REFERENCE

DIAGNOSIS & TREATMENT OF MYASTHENIA GRAVIS

IKA WULAN PERMATA

1102012118

COUNSELLOR : DR. DONNY H HAMID, SP.S

FACULTY OF MEDICINE YARSI UNIVERSITY

CLINICAL CLERKSHIP DEPARTEMENT OF NEUROLOGY
PASAR REBO REGIONAL GENERAL HOSPITAL
INTRODUCTION

Myasthenia Gravis; (My-as-theen-ee-a Grav-us) comes from the Greek and Latin words meaning "grave muscular
weakness."
Chronic autoimmune neuromuscular disorder that is characterized by fluctuating weakness of the voluntary
muscle groups.
The prevalence of MG in the United States is estimated to be about 20/100,000 population. However, MG is
probably under diagnosed and the prevalence may be higher.
ANATOMY
DEFINITION

Myasthenia gravis (MG) is an acquired autoimmune disorder characterized by fatigable and fluctuating muscle
weakness preferentially affecting certain muscle groups.
Myasthenia gravis is a disorder of neuromuscular transmission characterised by:
Weakness and fatiguing of some or all muscle groups.
Weakness worsening on sustained or repeated exertion, or towards the end of the day, relieved by rest.
This condition is a consequence of an autoimmune destruction of the NICOTINIC
POSTSYNAPTIC RECEPTORS FOR ACETYLCHOLINE.
ETIOLOGY

MG is idiopathic in most patients. Although the main cause behind its development remains speculative, the end
result is a derangement of immune system regulation. MG is clearly an autoimmune disease in which the specific
antibody has been characterized completely.
Various drugs may induce or exacerbate symptoms of MG, including the following:
Antibiotics (eg, aminoglycosides, polymyxins, ciprofloxacin, erythromycin, and ampicillin), Penicillamine, Beta-
adrenergic receptor blocking agents (eg, propranolol and oxprenolol), Lithium, Magnesium, Procainamide,
Verapamil, Quinidine, Chloroquine, Prednisone, Timolol (ie, a topical beta-blocking agent used for glaucoma),
Anticholinergics (eg, trihexyphenidyl), Neuromuscular blocking agents (eg, vecuronium and curare), Nitrofurantoin
Thymic abnormalities
EPIDEMIOLOGY
International statistics
In the United Kingdom, the prevalence of MG is 15 cases per 100,000 population. In Croatia, it is 10 cases per
100,000. In Sardinia, Italy, the prevalence increased from 0.75 per 100,000 in 1958 to 4.5 cases per 100,000 in 1986.
Age-related demographics
MG can occur at any age. Female incidence peaks in the third decade of life, whereas male incidence peaks in the
sixth or seventh decade. The mean age of onset is 28 years in females and 42 years in males.
Sex-related demographics
Classically, the overall female-to-male ratio has been considered to be 3:2
Race-related demographics
The onset of MG at a young age is slightly more common in Asians than in other races
PATHOPHYSIOLOGY
CLINICAL MANIFESTATION
Ocular weakness
DIAGNOSIS

History
Physical Examination
Evidence of coexisting autoimmune diseases
Laboratory
Radiography, CT, and MRI
Electrodiagnostic Studies
Repetitive nerve stimulation
CT-Scan of chest and mediastinum showing thymoma
in patients with Myasthenia Gravis
DIFFERETIAL DIAGNOSIS

Ocular MG : Thyroid ophthalmopathy, Cranial neuropathy, Brainstem pathology

Generalized MG : LambertEaton myasthenic syndrome (LEMS), Motor neuron disorders,
Botulism, Drug-induced MG
TREATMENT

First-line
Cholinesterase
inhibitors
Corticosteroids
Thymectomy

Second-line
Chronic
immunotherapy

Third-line
Cyclosporine
Tacrolimus
Rituximab
Plasma exchange
IV immunoglobulin
COMPLICATION

Myasthenic crisis
Thymus tumors
Other disorders
Autoimmune conditions
PROGNOSIS

Patients who present with ocular complaints have an 8085% chance of generalizing.
There is some evidence that treatment with corticosteroids may decrease this risk.
The mortality rate from MG was more than 30% before the 1960s, but with the onset of modern critical care and
immunosuppressive therapy life expectancy in MG now approaches normal.
At least 80% of patients are able to experience significant improvement in their symptoms; however, fixed
weakness may develop later in the disease course if muscle weakness is not treated optimally.
Few patients are able to wean off immunotherapy completely.
REFERENCES

National Institute of Neurological Disorders and Stroke. Myasthenia Gravis. March, 2017
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Myasthenia-Gravis-Fact-Sheet#3153_2
Shah, AK et.al, Myasthenia Gravis. Medscape. WebMD. 23 March 2016
http://emedicine.medscape.com/article/1171206-overview
Lindsay, KW et.al, Neurology and Neurosurgery Illustrated 5th. Elsevier 2010.
Gorelick, PB et.al, Clinical Neurology 2nd edition. CRC Press. Newyork 2014.
Grob D, Brunner N, Namba T, Pagala M. Lifetime course of myasthenia gravis. Muscle Nerve. 2008 Feb. 37(2):141-9.