Podcast
Questions and Answers
What is the primary cause of Neonatal Myasthenia Gravis?
What is the primary cause of Neonatal Myasthenia Gravis?
What is the primary goal of Thymectomy in Myasthenia Gravis treatment?
What is the primary goal of Thymectomy in Myasthenia Gravis treatment?
Which of the following is NOT a diagnostic test for Myasthenia Gravis?
Which of the following is NOT a diagnostic test for Myasthenia Gravis?
What is the treatment for Cholinergic Crisis?
What is the treatment for Cholinergic Crisis?
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Which of the following is a type of Myasthenia Gravis that is less severe but less responsive to treatment?
Which of the following is a type of Myasthenia Gravis that is less severe but less responsive to treatment?
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What is the name of the test used to diagnose Myasthenia Gravis in children under 2 years old?
What is the name of the test used to diagnose Myasthenia Gravis in children under 2 years old?
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What is the name of the new biological treatment option for Myasthenia Gravis?
What is the name of the new biological treatment option for Myasthenia Gravis?
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What is the primary indication for Thymectomy in Myasthenia Gravis?
What is the primary indication for Thymectomy in Myasthenia Gravis?
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What is a potential risk factor for a comatose patient developing respiratory failure?
What is a potential risk factor for a comatose patient developing respiratory failure?
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Which of the following is NOT a type of crisis that can lead to respiratory failure in a comatose patient?
Which of the following is NOT a type of crisis that can lead to respiratory failure in a comatose patient?
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What is the purpose of prescribing prophylactic antibiotics to a comatose patient?
What is the purpose of prescribing prophylactic antibiotics to a comatose patient?
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Which of the following healthcare professionals is NOT part of the multidisciplinary team (MDT) involved in the care of a comatose patient?
Which of the following healthcare professionals is NOT part of the multidisciplinary team (MDT) involved in the care of a comatose patient?
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What is the purpose of advising a comatose patient to wear a bracelet?
What is the purpose of advising a comatose patient to wear a bracelet?
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What is the reason for giving extra vaccines to a comatose patient?
What is the reason for giving extra vaccines to a comatose patient?
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What is a possible risk factor for developing an autoimmune disease?
What is a possible risk factor for developing an autoimmune disease?
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Which of the following drugs may interfere with a patient's condition and should be asked about in the patient's history?
Which of the following drugs may interfere with a patient's condition and should be asked about in the patient's history?
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What is an important aspect to ask about in the patient's perinatal history?
What is an important aspect to ask about in the patient's perinatal history?
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Which of the following is a possible complication of an autoimmune disease?
Which of the following is a possible complication of an autoimmune disease?
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What is an important aspect to ask about in the patient's developmental history?
What is an important aspect to ask about in the patient's developmental history?
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Which of the following is a possible underlying cause of myopathy?
Which of the following is a possible underlying cause of myopathy?
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What is an important aspect to ask about in the patient's family history?
What is an important aspect to ask about in the patient's family history?
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Which of the following is a possible symptom of an autoimmune disease?
Which of the following is a possible symptom of an autoimmune disease?
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Study Notes
Types of Ptosis
- Unilateral: idiopathic, trauma, infection, tumor (retinoblastoma), congenital, 3rd cranial nerve palsy, Horner's, Marcus Gunn jaw-winking, local pressure on the eye
- Bilateral: Noonan, Smith-Lemli-Opitz, Rubinstein-Taybi, bilateral 3rd cranial nerve palsy, migraine, craniocystenosis
Types of Myasthenia Gravis
- Neonatal: transient, babies born to mothers with myasthenia gravis, presented with floppiness, feeding difficulties, weak cry, and breathing difficulties, improves with anticholinesterase drugs within 4-8 weeks
- Congenital: defect in neuromuscular junction, less severe, less responsive to treatment, babies born to mothers without the disease
- Juvenile: presents after 10 years, more common in females, same as adult type with fluctuating fatigability
Investigations
- Diagnostic: EMG, anti-acetylcholine receptor Ab, anti-muscle kinase Ab
- Lab: ANA, anti-DNA, screen for other autoimmune diseases
- Imaging: chest x-ray for thymus shadow
- Clinical tests: edrophonium test (>2 years), neostigmine test with peak at 20 minutes
Types of Crises
- Myasthenic crisis: acute respiratory failure, treatment with admission, IVIG, plasmapheresis
- Cholinergic crisis: overdose of medications, symptoms include loose motion, lacrimation, salivation, treatment with Atropine
Treatment
- Antimuscarinic: neostigmine and pyridostigmine
- Immunosuppressant: steroids and azathioprine
- Thymectomy: indication for thymectomy includes antibody positivity, acute presentation, generalized affection with pressure symptoms, benefits include reduced dose of steroid and need for immunosuppressant, decreased need for hospitalization for crisis
- New biological treatments: rituximab, belimumab, G-CSF
Care of the Coma Patient
- Admission to ICU
- Diet modification and feeding support
- Pain management
- Ventilatory support (if indicated)
- Prevention of contractures, bedsores, DVT, and 2ry infection
Advice
- Wear a bracelet
- Avoid triggers such as stress, exhaustion, and tiredness
- Avoid certain medications
- Educate parents about side effects of medicines
- Emergency card plan
- Give extra vaccines (pneumococcal and flu)
- Liaise with school
History Taking
- Past medical history: enquire about head trauma, drugs, and other conditions
- Perinatal, natal, and postnatal history: enquire about foetal movements, amount of liquor, feeding difficulties, and admission to NICU
- Vaccination history
- Nutrition and development
- Family history: consanguinity, history of similar condition, and history of deaths in the family especially autoimmune diseases
- Social and financial history: occupation, access to hospital, insurance, and support
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