Management of Myasthenia Gravis in ENT Practice
Dr.K.K.Desarda. Leena Parulekar .Hemant Todmal
Abstract:
Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response.
In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain movement does not adequately reach the muscle cells. This is caused when immune cells target and attack the body's own cells (an autoimmune response). This immune response produces antibodies that attach to affected areas, preventing muscle cells from receiving chemical messages (neurotransmitters) from the nerve cell.
The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gravis have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus.
Myasthenia gravis affects about 3 of every 10,000 people and can affect people at any age. It is most common in young women and older men.
Introduction
Myasthenia gravis is a neuro–muscular disorder characterized by marked weakness and fatigue of muscles affecting the motor apparatus at the Myoneural junction. The management of this disease in ENT practice has been outlined briefly below. This disorder involves all age groups and any muscles in the body, but the disease shows special affinity for muscles innervated by bulbar nuclei (face, lips, eyes, tongue and neck). It is because of this affection that patients of this disorder first go to see the Otolaryngologists.
The cause of the myasthenia gravis is not known, but it is said to be a metabolic disorder. Thymus gland abnormalities have been described in some patients. It has also been said that myasthenia gravis is an auto immune disease, since multiple auto antibodies have been found in the sera of patients of this disorder. The women are more often affected than men and the disease appears between 20 and 40 years of age.
Twenty cases of proved myasthenia gravis were being treated at KEM hospital in Pune since 1978. Out of this, three patients required thymectomy, two patients went into respiratory failure and had to be subjected for tracheotomy and were subsequently put on the respirator. They died ultimately. Fourteen cases were managed on medical therapy e.g. neostigmine, alternate day steroids and supportive therapy.
Three interesting cases which were presented to our department are given below. The patients were diagnosed for oropharyngeal complaints like dysphagia, dyspnoea, dysphonia, hoarseness, diplopia and ptosis, mimicking the presentation of bulbar palsy, cricopharyngeal and nasopharyngeal malignancies, with neurological involvements.
Case Reports:
Case 1
A 40 year old man complaining of difficulty in swallowing, heaviness of speech and inability to open his eye, was referred by his practitioner to ENT OPD as the symptoms were rapidly progressing. The detailed examination of his nose, pharynx, nasopharynx and larynx did not reveal any abnormality except weak movements of palate (soft), pharyngeal wall and vocal cords. There was no evidence of any malignancy either.
Routine blood, urine, X–ray chest, ECG and lipid profiles were within normal limits. The only positive findings were ptosis, diplopia and marked fatigue. The nasal regurgitation ,dysarthria and the patient had difficulty in swallowing even liquids. A provisional diagnosis of Myasthenia gravis was made and confirmed by a positive neostigmine test.
Case 2
A 40 year old female was admitted for difficulty in breathing, hoarseness of voice and difficulty in swallowing. A routine ENT examination was done. Her nose, nasopharynx and larynx looked normal except for weakness in palatal movements, pharyngeal musculature and sluggish mobility of the cords. She had ptosis though diplopia and fundi were normal. Routine blood, urine and serum electrolyte studies did not reveal any abnormality. An X–ray of the chest revealed huge shadows in the mediastinum suggestive of thymoma. Provisional diagnosis of myasthenia gravis was confirmed by positive neostigmine test.
Case 3
A 35 year old female was attending an ENT clinic for slurring of speech, nasal regurgitation and change of voice for six weeks. She would feel fine in the mornings, but as the day advanced, the symptoms would progress and at the end of the day, she would become dyspneic and dysphonic with marked fatigue in articulation.
A routine ENT examination did not reveal any marked abnormality except weakness in palatal movements, nasal twang, and weak movements of vocal cords. Even routine hemogram, urine and chest X–ray did not reveal any abnormality. A provisional diagnosis of myasthenia gravis was made and confirmed by a positive neostigmine test.
Clinical Presentation
Most of these patients come to an otolaryngologist for their nasal or pharyngeal symptoms.
Clinical presentation is as follows:
• Occular Symptoms: Ptosis, diplopia or both.
• Oropharyngeal, symptoms: Dysphagia, dysarthria, dyspnoea.
• Weakness of muscles of mastication and facial muscles.
• Nasal twang (nasal speech), nasal regurgitation and marked fatigue in articulation.
• Pharyngeal musculature weakness may cause dysphagia for liquids and solids.
• Laryngeal muscle weakness may cause dyspnea, dysphonia and hoarseness of voice and weak cough reflex.
• Involvement of respiratory muscle may cause respiratory striders.
Diagnosis
It is based on a detailed history of the clinical course of the disease, clinical examination and laboratory investigation. The positive finding like ptosis, diplopia, facial weakness and progressive fatigue in chewing, swallowing and speaking. The diagnosis is confirmed by a positive neostigmine test.
The hysteria, disseminated sclerosis, bulbar lesions, and polymyositis do mimic the picture of myasthenia gravis and should be excluded by appropriate tests and laboratory investigations. It is also stressed that malignancy of oropharyngeal, nasopharyngeal spaces should be kept in mind in evaluating the diagnosis. The importance of positive neostigmine test is the main stay in the diagnosis of the myasthenia gravis.
Treatment
There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods of remission.
Lifestyle adjustments may enable continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms.
Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications that suppress the immune response (such as azathioprine, cyclosporine, or mycophenolate mofetil) may be used if symptoms are severe and there is inadequate response to other medications.
Plasmapheresis, a technique in which blood plasma containing antibodies against the body is removed from the body and replaced with fluids (donated antibody-free plasma or other intravenous fluids), may reduce symptoms for up to 4 - 6 weeks and is often used to optimize conditions before surgery.
When other treatments do not improve systems, patients may receive intravenous immunoglobulin.
Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines.
Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.
Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.
Medical treatment
• Most of these patients are well maintained on a regime of medical drugs.
• Neostigmine bromide 15 mg orally four times/day and may be increased upto 180 mg/day.
• Edrophonium chloride (Tensilon) 10 mg 1/v may relieve myasthenic weakness in 20 to 30 seconds. 25–30 mg im daily gives relief for several hours.
• Ephenrine sulphate: 12 mg/day. The side effects of these drugs like abdominal cramps, nausea, vomiting may be prevented by adding an atropine like drug.
• Corticosteroids and corticotropine: Encouraging results have been reported. Taking alternative day steroids orally is the recommended therapy.
Surgical treatment
Myasthenia gravis with Thymus tumor can be subjected for Thymectomy in females under 40 years, where medical treatment has failed. Complete remission may occur in 1/3rd of patients. The surgical result in men are uncertain.
Deep X–ray therapy
Those patients whose medical treatment has failed and those who have refused surgery, may be subjected for deep X–ray therapy (3000 R) to thymus in 10 to 12 divided doses. Partial remission may occur in half the patients.
Emergency Treatment
The Myasthenic crisis is an emergency and should be attended to immediately. Sudden inability to breath or swallow may occur any time. These patients should carry two ampules of 0.5 mg. of Neostigmine methysulphate to be given immediately S/c or 1/m if severe symptoms develop.
Progressive and potentially dangerous respiratory stridor may take place and will require immediate emergency tracheostomy. Therefore both, a tracheostomy oxygen and suction unit should be kept near the patient. If these facilities are not available, then the patient should be transferred to an intensive care unit for management where respirator facilities will be available. During this emergency treatment body fluids and electrolytes should be maintained.
Prognosis:
As regards the prognosis of this condition, it is always guarded. The spontaneous remission occurs frequently but a relapse is the rule. Myasthenic crisis and sudden death may also occur. The most critical period following the onset is two years.There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. Patients that only have eye symptoms (ocular myasthenia gravis), may progress to have generalized myasthenia over time.
Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.
Possible Complications
Restrictions on lifestyle (possible)
Side effects of medications (see the specific medication)
Complications of surgery
Myasthenic crisis (breathing difficulty), may be life threatening
.
References
• Brumner N. G. (1972). ‘Corticosteroids in Myasthenia Gravis’. Neurology 22, COC.
• Cape C. A., Utterback R. A. (1973). ‘Maintenance of ARTH in Myasthenia Gravis’. New England Journal Med. 27,288.
• Kreal et al. (1967). ‘Role of Thymectomy in the management of Myasthenia Gravis’. Ann Surgery 165,111.
• Warmolts J. R., Engel W. K. (1972). ‘Benefit from alternate prednisone in Myasthenia Gravis’. New England J Med. 17,286.
•
Contributed by
Dr. K. K. Desarda
Prof.& Head otolaryngology,
KEM Hospital Pune.
Dr.K.K.Desarda. Leena Parulekar .Hemant Todmal
Abstract:
Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response.
In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain movement does not adequately reach the muscle cells. This is caused when immune cells target and attack the body's own cells (an autoimmune response). This immune response produces antibodies that attach to affected areas, preventing muscle cells from receiving chemical messages (neurotransmitters) from the nerve cell.
The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gravis have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus.
Myasthenia gravis affects about 3 of every 10,000 people and can affect people at any age. It is most common in young women and older men.
Introduction
Myasthenia gravis is a neuro–muscular disorder characterized by marked weakness and fatigue of muscles affecting the motor apparatus at the Myoneural junction. The management of this disease in ENT practice has been outlined briefly below. This disorder involves all age groups and any muscles in the body, but the disease shows special affinity for muscles innervated by bulbar nuclei (face, lips, eyes, tongue and neck). It is because of this affection that patients of this disorder first go to see the Otolaryngologists.
The cause of the myasthenia gravis is not known, but it is said to be a metabolic disorder. Thymus gland abnormalities have been described in some patients. It has also been said that myasthenia gravis is an auto immune disease, since multiple auto antibodies have been found in the sera of patients of this disorder. The women are more often affected than men and the disease appears between 20 and 40 years of age.
Twenty cases of proved myasthenia gravis were being treated at KEM hospital in Pune since 1978. Out of this, three patients required thymectomy, two patients went into respiratory failure and had to be subjected for tracheotomy and were subsequently put on the respirator. They died ultimately. Fourteen cases were managed on medical therapy e.g. neostigmine, alternate day steroids and supportive therapy.
Three interesting cases which were presented to our department are given below. The patients were diagnosed for oropharyngeal complaints like dysphagia, dyspnoea, dysphonia, hoarseness, diplopia and ptosis, mimicking the presentation of bulbar palsy, cricopharyngeal and nasopharyngeal malignancies, with neurological involvements.
Case Reports:
Case 1
A 40 year old man complaining of difficulty in swallowing, heaviness of speech and inability to open his eye, was referred by his practitioner to ENT OPD as the symptoms were rapidly progressing. The detailed examination of his nose, pharynx, nasopharynx and larynx did not reveal any abnormality except weak movements of palate (soft), pharyngeal wall and vocal cords. There was no evidence of any malignancy either.
Routine blood, urine, X–ray chest, ECG and lipid profiles were within normal limits. The only positive findings were ptosis, diplopia and marked fatigue. The nasal regurgitation ,dysarthria and the patient had difficulty in swallowing even liquids. A provisional diagnosis of Myasthenia gravis was made and confirmed by a positive neostigmine test.
Case 2
A 40 year old female was admitted for difficulty in breathing, hoarseness of voice and difficulty in swallowing. A routine ENT examination was done. Her nose, nasopharynx and larynx looked normal except for weakness in palatal movements, pharyngeal musculature and sluggish mobility of the cords. She had ptosis though diplopia and fundi were normal. Routine blood, urine and serum electrolyte studies did not reveal any abnormality. An X–ray of the chest revealed huge shadows in the mediastinum suggestive of thymoma. Provisional diagnosis of myasthenia gravis was confirmed by positive neostigmine test.
Case 3
A 35 year old female was attending an ENT clinic for slurring of speech, nasal regurgitation and change of voice for six weeks. She would feel fine in the mornings, but as the day advanced, the symptoms would progress and at the end of the day, she would become dyspneic and dysphonic with marked fatigue in articulation.
A routine ENT examination did not reveal any marked abnormality except weakness in palatal movements, nasal twang, and weak movements of vocal cords. Even routine hemogram, urine and chest X–ray did not reveal any abnormality. A provisional diagnosis of myasthenia gravis was made and confirmed by a positive neostigmine test.
Clinical Presentation
Most of these patients come to an otolaryngologist for their nasal or pharyngeal symptoms.
Clinical presentation is as follows:
• Occular Symptoms: Ptosis, diplopia or both.
• Oropharyngeal, symptoms: Dysphagia, dysarthria, dyspnoea.
• Weakness of muscles of mastication and facial muscles.
• Nasal twang (nasal speech), nasal regurgitation and marked fatigue in articulation.
• Pharyngeal musculature weakness may cause dysphagia for liquids and solids.
• Laryngeal muscle weakness may cause dyspnea, dysphonia and hoarseness of voice and weak cough reflex.
• Involvement of respiratory muscle may cause respiratory striders.
Diagnosis
It is based on a detailed history of the clinical course of the disease, clinical examination and laboratory investigation. The positive finding like ptosis, diplopia, facial weakness and progressive fatigue in chewing, swallowing and speaking. The diagnosis is confirmed by a positive neostigmine test.
The hysteria, disseminated sclerosis, bulbar lesions, and polymyositis do mimic the picture of myasthenia gravis and should be excluded by appropriate tests and laboratory investigations. It is also stressed that malignancy of oropharyngeal, nasopharyngeal spaces should be kept in mind in evaluating the diagnosis. The importance of positive neostigmine test is the main stay in the diagnosis of the myasthenia gravis.
Treatment
There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods of remission.
Lifestyle adjustments may enable continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms.
Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications that suppress the immune response (such as azathioprine, cyclosporine, or mycophenolate mofetil) may be used if symptoms are severe and there is inadequate response to other medications.
Plasmapheresis, a technique in which blood plasma containing antibodies against the body is removed from the body and replaced with fluids (donated antibody-free plasma or other intravenous fluids), may reduce symptoms for up to 4 - 6 weeks and is often used to optimize conditions before surgery.
When other treatments do not improve systems, patients may receive intravenous immunoglobulin.
Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines.
Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.
Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.
Medical treatment
• Most of these patients are well maintained on a regime of medical drugs.
• Neostigmine bromide 15 mg orally four times/day and may be increased upto 180 mg/day.
• Edrophonium chloride (Tensilon) 10 mg 1/v may relieve myasthenic weakness in 20 to 30 seconds. 25–30 mg im daily gives relief for several hours.
• Ephenrine sulphate: 12 mg/day. The side effects of these drugs like abdominal cramps, nausea, vomiting may be prevented by adding an atropine like drug.
• Corticosteroids and corticotropine: Encouraging results have been reported. Taking alternative day steroids orally is the recommended therapy.
Surgical treatment
Myasthenia gravis with Thymus tumor can be subjected for Thymectomy in females under 40 years, where medical treatment has failed. Complete remission may occur in 1/3rd of patients. The surgical result in men are uncertain.
Deep X–ray therapy
Those patients whose medical treatment has failed and those who have refused surgery, may be subjected for deep X–ray therapy (3000 R) to thymus in 10 to 12 divided doses. Partial remission may occur in half the patients.
Emergency Treatment
The Myasthenic crisis is an emergency and should be attended to immediately. Sudden inability to breath or swallow may occur any time. These patients should carry two ampules of 0.5 mg. of Neostigmine methysulphate to be given immediately S/c or 1/m if severe symptoms develop.
Progressive and potentially dangerous respiratory stridor may take place and will require immediate emergency tracheostomy. Therefore both, a tracheostomy oxygen and suction unit should be kept near the patient. If these facilities are not available, then the patient should be transferred to an intensive care unit for management where respirator facilities will be available. During this emergency treatment body fluids and electrolytes should be maintained.
Prognosis:
As regards the prognosis of this condition, it is always guarded. The spontaneous remission occurs frequently but a relapse is the rule. Myasthenic crisis and sudden death may also occur. The most critical period following the onset is two years.There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. Patients that only have eye symptoms (ocular myasthenia gravis), may progress to have generalized myasthenia over time.
Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.
Possible Complications
Restrictions on lifestyle (possible)
Side effects of medications (see the specific medication)
Complications of surgery
Myasthenic crisis (breathing difficulty), may be life threatening
.
References
• Brumner N. G. (1972). ‘Corticosteroids in Myasthenia Gravis’. Neurology 22, COC.
• Cape C. A., Utterback R. A. (1973). ‘Maintenance of ARTH in Myasthenia Gravis’. New England Journal Med. 27,288.
• Kreal et al. (1967). ‘Role of Thymectomy in the management of Myasthenia Gravis’. Ann Surgery 165,111.
• Warmolts J. R., Engel W. K. (1972). ‘Benefit from alternate prednisone in Myasthenia Gravis’. New England J Med. 17,286.
•
Contributed by
Dr. K. K. Desarda
Prof.& Head otolaryngology,
KEM Hospital Pune.
I had myasthenia gravis since 2015. I got medically discharged out of the Army, a job I loved well. My heart had 5 myasthenic crises, 3 being severe enough to be ventilated or require CPAP (continuous positive airway pressure) helmet. I have had countless plasma exchanges as my veins are bad. I also needed Hickman lines inserted. I have been on azathioprine, mycophenolate (CellCept), methotrexate and none have worked. I'm currently done with my herbal remedy I purchase from totalcureherbsfoundation .com which has totally cured my condition with a surprise after almost four months of their usage, I was discouraged and never thought I would be myasthenia gravis (MG) free ,to me the best to get rid of this condition is totalcureherbsfoundation com treatment because all medications I used never worked include mycophenolate (CellCept)
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