Guillain Barre Syndrome and Myasthenia Gravis.ppt

Guillain barre syndrome.

1. Guillain-Barré Syndrome
PT ABDULLA

2. Guillain-Barré Syndrome
•Is an acute inflammatory demyelinating
polyneuropathy (AIDP), a disorder affecting
the peripheral nervous system. It is usually
triggered by an acute infectious process. The
syndrome was named after the French
physicians Guillain, Barré and Strohl, who
were the first to describe it in 1916. It is
sometimes called Landry's paralysis, after the
French physician who first described a variant
of it in 1859.

3. ETIOLOGY
•The etiology of Guillain-Barré syndrome is
unclear, but an autoimmune response is
strongly suspected.
•Occurs as an autoimmune response following
a Gastrointestinal or respiratory infection
•Occasionally, vaccinations have been known to
trigger Guillain-Barré syndrome.

4. •Two thirds of people with Guillain-Barré
syndrome have experienced an infection
before the onset of the condition.
•Most commonly these are episodes of
gastroenteritis or a respiratory tract infection
•Approximately 25% of patients with this
disease have antibodies to either
cytomegalovirus or Epstein-Barr virus.

5. PATHOPHYSIOLOGY
•In Guillain-Barré syndrome, the myelin sheath
surrounding the axon is lost.
•Loss of the myelin sheath in Guillain-Barré
syndrome makes nerve impulse transmission
is aborted.

7. Risk factors of GBS
•Age 15-35 and 60-75 years
•Men are at risk than women
•Recent GI or respiratory infection by virus or
bacteria
•Recent surgery and trauma
•Hx of SLE HIV/AIDS

8. Clinical features of GBS
•Progressive symmetric weakness beginning in
the legs and advancing upward
•Reduced/absent of DTR
•Back pain
•Peripheral loss of sensation

9. CLINICAL MANIFESTATIONS
•The syndrome may develop rapidly over the
course of hours or days, or may take up to 3 to
4 weeks to develop.
•Most patients demonstrate the greatest
weakness in the first weeks of the disorder.
•In the beginning, a flaccid, ascending paralysis
develops quickly.
•The patient is most commonly affected in a
symmetrical pattern.

10. •The patient may first notice weakness in the
lower extremities that may quickly extend to
include weakness and abnormal sensations in
the arms.
•Deep tendon reflexes are usually lost, even in
the earliest stages.
•The trunk and cranial nerves may become
involved.
•Respiratory muscles can become affected,
resulting in respiratory compromise.

11. CLINICAL MANIFESTATIONS
•Autonomic disturbances such as urinary
retention and orthostatic hypotension may
also occur
•Some patients experience tenderness and
pain on deep pressure or movement of some
muscles.

12. CLINICAL MANIFESTATIONS
•If there is cranial nerve involvement, cranial
nerve VII, the facial nerve, is most often
affected.
•Guillain-Barré syndrome does not affect level
of consciousness, pupillary function, or
cerebral function.

13. •Symptoms may progress for several weeks.
The level of paralysis may stop at any point.
•Motor function returns in a descending
fashion.
•Demyelination occurs rapidly, but the rate of
remyelination is approximately 1 to 2 mm per
day.

14. CLASSIFICATION OF GBS
•Acute inflammatory demyelinating
polyneuropathy (AIDP)
•Acute motor axonal neuropathy (AMAN)
•Acute motor sensory axonal neuropathy
(AMSAN)
•Miller-Fisher syndrome

15. Diagnostic Criteria for Guillain-
Barré Syndrome
•Progressive weakness of 2 or more limbs due
to neuropathy–usually ascending.
•Areflexia
•Disease course <4 weeks

16. Clinical differentiation
Symptoms Polio GBS Transverse
Myelitis
Flaccidity Asymmetric
proximal
Symmetric
Distal
Lower limbs, sym.
Progress of
paralysis
< 4 days Hours to 20
days
Hours to 4 days
Sensation intact Paresthesia
toe, finger
tips
Sensory level
Respiratory
Problems.
Bulbar/
Bulbo spinal
Common in
young
absent
Bladder
dysfunction
transient sometimes present

17. DIAGNOSIS
•The history of the onset of symptoms can be
revealing because symptoms of Guillain-Barré
syndrome usually begin with weakness or
paresthesias of the lower extremities and
ascend in a symmetrical pattern.
•A lumbar puncture may be performed and
reveal increased protein.

18. •Nerve conduction studies record impulse
transmission along the nerve fiber.
•Pulmonary function tests are done when
Guillain-Barrésyndrome is suspected to
establish a baseline for comparison as the
disease progresses.
•Stool cultures for campylobacter

19. COMPLICATION OF GBS
•Respiratory muscle paralysis
•Permanent muscle weakness
•Permanent numbness
•Total paralysis
•Recurrent attacks of GBS

20. Management of GBS
•There is no cure for GBS however many
treatment are available to reduce the
symptoms, treat complications and speed
recovery.

21. Supportive management(ICU
monitoring)
Ventilatorysupport; atelctasisinitially treated
by;
•Incentive spiratory
•Frequent sunctioning
•Chest physiotherapy, to mobilise secretion
•Intubation to prevent aspiration
•Tracheostomy 4 intubated patients

22. CLINICAL MANAGEMENT
•Preventive measures need to be established
to prevent DVT and pulmonary embolism do
not develop.
•Heparin 5000 units subcutaneously may be
given along with antiembolism stockings and
sequential compression devices

23. •The first therapy proven to benefit patients
with Guillain-Barré syndrome is
plasmapheresis.
•Often done to remove extra Ab, abnormal
proteins or other harmful substances from the
blood
•Intravenous immunoglobulin (IVIG) is also
useful in managing Guillain-Barré syndrome.

24. Rehabilitation management
•To prevent lung infection
•To maintain chest mobility
•To prevent DVT
•To prevent contractures
•To prevent bedsores
•To maintain circulation
•To reduce pain

25. Prognosis of GBS
•Approximately 85% of all patients with GBS
achieve a fully functional recovery within
months to years.
•Death in up to 25% of those who require ICU
has been reported often from autonomic
abnormalities leading to pulmonary
complications.

26. Prognosis cont…………………..
•Approx 16% patients suffer permanent
disability
•Those who require ventilation, improve after
more than 3 weeks, not improved within 1
month have greater risk of poorer outcome
•Gradual improvement may occur over 18
months -2years
•Recurrence n 2-5% cases, leading to chronic
inflammatory demyelinating polyneuropathy

27. PREVENTION OF GBS
•No guideline for prevention of GBS.
•GIT of domestic and wild birds and animals is
the reservoir of campylobacter jejuni.
•Avoid campylobacter food poisoning
-hand hygiene in handling raw poultry.
-hand hygiene in handling cats, dogs.
-symptomatic people should be excluded in
handling food.
-care in children care centers.