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BS BIOLOGY 4a
Trajera,Francis
Benemerito, Nemorie
Myasthenia Gravis
Introduction
varying degrees of weakness of the skeletal (voluntary) muscles of the body. The name
myasthenia gravis, which is Latin and Greek in origin, literally means "grave muscle weakness."
With current therapies, however, most cases of myasthenia gravis are not as "grave" as the name
implies. In fact, most individuals with myasthenia gravis have a normal life expectancy (Tzartos
et al., 1998).
The hallmark of myasthenia gravis is muscle weakness that increases during periods of
activity and improves after periods of rest. Certain muscles such as those that control eye and
eyelid movement, facial expression, chewing, talking, and swallowing are often, but not always,
involved in the disorder. The muscles that control breathing and neck and limb movements may
also be affected.
Description
degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes,
face, and swallowing. It can result in double vision, drooping eyelids, trouble talking, and trouble
walking. Onset can be sudden. Those affected often have a large thymus gland or develop a
Myasthenia gravis is an autoimmune disease which results from antibodies that block or destroy
nicotinic acetylcholine receptors at the junction between the nerve and muscle. This prevents
nerve impulses from triggering muscle contractions.Rarely, an inherited genetic defect in the
mothers with myasthenia may have symptoms during their first few months of life, known as
neonatal myasthenia. Diagnosis can be supported by blood tests for specific antibodies, the
azathioprine, may also be used. The surgical removal of the thymus gland may improve
symptoms in certain cases. Plasmapheresis and high dose intravenous immunoglobulin may be
used during sudden flares of the condition. If the breathing muscles become significantly weak,
Case History
Case #1:
A 67-year-old man, complaining of double vision, was found to have bilateral ptosis, covering
most of the pupil on the right side and partially obscuring that on the left. The ptosis was worse
in the evening and almost absent in the morning. He admitted to tiredness in the arms and legs on
exercise, which recovered with resting. A clinical diagnosis of ocular myasthenia gravis was
made. His Tensions test was positive but electromyography was inconclusive. His serum
contained antibodies to thyroid microsomes (positive at 1/1600) and to acetylcholine receptors.
Case #2:
A 21-year-old woman was referred to a neurology clinic with a 1-month history of double
vision, difficulty swallowing and weakness in her upper arms. These symptoms were mild or
absent in the morning and tended to worsen through the day. When she was seen towards the end
of an afternoon neurology clinic she was found to have a bilateral ptosis and disconjugate eye
movements that could not be ascribed to any individual cranial nerve lesion. Her upper limb
power was initially normal but deteriorated with repeated testing. An intravenous injection of
but her eye movements deteriorated again 30min after the injection. A clinical diagnosis was
made of myasthenia gravis. Subsequent blood testing showed the presence of a high level of
She was treated with oral cholinesterase inhibitors with some improvement. However, 1
month later she deteriorated and corticosteroids were introduced with no improvement. A
computed tomography scan of her thorax showed no evidence of a thymoma but she was
nevertheless referred to a thoracic surgeon for thymectomy as this can sometimes induce
remission in myasthenia even in the absence of a thymoma. A small thymic remnant was
removed and she recovered uneventfully and was able to withdraw from all medication without
deterioration in her symptoms. Acetylcholine receptor antibody levels fell but remained
detectable. One year later, she became pregnant and after an uneventful 41-week pregnancy she
delivered a 4-kg male infant. There were immediate concerns about the baby who failed to make
adequate respiratory efforts and who appeared limp and hypotonic. The baby was intubated and
ventilated on the neonatal intensive care unit. In the light of the mother's history, a provisional
diagnosis of neonatal myasthenia gravis was made, although care was taken to exclude other
pathology. Subsequent testing of a blood sample taken from the umbilical cord showed low
levels of acetylcholine receptor antibody. The baby needed ventilation and feeding via a
nasogastric tube for 3 days at which time the ventilation was successfully withdrawn. There were
some initial feeding problems due to difficulty sucking and swallowing but these resolved over
the next 48h. The child's subsequent development has been entirely normal. The mother also
remains well.
Myasthenia Gravis (MG) is a neuromuscular disorder that causes muscle weakness. The
name Myasthenia gravis came from a Latin and Greek words which literally means grave
muscle weakness. It usually affects voluntary muscles or muscles that are can be controlled
consciously. It is characterized by weakness of muscles such as ocular, neck, limbs, bulbar and
respiratory. It is not a permanent weakness of muscle but it is fluctuating without losing the
reflexes of sensation or other neurologic function. The symptoms worsen temporarily and
improve with a rest. MG is classified as an autoimmune disease which means that the bodys
immune system attacks the connection between the nerve and muscle mistakenly. The weakness
of the muscle is the result when the muscle cells cannot respond properly to the nerve impulses
Myasthenia gravis affects approximately 100 patients per million people (Jacob et al.,
2009). According to Yu et al., 1989 women are more affected twice than men but it has no
significant gender difference in incidence exists before puberty or after the age of forty. This
deases affects all age groups and peaks in ages twenty and thirty in women while fifty and sixty
years old in men (Jacob et al., 2009). The first case reported of MG is a native American Chief
Opechancanough who died in 1664. It was described as an excessive fatigue with macerated
flesh. The vigority has been lost their tone and elasticity. The person is not able to open his
eyelids since he felt that it is so heavy and he cannot see unless his eyelids were lifted up by his
attendants. Furthermore he cannot walk due to weakness of his leg muscles (Conti, 2006).
Myasthenia gravis is considered as a serious disease but can be treated. The fatigability of
the voluntary muscles are caused by autoantibodies against the nicotinic acetylcholine receptor
(AChR) on the postsynaptic membrane at the muscular junction (Tzartos et al., 1998). The
antibodies are produced by bodys own immune system to protect it from foreign organisms but
takes place if the normal communication between the nerve and muscle is interrupted at the
neuromuscular junction. Neuromuscular junction is the place where nerve cells connect with the
muscles they innervate. Acetylcholine is one of the neurotransmitter in our brain cells that is used
by the nervous system to communicate with other brain cells. It is found in the interface between
the presynaptic nerve terminal and postsynaptic muscle membrane. Acetylcholine are released by
nerve endings and it travels from neuromuscular junction and binds to acetylcholine receptors
which are activated and generate a muscle contraction (Thanvi and Lo, 2008).
the face, difficulty in talking and breathing due to muscle weakness, trouble in swallowing and
chewing, having a hoarse voice, drooping of eyelids, fatigue, and double vision (Herndon, 2016).
Myasthenia gravis are divided into five main classes and several subclasses by the
Class I is characterized by ocular muscle weakness; inability to close the eyes; other
any severity
Class IIa is characterized by lesser involvement of oropharyngeal muscles and the limbs,
severity
Class IIIa: Affecting limb and axial muscles; involvement of oropharyngeal muscles
Class IIIb is characterized by affected oropharyngeal and respiratory muscles; lesser or
oropharyngeal muscles
Class IVb is characterized by predominantly affected oropharyngeal, respiratory or both;
lesser or equal involvement of limb, axial muscles or both; feeding of a patient is done
ventilation
who share the same severity of disease which may indicate different responses to therapy (Trouth
et al., 2012).
Several factors could trigger and worsen the disease. The following factors are: Bright
sunlight, surgery, immunization, emotional stress, menstruation, intercurrent disease such as viral
Diagnosis
Many disorders can cause weakness of muscles, Myasthenia Gravis is one of them. The
American Academy of Neurology (2016) the evaluation may include medical and neurologic
evaluation, Blood tests to check for antibodies, Blood tests or other studies to rule out other
causes of weakness, imaging scans, electrical tests of nerve and muscle function
(electromyography and nerve conduction studies), and Ice pack test to improve strength of the
eyelid.
A provisional diagnosis may be made using the edrophonium test or Tonsilon test
(Matthew, 2004), a drug called Tonsilon or a dummy medicine (inactive placebo) is given during
this test. The health care provider gives the appropriate one through one of the patient's veins, the
after injection, improvement in strength that may persist for up to 5 minutes, and requires
Myasthenia gravis frequently have other disease that have presumed or known immunological
cause such as thyroid disease, rheumatoid arthritis, and vitamin B12 deficiency (Howard, 2008).
Treatment
Thanvi (2004) the following treatment modalities are available: acetylcholinesterase inhibitors -
these drugs act by inhibiting acetylcholinesterase and thus increase availability of the
acetylcholine to act on the ACh receptors. They are usually the initial drugs used in the treatment
of myasthenia gravis and may the only drug required to treat mild disease, corticosteroids,
treatment of myasthenia gravis can be considered to involve three steps: (1) initial treatment
usually involves use of the acetylcholinesterase inhibitors. However, these drugs are usually not
adequate to control disease on their own and an additional therapy is mostly needed. (2) Often an
immune directed treatment is added, beginning with either thymectomy or high dose
corticosteroids. (3) In the long term, steroid-sparing medications are usually added to facilitate
the tapering phase. Short term therapies, intravenous immunoglobulin or plasmapheresis, may be
effective in the early stages of treatment, before thymectomy, or later during an exacerbation
(Thanvi, 2004).
References:
Conti-Fine B. M., M. Milani, and H. J. Kaminski.2006. Myasthenia gravis: past, present, and
future, Journal of Clinical Investigation, vol. 116, no. 11, pp. 28432854
Howard, J. F. 2008. Myasthenia Gravis: A Manual for Health Care Provider. Myasthenia Gravis
Foundation of America. ISBN: 0981888305
Jacob S., S. Viegas, D. Lashley & D. H. Jones. 2009. Myasthenia gravis and other neuromuscular
junction disorders. Neurology in Practice. The Bare Essentials. 9:364. doi:
10.1136/jnnp.2009.193912
Shah, A., F. Fana & N. Lorenzo. 2016. Myasthenia gravis. Medscape Drugs &
Diseases>Neurology. Retrieved from www.medscape.com/cardiology
Trouth A. J., A. Dabi, n. Solieman, M. Kurukumbi & J. Kalyanam. 2012. Review Article
Myasthenia Gravis: A Review. Hindawi Publishing Corporation. Autoimmune Diseases.
Volume 2012, Article ID 874680, doi:10.1155/2012/874680
Tzartos SJ, Barkas T, Cung MT, et al. 1998. Anatomy of the antigenic structure of a large
membrane autoantigen, the muscle-type nicotinic acetylcholine
receptor.Immunol;163:89120
YuYL, Hawkins BR, Wong VCN, et al. The Hong Kong Myasthenia Gravis Data Bank. J Hong
Kong Med Assoc. 1989;41:259-265.