Guillain-Barré syndrome is an autoimmune disorder that causes inflammation of the nerves. It is characterized by progressive weakness in the legs and arms, loss of reflexes, and ascending paralysis over 4 weeks or less. The disorder is usually preceded by a gastrointestinal or respiratory infection. Treatment involves supportive care and immunoglobulin therapy. Most patients recover, though some experience long-term weakness depending on the severity of symptoms.

1. DR.RAVIKUMAR S
1ST YEAR PG
DEPT OF PEDIATRICS
MGMCRI

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

18. Thickening of the cauda equina and
intrathecal nerve roots with gadolinium enhancement.

19. SPINAL CORD LESIONS
 Acute transverse myelitis
 Epidural abscess
 Tumors
 Poliomyelitis
TOXINS
 Organophosphate
pesticides
INFECTIONS
 Diphtheria
 Lyme disease
NEUROMUSCULAR JUNCTION
DISORDERS
 Tick paralysis
 Myasthenia gravis
 Botulism

20.  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.

21.  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.

22.  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.