1. Guillain-Barré syndrome

2. Guillain-Barré syndrome
Discuss the pathogenesis and triggering organisms .
Describe the clinical features of Guillain-Barré syndrome and the main types.
Recognize the complications of the syndrome.
List investigations to differentiate it from other causes of muscle weakness.
Outline of the treatment modalities.

3. Post-infectious polyneuropathy
Involving mainly motor nerves
Sometimes also sensory and autonomic nerves.
Affects people of all ages
Most patients have a demyelinating neuropathy
Primarily axonal degeneration

4. Usually follows a nonspecific viral infection by about 10 days.
Gastrointestinal (especially Campylobacter jejuni, but also Helicobacter pylori)
Respiratory tract (especially Mycoplasma pneumoniae) symptoms.
Reported following administration of vaccines
Rabies
Influenza
Poliomyelitis (oral)
Conjugated meningococcal vaccine (serogroup C).

5. CLINICAL MANIFESTATIONS
Landry ascending paralysis
Proximal and distal muscles are involved relatively symmetrically
9% Asymmetry
Tendon reflexes are lost, usually early in the course, but are sometimes preserved until later.
This variability can cause confusion.

6. Cases with an abrupt onset
The onset: gradual
progresses over days or weeks.
Cases with an abrupt onset
tenderness on palpation and pain in muscles is common in the initial stages.
Weakness can progress →inability or refusal to walk → flaccid tetraplegia.
Paresthesias: some cases.

7. Bulbar involvement occurs in about half of cases.
Respiratory insufficiency can result.
Dysphagia and facial weakness are often impending signs of respiratory failure.
Extraocular muscle involvement is rare

8. Miller-Fisher syndrome
Acute external ophthalmoplegia
Ataxia
Areflexia.
Overlaps with Bickerstaff brainstem encephalitis
Shares many features with Guillain-Barré syndrome with lower motor neuron involvement

9. Urinary incontinence or retention of urine: 20% of cases
Usually transient

10. The autonomic nervous system is also involved in some cases.
Labiality of blood pressure and cardiac rate
Postural hypotension
Episodes of profound bradycardia
Occasional asystole
Cardiovascular monitoring is important.

11. Classification of Guillain-Barre syndrome and related disorders

12. DIFFERENTIAL DIAGNOSIS
SPINAL CORD LESIONS
PERIPHERAL NEUROPATHIES
Acute transverse myelitis
Epidural abscess
Tumors
Poliomyelitis
Hopkins syndrome
Vascular malformations
Cord infarction
Fibrocartilaginous embolism
Cord compression from vertebral subluxation related to congenital abnormalities or trauma
Acute disseminated encephalomyelitis
Toxic
Vincristine
Glue sniffing
Heavy metal
Organophosphate pesticides
Infections
HIV
Diphtheria
Lyme disease

13. DIFFERENTIAL DIAGNOSIS
PERIPHERAL NEUROPATHIES
NEUROMUSCULAR JUNCTION DISORDERS
Inborn errors of metabolism
Leigh disease
Tangier disease
Porphyria
Critical illness: polyneuropathy/myopathy
Tick paralysis
Myasthenia gravis
Botulism
Hypercalcemia
Myopathies
Periodic paralyses,
Dermatomyositis
Critical illness myopathy/polyneuropathy

14. Work up Serum creatine kinase (CK)
may be mildly elevated or normal
CSF studies are essential for diagnosis.
protein is elevated to more than twice the upper limit of normal
Glucose level is normal
No pleocytosis.
Fewer than 10 white blood cells/mm3 are found.
Bacterial cultures are negative

15. Results of stool cultures are rarely positive
Serologic testing for Campylobacter and Helicobacter infections helps establish the cause
does not alter the course of treatment.
Results of stool cultures are rarely positive
The infection is self-limited (occurs for about 3 days), and the neuropathy follows the acute gastroenteritis.

16. MRI Of the spinal cord may be indicated to rule out disorders.
MRI findings include thickening of the cauda equina and intrathecal nerve roots with gadolinium enhancement.
These finds are fairly sensitive and are present in >90% of patients

18. NCS Motor NCVs are greatly reduced
Sensory nerve conduction time is often slow.
Electromyography (EMG) shows evidence of acute de-nervation of muscle.

19. Antiganglioside antibodies
Mainly against GM1 and GD1
Sometimes elevated in the serum in Guillain-Barré syndrome, particularly in cases with primarily axonal rather than demyelinating neuropathy

20. biopsies
Muscle and nerve is not usually required for the diagnosis

21. TREATMENT In early stages of this acute disease
Should be admitted to the hospital for observation because the ascending paralysis can rapidly involve respiratory muscles during the next 24 hr
Respiratory effort (negative inspiratory force, spirometry) must be monitored to prevent respiratory failure and respiratory arrest.

22. Rapidly progressive ascending paralysis
slow progression
observation for stabilization
spontaneous remission without treatment possible
Rapidly progressive ascending paralysis
Intravenous immunoglobulin (IVIG), administered for 2, 3, or 5 days.
A commonly recommended protocol is IVIG 0.4 g/kg/day for 5 consecutive days.
Plasmapheresis and/or immunosuppressive drugs
if IVIG is ineffective.
Steroids are not effective.

23. Supportive care Respiratory support
Prevention of decubiti in children with flaccid tetraplegia
Treatment of secondary bacterial infections

24. PROGNOSIS
Usually benign, and spontaneous recovery begins within 2-3 wk.
Most patients regain full muscular strength
some are left with residual weakness.
The tendon reflexes are usually the last function to recover.
Improvement usually follows a gradient opposite the direction of involvement:
bulbar function recovering first
lower extremity weakness resolving last.
Bulbar and respiratory muscle involvement can lead to death if the syndrome is not recognized and treated.

25. 3 clinical features are predictive of poor outcome with sequelae:
Cranial nerve involvement
Intubation
Maximum disability at the time of presentation.
The electrophysiologic features of conduction block are predictive of good outcome.

26. Easy fatigue is one of the most common chronic symptoms
Among patients with the axonal form of Guillain-Barré syndrome, most who had slow recovery over the first 6 mo could eventually walk, although some required years to recover.
EMG and NCV electrophysiologic studies do not necessarily predict the long-term outcome.

27. Chronic inflammatory demyelinating polyradiculoneuropathies
(CIDP, sometimes called chronic inflammatory relapsing polyneuritis or chronic unremitting polyradiculoneuropathy)
Chronic varieties of Guillain-Barré syndrome
Recur intermittently
Do not improve
Progress slowly and relentlessly for periods of months to years.
About 7% of children with Guillain-Barré syndrome suffer an acute relapse.