to systemic processes, including Hodgkin
lymphoma, systemic lupus erythematosus, and sarcoidosis

6. CLINICAL FEATURES
• Rapidly evolving areflexic motor paralysis, with or without sensory disturbance.
• An ascending paralysis that may be first noticed as rubbery legs.
• Absent or depressed deep tendon reflexes.
• The legs are usually more affected than the arms, and facial diparesis is present in
50% of affected individuals.
• Cutaneous sensory deficits (e.g., loss of pain and temperature sensation) are
usually relatively mild.
• Autonomic involvement is common.
• Pain is another common symptoms.

7. CLINICAL FEATURES
• No cranial nerve, bladder and bowel involvement
• The syndrome of inappropriate antidiuretic hormone secretion (SIADH), which may be due to
autonomic involvement, is another complication of GBS.
• Unusual features of GBS include papilledema, facial myokymia, hearing loss, meningeal
signs, vocal cord paralysis, and mental status changes, reversible posterior
leukoencephalopathy syndrome has been associated with GBS.
• Once clinical worsening stops and the patient reaches a plateau (almost always within 4
weeks of onset), further progression is unlikely.

9. GBS VARIANTS

10. Acute inflammatory demyelinating
polyneuropathy
• most common form in the United States and Europe.
• Peripheral nerve myelin is the target of immune attack in AIDP.
• Studies from the U.S and Europe, reflecting that in AIDP, shows the following clinical
features:
• The weakness usually starts in the legs, but begins in the arms or facial muscles in
about 10%.
• Severe respiratory muscle weakness need of ventilatory support develops in 10 to
30 %.
• Facial nerve palsies occur in more than 50 %, and oropharyngeal weakness
eventually occurs in 50 %.

11. Acute inflammatory demyelinating
polyneuropathy
• Oculomotor weakness occurs in about 15 %.
• Decreased or absent reflexes in affected arms or legs are about 90 % of presentation and
with disease progression.
• Paresthesia's in the hands and feet accompany the weakness in more than 80 %, but
sensory abnormalities on examination are frequently mild.
• Pain due to nerve root inflammation, typically located in the back and extremities, can be
a presenting feature.
• Dysautonomia occurs in 70 %.

12. Acute motor axonal neuropathy
• Known as AMAN, was first recognized in 1986.
• Preceded by Campylobacter jejuni infection, so has a seasonal incidence, being more frequent
in the summer.
• Occurs frequently in Asia, particularly in young people.
• Deep tendon reflexes are occasionally preserved & Sensory nerves are not affected.
• The presenting clinical features and recovery of AMAN are otherwise similar to those of
AIDP.

13. Acute motor and sensory axonal neuropathy
• More severe form of AMAN, in which both sensory and motor fibers are affected with
marked axonal degeneration, causing delayed and incomplete recovery.
• Clinically, AMSAN resembles the AMAN variant but has more sensory symptoms.
• The pathology is predominantly axonal lesions of both motor and sensory nerve fibers.

14. Miller Fisher syndrome
• The typical presentation is that of ophthalmoplegia with ataxia and areflexia.
• Incomplete forms include acute ophthalmoplegia without ataxia, and acute ataxic neuropathy
without ophthalmoplegia.
• The MFS variant accounts for ~5% of all cases and is strongly associated with antibodies to
the ganglioside GQ1b

15. Other variants
• Bickerstaff encephalitis
• Pharyngeal-cervical-brachial weakness
• Paraparesis
• Acute pan dysautonomia
• Pure sensory GBS.

18. Laboratory Features
• Cerebrospinal fluid analysis:
• an elevated CSF protein with a normal CSF white blood cell count.
• known as albumin cytologic dissociation
• Present in first week after the onset of symptoms

19. Laboratory Features
• Electrodiagnostic studies:
• Nerve conduction studies (NCS) and needle electromyography (EMG) are valuable for
confirming & for providing information regarding prognosis. Also useful in classifying the
main variants of GBS as demyelinating (eg, AIDP ) or axonal (eg, AMAN )
• Demyelinating forms: are supported by features of demyelination, including decreased motor
nerve conduction velocity, prolonged distal motor latency, increased F wave latency, conduction
blocks, and temporal dispersion.
• Axonal forms: are supported by decreased distal motor and/or sensory amplitudes. Transient
motor nerve conduction block (ie, reversible conduction failure) can be present

20. Laboratory Features
• Antibodies:
• testing for serum IgG antibodies to GQ1b is useful for the diagnosis of Miller Fisher
syndrome
• Antibodies to GQ1b may also be present in GBS with ophthalmoparesis, Bickerstaff
encephalitis, and the pharyngeal-cervical brachial but not in disorders other than GBS
• MRI:
• Spinal MRI may reveal thickening and enhancement of the intrathecal spinal nerve roots
and cauda equine and also enhancement of the oculomotor, abducens, and facial nerves
may be seen.

22. DIFFERENTIAL DIAGNOSIS

23. TREATMENT
• In the vast majority of patients with GBS, treatment should be initiated as soon after
diagnosis as possible
• Each day counts; -2 weeks after the first motor symptoms, it is not known whether
immunotherapy is still effective.
• If the plateau stage is reached , then treatment probably is no longer indicated, unless
there is severe motor weakness and one cannot exclude the possibility that an
immunologic attack is still ongoing
• High-dose intravenous immune globulin (IVIg) or plasmapheresis can be initiated
• IVIg is administered as five daily infusions for a total dose of 2 g/kg body weight
• A course of plasmapheresis usually consists of ~40–50 mL/kg PE 4–5 times over 7–10
days.
• Glucocorticoids have not been found to be effective in GBS.

24. Prognosis and Recovery
• Approximately 85% achieve a full functional recovery within several months to
a year, although minor findings (such as areflexia) may persist & often complain
of continued symptoms, including fatigue.
• The mortality rate is <5% in optimal settings; death usually results from
secondary pulmonary complications. The outlook is worst in patients with
severe proximal motor and sensory axonal damage.
• Such axonal damage may be either primary or secondary in nature.
• Between 5 and 10% of patients with typical GBS have one or more late
relapses; many of these cases are then classified as chronic inflammatory
demyelinating polyneuropathy (CIDP).

25. REFERENCES
• Davidsons-Principles-and-Practice-of-Medicine-23rd-Edition
• Harrisons Principle of Internal Medicine 21th edition.