detail of myasthenia gravis and GBS

3.  Formation of ach: acetate present in mitochondria
of nerves and choline received from diet from GI
system. so both combine and form ach which is
present in vesicles
 Wave of depolarization starts and it cause na influx
 Then calcium influx also starts with depolarization
wave.
 Calcium combine with ach so calcium fused with
ach in nerve ending membrane.
 Then calcium mediated ach vesicle fused with
neurolema( nerve ending membrane) It produce
small hole in that membrane.

4.  In post synaptic membrane special type of
receptors known as cholinergic receptors.
 Ach binds to these receptors. These receptors
also stimulated by nicotiene.
 As ach helps in muscle contraction. Once ach
completed their work it is destroyed as it can
not stay forever. To destroy ach a enzyme
known as ach estrase works.

5. DEFINITION:-
 Myasthenia (Weakness) gravis (Serious) is a
chronic, rare autoimmune neuromuscular
disease, caused by an antibody-mediated
blockage of neuromuscular transmission
resulting in skeletal muscle weakness
&fatigability.
 It is an autoimmune disorder, in which weakness
is caused by circulating antibodies that block
acetylcholine receptors at the postsynaptic
neuromuscular junction, inhibiting the excitatory
effects of the neurotransmitter acetylcholine on

6. INCIDENCE –
 Annual incidence in US- 2/10,00000
 Worldwide prevalence 1/10,000
 Mortality/morbidity
 Recent decrease in mortality rate due to
advances in treatment

7. ETIOLOGY –
 Idiopathic
 The cause of MG is unknown, but 85% of people with the generalized
form of the disease have elevated auto antibodies to the nicotine Ach
receptor in the serum but 15% of people have elevated antibodies
against musk(muscle specific kinasis) protein Ach receptor in the
serum.
 Congential (genetic defect in any part of synaptic membrane)
 Factors that can worsen myasthenia gravis
 Thymus defects ( thymoma, thymic hyperplasia)
 Illness
 Stress
 Extreme heat
 Some medications are lowers calcium production and ach such
as beta blockers, quinidine phenytoin (Dilantin), certain
anesthetics and some aminoglycoside(genta) so avoid in MG
patient

8. CONT……
 MG is more common in families with other
autoimmune diseases. A familial predisposition
found in 5% of the cases.
 Myasthenia gravis is associated with various
autoimmune diseases, including:
 Thyroid diseases
 Diabetes mellitus type 1
 Rheumatoid arthritis

9.  Side effects of some Drugs
 Aminoglycosides: gentamycin,amikamycin
 Quinine
 Beta blockers
 It block calcium influx so it inhibit release of
ach

10. PATHOPHYSIOLOGY

12. Normal neuromuscular
transmission
When stimulus travels down a motor nerve (By influx of
na & ca channel)
Ach released from motor nerve terminal at presynaptic
memrane
Ach combines with AchRs of postsynaptic folds
Channels in the AchRs open
Depolarization of muscle fiber
Muscle contraction

13. After Ach reach to receptors then Ach
destroyed by enzyme
acetylcholinestrase AchE. If it not occur
only contraction of muscles take place,
not expansion or go back to original
position
Termination of contraction

14. PATHOPHYSIOLOGY
 In MG, in 85% patient antibodies are directed
toward the acetylcholine receptor at the
neuromuscular junction of skeletal muscles
 Results in:
 Decreased number of acetylcholine receptors at
the motor end-plate
 Some forms of the antibody impair the ability
of acetylcholine to bind to receptors.
 Others lead to the destruction of receptors.

15.  Myasthenia gravis is a chronic autoimmune
neuromuscular disease, characterized by
muscle weakness and fatigability.
 It is an autoimmune disorder, in which
weakness is caused by circulating antibodies
that block acetylcholine receptors at the
postsynaptic neuromuscular junction, inhibiting
the excitatory effects of the neurotransmitter
acetylcholine on receptors.

16. Clinical features

17. CLASSIFICATION:-
 The most widely accepted classification of
myasthenia gravis is the Myasthenia Gravis
Foundation of America Clinical Classification:
 Class I: Any eye muscle weakness, possible
ptosis, no other evidence of muscle
weakness elsewhere
 Class II: Eye muscle weakness of any
severity, mild weakness of other muscles

18. CLASSIFICATION:-
 Class III: Eye muscle weakness of any
severity, moderate weakness of other
muscles
 Class IV: Eye muscle weakness of any
severity, severe weakness of other
muscles
 Class V: Intubation needed to maintain
airway

19. CLINICAL FEATURES:-
 weakness of voluntary Muscles:
 painless., graudual, fluctuating(more or less)
fatigability(start everything actively but feel fatigue
after sometime)
 Mainly involve head and neck mucles, The first
noticeable symptom is weakness of the eye
muscles, diplopia, ptosis due to extraoccular
muscle weakness
 difficult in facial expressions, chewing,
(Dysphagia)
talking, and swallowing are especially
susceptible.
 Difficulty in swallowing and slurred

20. CONT……
 Weakness develop in muscles of other parts
like down muscles
 Unstable gait
 Painless, gradual onset of Weakness of
muscles like in arms, hands, fingers, legs, and
neck, a change in facial expression
 Shortness of breath (mysthenia crisis)

21. CONT……..
 Muscles become progressively weaker during
periods of activity and improve after periods of
rest.
 In myasthenic crisis a paralysis of the
respiratory muscles occurs, necessitating
assisted ventilation to sustain life.

23.  MG can be a difficult diagnosis, as the
symptoms can be hard to distinguish from
other neurological disorders. A thorough
physical examination can reveal easy
fatigability, with the weakness improving after
rest and worsening again on repeat of the
exertion testing.

24. Physical examination:-
 Muscle fatigability can be tested for many
muscles. A thorough investigation includes:
 looking upward and sidewards for 30 seconds:
ptosis and diplopia
 looking at the feet while lying on the back for
60 seconds
 keeping the arms stretched forward for 60
seconds
 ten deep knee bends
 walking 30 steps on both the toes and the

25. Blood tests:-
 Blood test is for antibodies against the
acetylcholine receptor.
 Repetitive motor nerve stimulation test shows
decrease motor response.

26. Edrophonium test-
 This test requires the IV administration of
edrophonium chloride drugs that block the
breakdown of acetylcholine by blocking
enzyme acetylcholinestrase and temporarily
increases the levels of acetylcholine so it
increase muscle contractions and relieve
weakness.
 If pt has ptosis, apply ice on upper eyelid,
muscles stimulate and eyelid moves
upward as ice freezes
acetylcholinestrase.

27.  Imaging-
 Chest X-ray, MRI identify widening of the
mediastinum suggestive of thymoma.
 Pulmonary function test-

29. Medication:-
 Acetylcholinesterase inhibitors: neostigmine
and pyridostigmine can improve muscle
function. Start with a low dose, and increase
until the desired result is achieved.
 Immunosuppressive drugs: prednisone,
cyclosporine, may be used. Patients are
commonly treated with a combination of these
drugs with an acetylcholinesterase inhibitor.

30. Plasmapheresis
 If the myasthenia is serious (myasthenic
crisis), plasmapheresis can be used to remove
the antibodies from the circulation.
 Drugs like betablockers, aminoglycosides
avoided.

31.  Inspiratory muscle therapy
 Surgery- Thymectomy, the surgical removal of the
thymus, is essential in cases of thymoma in view of the
potential neoplastic effects of the tumor.

32. Behavioral modifications:-
 Diet
 Patients may experience difficulty chewing and
swallowing due to oropharyngeal weakness
 If dysphagia develops, liquids should be thickened
 Thickened liquids decrease risk for aspiration
 Activity
 Patients should be advised to be as active as
possible but should rest frequently and avoid
sustained activity
 Educate patients about fluctuating nature of
weakness and exercise induced fatigability

33. NURSING MANAGEMENT:-
 ASSESSMENT:-
 Assess pain level due to muscle spasms.
 Assess cardiac function ( orthostatic
hypotension ).
 Assess respiratory status closely to determine
hypoventilation due to weakness.
 Cranial nerve assessment, gag reflex, motor
strength.
 Monitor life threatening complications such as
respiratory failure, DVT.

34. NURSING DIAGNOSIS:-
 Ineffective breathing pattern related to weakness,
paralysis of respiratory muscles.
 Impaired physical mobility related to paralysis.
 Imbalanced nutrition: less than body requirement
related to cranial nerve dysfunction.
 Impaired verbal communication related to vocal
paralysis.
 Self care deficit related to muscle weakness.
 Chronic pain related to disease pathology .
 Anxiety related communication difficulties and
deteriorating physical condition.

35. Prognosis:-
 With treatment, patients have a normal life
expectancy, except for those with a malignant
thymoma (whose lesser life expectancy is on
account of the thymoma itself and is otherwise
unrelated to the myasthenia).
 Quality of life can vary depending on the severity
and the cause.
 The drugs used to control MG either diminish in
effectiveness over time (acetylcholinesterase
inhibitors) or cause severe side effects of their
own (immunosuppressants).

36. GUILLAIN –
BARRE
SYNDROME

37. INTRODUCTION:-
 The French physician Jean Landry first
described the disorder in 1859. In 1916,
Georges Guillain, Jean Alexandre Barré,
diagnosed two soldiers with the illness.
 GBS is also known as acute idiopathic
polyneuritis, French polio, Landry's ascending
paralysis and Guillain Barré syndrome.

38. DEFINITION:-
GBS is an autoimmune disorder which
results demyelination of peripheral nerves
producing Ascending weakness with
Dyskinesia, Hyporeflexia, Paraesthesias.
It is an acute inflammatory demyelinating
disorder of the periphery nervous system in
which attack of the myelin sheath of nerves
by antibodies.

40. INCIDENCE:-
 Worldwide, the annual incidence is about 0.6–4
occurrences per 100,000 people.
 Men are one and a half times more likely to be
affected than women.
 The incidence increases with age.
 The incidence of GBS during pregnancy is 1.7 cases
per 100,000 of the population.
 Mortality rate –2-12%, increases with age.
 Death rate 1.3 times > in men than females after age
40s.
 Congenital and neonatal Guillain–Barré syndrome
have also been reported.

41. ETIOLOGY:-
 Exact cause is unknown.
 it is believed that the disorder is an autoimmune disease
 All forms of Guillain–Barré syndrome are due to an
immune response to foreign antigens
 Campylobacter jejuni most common.( 60% )
 CMV{Cytomegalovirus}
 EBV {Epstein-Barr Virus}
 Mycoplasma Pneumoniae
 Varicella ZosteerVirus
 HIV, Haemophillus Influenza, Herpes Zoster.
 Precipitating event include a respiratory or GI viral or
bacterial infection 1-3 weeks prior to the onset of
manifestations.

42. PATHOPHYSIOLOGY:-
Due to any cause, immune mediated
response triggers destruction of the myelin
sheath surrounding the cranial and spinal
nerves.
Accompanied by edema, inflammation of the
peripheral nerves.
Demyelinization of axons results in poor
conduction of the nerve impulses.
Sudden muscle weakness and loss of reflex
response.

43. CLINICAL FEATURES:-
 Symptoms start with numbness or tingling in
the fingers and toes, followed by weakness
in the leg and arm muscles. Unable to climb
stairs
Ascending & symmetrical in nature, lower limbs
usually involve before upper limbs & weakness
develops acutely & progressive over days to week
 CRANIAL NERVE INVOLVEMENT
• {45%-75%}
• Facial droop
• Diplopia
• Dysarthria
• dysphagia

44. Cont………
 PAIN
• {89%} Pts C/O pain during illness
• Mainly at back & legs with uncertain cause
• Throbbing in nature.
 AUTONOMIC CHANGES
• Dysfunction in SNS & PSNS include
• Tachycardia, Bradycardia
• Facial flushing, Orthostatic HTN
• Diaphoresis
• Urinary retention & Paralytic ileus
• Bowel/Bladder dysfunction

45. • Dysautonomia with severe weakness &
Respiratory failure (Dysautonomia refers to a
disorder of autonomic nervous system (ANS)
function that generally involves failure of the
sympathetic or parasympathetic system, but
dysautonomia involving excessive or overactive
ANS actions also can occur.)

46. CONT…..
 RESPIRATORY INVOLVEMENT
• {40%}C/O
• Dyspnoea on exertion
• Shortness of breath
• Difficulty in swallowing
• Slurred sppech
• Reflex changes
• { hyporeflexic/absent reflexes }

47. DIAGNOSTIC EVALUATION:-
 History taking and physical examination-
 LAB STUDIES
 CSF studies
{ elevated CSF protein, > 0.5gm/L }
 Basic LAB studies:
 CBC
 RA factor
 VIT B12, folic acid
 Hb A1C,
 ESR

48.  SEROLOGIC STUDIES:
Increase in titers for infectious agent
{ CMV, EBV, MYCOPLASMA, HIV }
 NCS
 PULMONARY FUNCTION TESTS:
Maximal Inspiratory/Expiratory pressures & Vital
Capacities

49. MANAGEMENT:-
 PHARMACOTHERAPY:
 Antibiotic agents
 Anticoagulant agents
 Analgesic
 Plasmapharesis
 IV IgG- Once a day 40ml/kg, IV, for 5 days.
 Tracheostomy- For long term mechanical ventilation.
 REHABILITATION PROGRAMME:
• Physical therapy
• Occupational therapy
• Speech therapy
• Recreational therapy

50. NURSING MANAGEMENT:-
 NURSING DIAGNOSIS-
• Ineffective breathing pattern & impaired gas
exchange R/T rapidly progressive weakness &
impending respiratory failure.
• Impaired physical mobility R/T paralysis.
• Imbalanced nutrition less than body requirement
R/T inability to swallow
• Impaired verbal communication R/T cranial nerve
dysfunction.
• Fear & anxiety R/T loss of control & paralysis

51. PROGNOSIS:-
 Recovery usually starts after the fourth week
from the onset of the disorder.
 Approximately 80% of patients have a
complete recovery within a few months to a
year, although minor findings may persist,
such as areflexia.
 About 5–10% recover with severe disability.
Worldwide, the death rate runs slightly higher
(4%).

52. REFERENCES:-
 Black Joyce, M Hawks Jane. Medical Surgical Nursing. 8th ed.
Noida: Elsevier; 2009. Vol 2.
 Monahan Frances Donavan. Sands Judith K. Neighbors Marianne.
Marek Jane F. Green Carel J. Phipp’s Medical surgical Nursing
Health & illness Perspective. Eseiviers Noida 2009. 796-802.
 Phipps Wilma J. Lang Bardora C. woods Nancy Fugate Shater’s
Medical Surgical Nursing BI Publications. New Delhi 1994 890-91.
 Lewis Sharon L. Heitkemper Margaret Mcleam. Dirksen Shannon
Ruff. O’ Brien Partricia Graber. Bucher Linda Medical Surgical
Nursing Assessment & ranagement of clinical Problems. Elseivier,
Naida. 2009 1845-49
 WWW.wikipedia.com
 http://myasthenia.org/WhatisMG.aspx#sthash.fUnxa0cj.dpuf

53. !!!ANY QUERRIES!!!