2. ⦿Guillain-Barré syndrome is an autoimmune disorder often
considered a postinfectious polyneuropathy involving
mainly motor but also sensory and sometimes autonomic
nerves.
3. AK Asbury Criteria (1990)
⦿ Required
1. Progressive weakness of both arms and legs
2. Areflexia
3. Disease course <4weeks
4. Exclusion of other cause
(vasuculitis,toxins,botulism,diptheria
,porphyria,localized spinal cord or cauda equina
syndrome)
4. ⦿ Supportive
1. Symmetrical weakness
2. Mild sensory involvement
3. Facial & other cranial nerve involvement
4. Absence of fever
5. Typical CSF profile
6. Electrophysiological evidence of demyelination
5. ⦿ Incidence is 2 cases per 100,000/year
⦿ Nondiscriminatory : can affect person of any age,gender or
race.
⦿In western countries adults are more affected than
children.
⦿ Acute motor axonal neuropathy is documented in some
cases, mainly in China,Mexico, Bangladesh, and Japan.
6. ⦿The paralysis usually follows a nonspecific GI (C.jenuni &
H.pylori) or respiratory infection (M.pneumoniae) by
approximately 10 days.
⦿Consumption of undercooked poultry,unpasteurized milk,
and contaminated water are the main sources of
gastrointestinal infections
⦿GBS is also reported,following administration of vaccines
against rabies, influenza, and poliomyelitis(oral) and
following administration of conjugated meningococcal
vaccine, particularly serogroup C.
7. ⦿ No clear cause known
⦿Exogenous triggers are believed to activate T-cells ,which
act against variety of specific endogenous antigens like
myelin.
⦿Resemblance of triggering pathogens to antigens on
peripheral nerves leads to activation of autoimmune
response mounted by T-lymphocytes incooperation with B-
lymphocytes.
8. 1. Acute inflammatory demyelinating
polyradiculoneuropathy
2. Acute motor and sensory axonal neuropathy
3. Acute motor & senory neuropathy
4. Acute sensory neuropathy
5. Acute pandysautonomia
9. ⦿Initial symptoms include numbness and paresthesia,
followed by weakness. There may be associated neck,
back, buttock, and leg pain.
⦿ Weakness usually begins in the lower extremities and
progressively involves the trunk, the upper limbs, and
finally, the bulbar muscles,a pattern known as Landry
ascending paralysis.
⦿ Involvement of Proximal and distal muscles are often
symmetrical.
10. ⦿The onset is gradual and progresses over days or weeks,
the process plateaus in 1-28 days.
⦿Particularly in cases with an abrupt onset, tenderness on
palpation and pain in muscles are common in the initial
stages.
⦿ Bulbar involvement occurs in about half of cases.
⦿Respiratory insufficiency can result due to IC &
diaphragmatic muscle paralysis
11. ⦿Dysphagia and facial weakness are often impending signs
of respiratory failure. They interfere with eating and
increase the risk of aspiration.
⦿ Some young patients exhibit symptoms of viral meningitis
or meningoencephalitis.
⦿The autonomic nervous system is also involved in some
cases. Lability of blood pressure and cardiac rate, postural
hypotension, episodes of profound bradycardia, or
tachycardia and occasional asystole occur.
12. ⦿MFS consists of 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 do not have
significant lower extremity weakness compared with
Guillain-Barré syndrome. Distal paresthesias are noted in
MFS.
⦿Urinary incontinence or retention of urine is a
complication in approximately 20% of cases but is usually
transient.
13. ⦿ Chronic inflammatory demyelinating
polyradiculoneuropathy are that recur intermittently, or do
not improve,or progress slowly and relentlessly for periods
of months to years.
⦿ Patients are usually severely weak and can have a flaccid
tetraplegia with or without bulbar and respiratory muscle
involvement.
14. ⦿ History
⦿ Unable or refusal to walk and later to flaccid tetraplegia.
⦿Tendon reflexes in Guillain-Barré syndrome are lost,
usually early in the course.
15. ⦿The CSF protein concentration is raised in 80% cases while
mononuclear cell count is either normal
(albuminocytologic dissociation) or <50 cells/mm³
⦿ Serum creatine kinase level may be mildly elevated or
normal.
⦿Antiganglioside antibodies, mainly against GM1 and GD1,
are sometimes elevated in the serum in GBS particularly in
cases with primarily axonal rather than demyelinating
neuropathy.
16. ⦿Motor nerve conduction velocities are greatly reduced, and
sensory nerve conduction time is often slow.
⦿ Electromyography shows evidence of acute denervation of
muscle.
⦿ Sural nerve biopsy tissue shows segmental demyelination
and focal inflammation
17. Thickening of the cauda equina and
intrathecal nerve roots with gadolinium enhancement.
19. ⦿Patients with slow progression might simply be observed
for stabilization and spontaneous remission without
treatment.
⦿Rapidly progressive ascending paralysis is treated with
intravenous immunoglobulin (IVIG) 0.4 g/kg/day for 5
consecutive days.
⦿Plasmapheresis and/or immunosuppressive drugs are
alternatives if IVIG is ineffective.
20. ⦿Supportive care, such as respiratory support, prevention of
sores in children with flaccid tetraplegia, nutritional
support,pain management, prevention of deep vein
thrombosis, and treatment of secondary bacterial
infections.
⦿For CIDPs High-dose pulsed methylprednisolone given
intravenously is successful in some cases.
21. ⦿GBS is usually benign, and spontaneous recovery begins
within 2-3 wk.
⦿Most patients with the axonal form of GBS had a slow
recovery over the 1st 6 months and could eventually walk,
although some required years to recover.
⦿ Bulbar and respiratory muscle involvement can lead to
death if the syndrome is not recognized and treated.
⦿3 clinical features are predictive of poor outcome with
sequelae: cranial nerve involvement, intubation,and
maximum disability at the time of presentation.
