MYASTHENIA GRAVIS

1. MYASTHENIA
GRAVIS
BY
DR PULLA UMA MAHESWARI
DEPARTMENT OF PAEDIATRICS
PG PART - II

2. OBJECTIVES
• DEFINE MYASTHENIA GRAVIS
• LIST THE PREDISPOSING FACTORS OF MG
• EXPAIN THE PATHOPHYSIOLOGY OF MG
• CLASSIFY THE MF
• LIST THE INVESTIGATIONS
• EXPLAIN THE PROGNOSIS AND MANAGEMENT OF MG
• DESCRIBE THE HOMOEOPATHY MANAGEMENT OF MG
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3. DEFINITION
• Myasthenia gravis (MG) is a chronic autoimmune disease of the postsynaptic
endplate leading to abnormal neuromuscular transmission or blockade,
characterized clinically by rapid fatigability of striated muscle, particularly
extraocular and palpebral muscles and those of swallowing.
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4. PREDISPOSING FACTORS
• Myasthenia gravis may be inherited as a rare, genetic disease, acquired by babies born
to mothers with MG
• Nerves communicate with the muscles by releasing chemicals, called
neurotransmitters.
• Immune system produces antibodies that block or destroy many of the muscles'
receptor sites for a neurotransmitter called acetylcholine.
• With fewer receptor sites available, muscles receive fewer nerve signals, resulting in
weakness.
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5. PATHOPHYSIOLOGY
• Antibodies block the binding of ACh to the AChR.
• INCREASES THE DEGRADATION rate of AChR
ANTIBODIES
CROSS LINKING OF RECEPTORS
CLUSTERING
ENDOCYTOSIS
DEGRADATION
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7. THYMUS GLAND
• Thymus gland, a part of your immune
system located in the upper chest beneath
the breastbone, may trigger or maintain the
production of antibodies that result in the
muscle weakness common in MG.
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8. THYMIC AND OTHER ASSOCIATED DISORDERS
• Thymus is abnormal in 75% of patients with MG
• In ~65% the thymus is "hyperplastic,"
• 10% of patients have thymic tumors (“ neoplastic”)
• Thymomas with malignant characteristics may spread locally Upon distant spread, the lungs
and liver are usually affected.
• Muscle-like cells within the thymus (myoid cells) which bear AChRs on their surface may
trigger immune response
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10. CLASSIFICATION
• Classification according to age :
a. Congenital MG
b. NEONATAL ( transient MG)
c. Juvenile MG
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11. CONGENITAL MG
 Very rare non-immune form of MG that is inherited as an autosomal recessive disease.
 This means that both males and females are equally affected and that two copies
of the gene, one inherited from each parent, are necessary to have the condition.
 Symptoms of congenital MG usually begin in the baby's first year and are life-long.
 Family history often present
 Anti Ach R antibodies not present
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12. TRANSIENT MG
• Between 10 and 20 percent of babies born to mothers with MG may have a
temporary form of MG.
• This occurs when antibodies common in MG cross the placenta to the
developing fetus.
• Neonatal MG usually lasts only a few weeks, and babies are not at greater risk for
developing MG later in life.
• clinically : present with local or generalized weakness, respiratory depression
• symptoms subsided over 1 to 4 weeks
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13. JUVENILE MG
• This auto-immune disorder develops typically in female adolescents
• Has the same presentation and response to therapy as adult type
• Antibodies directed against AChR in skeletal muscle
• Cell and complement mediated process
• This leads to a reduced number and function of AChRs
• Almost never occurs before 1 year of age.
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14. CLINICAL MANIFESTATIONS
• The age of onset for immune-mediated MG ranges anywhere from 11 mo to 17 yr of age.
• In the prepubertal age-groups, the female:male ratio is about 1.5:1, and in the postpubertal
age-groups, the female:male ratio is about 1 : 1.
• In juvenile autoimmune MG, unilateral or bilateral but usually asymmetric ptosis and some
degree of extraocular muscle weakness are the earliest and most constant signs.
• Diplopia
• Dysphagia and facial weakness also are common
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15. • In early infancy, feeding difficulties are frequent as the cardinal sign of myasthenia; in
severe cases, aspiration and airway obstruction may occur.
• Poor head control because of weakness of the neck flexors may be prominent
• Rapid fatigue of muscles is a characteristic feature of MG that distinguishes it from most
other neuromuscular diseases
• Ptosis increases progressively as patients are asked to sustain an upward gaze for 30-90
sec.
• Repetitive opening and closing of the fists produces rapid fatigue of hand muscles, and
patients cannot elevate their arms for more than 1-2 min because of fatigue of the deltoids
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16. INVESTIGATIONS
 Diagnosis is made after the sudden or gradual onset of specific symptoms and after
diagnostic testing.
 It is confirmed with a Tensilon test. With this test, a small amount of medicine (Tensilon) is
injected into the child, if the child has MG, an immediate, but brief, increase in muscle
tone is noted.
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17.  Genetic tests - diagnostic tests that evaluate for conditions that have a
tendency to run in families.
 Electromyogram (EMG) - a test that measures the electrical activity of
a muscle or a group of muscles
 Muscle biopsy - a small sample of the muscle is removed and
examined to determine and confirm a diagnosis or condition.
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18. PROGNOSIS
• Long-term outlook better for children than adults
• Life expectancy slightly reduced
• Most favorable outcome for bulbar weakness
• Drug free remissions possible with thymectomy
• Death rate reduced from 30% to <5% with pharmacotherapy and surgery
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19. MANAGEMENT
 Intravenous immune globulin
 Therapeutic management
 Plasmapheresis. This procedure uses a filtering process similar to dialysis. Blood is routed
through a machine that removes the antibodies that are blocking transmission of signals
from nerve endings to muscles' receptor sites. However, the beneficial effects usually last
only a few weeks.
 Thymectomy
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20. RELATED RUBRICS
MURPHY REPERTORY :
• Muscles- myasthenia gravis
BOERICK REPERTORY
• Eyes, Ocular muscles, Paralysis
• FACE, Muscles, facial, Paralysis
• LOCOMOTOR SYSTEM, Thighs, legs, Weakness, easily fatigued
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21. HOMOEOPATHY MANAGEMENT
• ALUMINA
• COCCULUS
• GELSEMIUM
• CONIUM
• CURARE
• PLUMBUM MET
• ZINCUM MET
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22. ALUMINA
• lack of vital heat, prematurely old people due to debility.
• Heaviness, staggering and sluggishness with very bad constipation are the characteristics
of the remedy.
• Extremities feel paralyzed; leg feels asleep specially when sitting with legs crossed.
• Heels feel numb, tenderness in soles, feel soft and swollen.
• Inability to walk especially when eyes are open or in daytime.
• Pain in arm and fingers, as if hot iron penetrated.
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23. COCCULUS INDICUS
• Homeopathic medicine for myasthenia gravis that has weakness of every muscle.
• Affect primarily to the voluntary muscle system then sensorium.
• In muscular system cocculus produces paralysis, titanic convulsion and in sensorium produces
vertigo and confusion.
• Paralytic weakness in muscle.
• Weakness of cervical muscles cannot support head.
• Painful stiffness in neck when moving it. Hands feel numb and asleep.
• Hands and feet become cold after change of position
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24. GELSEMIUM
• Gelsemium act upon the muscles and motor nerves, weakness of throat, chest, larynx,
sphincter, extremities, etc. Muscular weakness.
• Complete relaxation and prostration.
• Lack of muscular co-ordination General prostration.
• Dizziness, drowsiness, dullness, and trembling.
• Loss of power of muscular control.
• Excessive trembling and weakness of all limbs. Hysteric convulsions.
• Fatigue after slight exercise.
• In muscles causes overpowering, aching, tiredness, heaviness, weakness and soreness.
• Heavy drooping of eyelids.
• Excessive trembling and weakness of limbs
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25. CONIUM
• Conium is an excellent remedy, such as difficult gait, trembling, sudden loss of strength while
walking, painful homeopathy medicine for myasthenia gravis stiffness of legs.
• It corresponds to the debility, hypochondriasis, urinary troubles, weakened memory, sexual
debility found here.
• Great debility in the morning in bed.
• Weakness of body and mind, trembling, and palpitation.
• Ascending symptoms, paralysis after diphtheria.
• Perspiration of hands.
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26. CURARE
• Homeopathic medicine for myasthenia gravis that has muscular paralysis without impairing
sensation and consciousness.
• Weakness of respiratory muscles.
• Reflex action diminished.
• Tired pain up and down spine.
• Arms weak, heavy.
• Cannot lift the fingers.
• Weakness of hands and fingers.
• Legs tremble; give way in walking.
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27. PLUMBUM MET
• Localized neuralgic pains, neuritis. Progressive muscular atrophy. Locomotor ataxia.
Excessive and rapid emaciation. The points of attack for Plumbum are the neuraxons and
the anterior horns. Cannot raise or lift anything with the hand. Extension is difficult.
Paralysis from overexertion of the extensor muscles in piano players. Pains in muscles of
thighs; come in paroxysms. Cramps in calves. Stinging and tearing in limbs, also twitching
and tingling, numbness, pain or tremor. Feet swollen. Pain in atrophied limbs alternates
with colic. Loss of patellar reflex. Hands and feet cold. Pain in right big toe at night, very
sensitive to touch.
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28. ZINCUM METALLICUM
• In chronic diseases with brain and spinal symptoms, trembling, convulsive twitching and
fidgety feet are guiding symptoms.
• Lameness, weakness, trembling and twitching of various muscles. Feet in continued
motion; cannot keep still.
• Transverse pains, especially in upper extremity. Soles of feet sensitive. Steps with entire
sole of foot on floor.
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