History - Myasthenia Gravis --1.pdf

Page 1 of 4 Myasthenia Gravis , Exam scenario: 7 yrs. old with h/o fatigability, reduce exercise tolerance and recurrent chest infection = myasthenia (Sudan 2019). Hi...

Page 1 of 4 Myasthenia Gravis , Exam scenario: 7 yrs. old with h/o fatigability, reduce exercise tolerance and recurrent chest infection = myasthenia (Sudan 2019). History of Present Illness: (Complaint Analysis + CNS, MSK + Other Systems Review) - Ask first are they started together or one by one, then start with the serious one first. - O C D ↑ ↓, timing (at the end of the day), increased with fatigue, tiredness and stress, relieved by rest. - Site of weakness, unilateral or bilateral, where starts first, does she have weakness in going up and down stairs, any weakness on combing her hair. - Any abnormal gait? Any abnormal movement? Any behavioural changes? - Any abnormalities in the eye (droopy eyelid)? Any concern regarding vision (diplopia)? Any redness or puffiness? Asymmetry of the face, any concern regarding her hearing, what about her sensation (if you but something cold on …, Can she feel it). - Is it associated with headache? Any morning vomiting? - What about her way of talking (i.e. nasal sound, interrupted, understandable, occur at certain time, dysarthria), any problem in swallowing to fluids or solids (dysphagia). - FWA SL. - Any other concern. - Any chronic illness or long-term medical condition (may be with other autoimmune disease). - MSK: any skin spots, any swelling in any part of the body, any hypo or hyper-pigmented lesions in the body (vitiligo), any hair loss (alopecia). - Chest: Recurrent Bugs – Cough – how many pillows using during sleep. - CVS: Heart Racing – Exercise Intolerance – chest pain or shortness of breathing especially on lying down – fainting attacks - GIT: Tummy Size – Tummy Pain – Throwing-up – describe for me his poo (loose or constipation) – yellowish discoloration. - Urinary: Pee concern (polyuria, polydipsia to exclude DM) - any one measure her Blood Pressure. - Endocrine: Hot or cold Intolerance (thyroid), any lump in the neck, any one measure his bl. Sugar. - Haematology: Pallor - recurrent bugs in the body – Swollen glands in the neck - numbness in the fingers (pernicious anaemia). Handwritten Notes Page 2 of 4 Past: 5T IOA In detail: - Ask about head trauma, drugs (if he is chronic case, ask about his medications, compliance & drug level), other drugs which may interfere with his condition (Beta-blockers, azathioprine, ciprofloxacin, nalidixic acid, neuropsychiatric drugs, penicillamine gentamycin). - previous condition (when discovered, by whom, how it was presented, is he admitted to hospital, in normal word or ICU, for how long, what they did for him, how long he stayed, after discharge any medicine was given to him, did you follow with any team? - Previous hospitalization, in normal word or ICU, what done, Investigations and medications given on admission, which team following with. Perinatal, natal & postnatal: foetal movements during pregnancy, amount of liquor around the baby, any feeding difficulties in early neonatal period, any admission to NICU. Vaccination: Missed any shots? Any extra shots? Allergy Nutrition Development: is he looks like his peers in way of walking & talking? Family: consanguinity – how many children – History of Similar Condition – history of deaths in the family especially autoimmune diseases especially mother side. Social & finance: what did you do for living, is it satisfied for you, did you have an easy access to hospital, who take care of the other kids when you go to the hospital with …, do you have insurance, receive any support. School. Psychological and impact Discussion: DD? Myopathy: DMD - Becker’s - JDM - Hyperthyroidism. Others: GBS - NMD - Horner’s. Drugs: Botulinum - Organophosphorus poisoning. Ptosis : - Bilateral: ($: Noonan, Smith-Lemli-Opitz, Rubinstein-Taybi, bilateral 3rd cranial nerve palsy, migraine, craniocystenosis). - Unilateral: Idiopathic, trauma, infection, tumour (retinoblastoma), congenital ptosis, unilateral 3rd cranial n palsy, Horner $, Marcus gun $, local pressure on the eye. Types of myasthenia gravis: Neonatal: = transient - Babies born to mothers who have myasthenia gravis due to transfer of acetylcholine Ab from mother to child. - Presented early with floppiness, feeding difficulties, weak cry, and difficulties in breathing. - Antenatally, polyhydramnios with decreased foetal movements may developed deformities like arthrogryposis. - Improves with within 4-8 weeks with anticholinesterases drugs. Congenital: - Babies born to mothers who do not have the disease, defect in neuromuscular junction in the receptors, its less severe, but less responsive to ttt. Juvenile: - Present after 10y, more in females, same as adult type with fluctuating fatigability. Handwritten Notes Page 3 of 4 Investigations: Diagnostic: (EMG, Anti-Acetylcholine Receptor Ab, Anti-Muscle Kinase Ab). Lab: ANA, anti-DNAs, screen for other autoimmune diseases. Imaging: chest x ray for thymus shadow. Plus: Clinical tests: - Endorphin test > 2yrs. (+ve if improvement for few sec & disappear in minutes) done with precautions to be done at hospital (IV-line, ECG monitoring, Atropine). - < 2 yrs. Neostigmine test with peak at 20 min. Types of Crises: Myasthenic Crisis: acute respiratory failure, ttt with admission, IVIG, plasmapheresis. Cholinergic Crisis: overdose of medications (loose motion, lacrimation, salivation) SE of pyridostigmine: miosis, bronchospasm & emesis. Ttt with: Atropine. Treatment: Antimuscarinic (Neostigmine& pyridostigmine). Immunosuppressant (steroids & azathioprine). Thymectomy. - Indication for Thymectomy: if antibody +Ve, acute presentation, generalized affection with pressure symptoms. - Benefits of Thymectomy: reduce dose of steroid & need for immunosuppressant, decrease need to go to hospital for crisis. New Biological ttt: Rituximab - Belimumab - G-CSF. Care of the Coma patient: - Admission to ICU. - Diet modification + Feeding support. - Pain management. - Ventilatory support (if indicated). - Prevention of :  Contractures: Physiotherapy  Bedsores: Frequent changing of position.  DVT: Gradient-pressure stockings.  2ry infection: Prophylactic antibiotics. MDT: Neurologist, physiotherapist, immunologist, occupational therapist, SALT for swallowing difficulties, social worker, dietitian, Liaise with school. Advice: Wear bracelet, if girl (if in labour, a senior neonatologist should attend the labour as the baby may have congenital myasthenia & need respiratory support). Avoid triggers (stress, exhaustion, tiredness). Avoid some medicines (B. blockers, azathioprine, ciprofloxacin, nalidixic acid, neuropsychiatric drugs, penicillamine gentamycin). Educate parents about side effects of medicines. Emergency card plan. Give extra vaccines (pneumococcal & flu). Handwritten Notes Page 4 of 4 Q/ Cause of Respiratory Failure: - myasthenic crisis, cholinergic crisis, respiratory infections. Handwritten Notes