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Questions and Answers
What is the primary cause of Neonatal Myasthenia Gravis?
What is the primary goal of Thymectomy in Myasthenia Gravis treatment?
Which of the following is NOT a diagnostic test for Myasthenia Gravis?
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?
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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?
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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?
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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?
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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?
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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?
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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?
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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?
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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?
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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?
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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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