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Myasthenia gravis and Muscular diseases Part One: Each item below contains a question or incomplete statement followed by suggested responses. Select the one best response to each question. 1. Myasthenia gravis cholinergic crisis is due to a. Anti acetylcholinesterase drug overdose b. Esterase activity disappeared c. Anti Che drug dosage is insufficient d. Anti Che drug action suddenly disappeared e. Anti acetylcholinesterase drug allergy 2. Which basis does not support the myasthenia gravis clinical? a. Limbs after motion is easy to strain b. Volatility ptosis and diplopia c. The morning light both heavy limbs weakness d. Low frequency electrical stimulation point attenuation is more than 10% e. Fatigue test symptoms without improved after the break 3. Low blood potassium periodic paralysis of the clinical manifestations, which is the right one? a. Is more common in elderly patients b. Cranial nerve damage c. Symmetry retardation paralysis d. Tendon reflex e. Positive pathologic reflex 4. The rash typically associated with dermatomyositis is characterized by which of the following? a. Adenoma sebaceum b. Shagreen patches c. Target-shaped erythematous lesions on the extremities d. A purplish discoloration around the eyes e. Telangiectasias 5. A 65-year-old man was diagnosed with lung cancer 6 months ago. Over the past 2 months, he has had worsening severe proximal muscle weakness. He is most likely to have which of the following? a. Dermatomyositis b. Trichinosis c. Multiple sclerosis (MS) d. Progressive multifocal leukoencephalopathy (PML) e. Myasthenia gravis 6. A 2-year-old male child has recently been diagnosed with muscular dystrophy. The parents are highly educated people, but not in the medical field. They have many specific and detailed questions. Which abnormal gene is responsible for the pathology of Duchenne muscular dystrophy? a. Glucose-6-phosphatase b. Hexosaminidase B c. Myosin d. Dystrophin e. Actin 7. A 67-year-old woman has noticed blurry vision and weakness over the past 4 months. Her symptoms are always worse toward the end of the day. She undergoes a neuromuscular evaluation, including electromyography, and the diagnosis of myasthenia gravis is made. Which of the following is the most obvious site of disease in myasthenia gravis? a. Anterior horn cell b. Neuromuscular junction c. Sensory ganglion d. Parasympathetic ganglia e. Sympathetic chain 8. A patient with amyotrophic lateral sclerosis develops progressive difficulty breathing. His cough becomes totally ineffective for clearing his airway, and he requires a tracheostomy. Facial muscle weakness and fasciculations are obvious at the time the tracheostomy is performed. Which of the following is the most appropriate treatment for this patient? a. Atropine sulfate b. Pyridostigmine c. Edrophonium d. Amantadine e. Chest physical therapy 9. A 28-year-old woman has the clinical diagnosis of myopathy and undergoes a muscle biopsy for diagnosis. The pathology demonstrates an inflammatory muscle disease characterized by non-caseating granulomas. Which of the following may have caused her symptoms? a. Cysticercosis b. Tuberculosis c. Sarcoidosis d. Schistosomiasis e. Carcinomatosis 10. A 62-year-old woman has limb discomfort and trouble getting off the toilet. She is unable to climb stairs and has noticed a rash on her face about her eyes. On examination, she is found to have weakness about the hip and shoulder girdle. Not only does she have a purplish-red discoloration of the skin about the eyes, but she also has erythematous discoloration over the finger joints and purplish nodules over the elbows and knees. Which of the following is the most likely diagnosis? a. Systemic lupus erythematosus b. Psoriasis c. Myasthenia gravis d. Dermatomyositis e. Rheumatoid arthritis 11. A 32-year-old woman has several family members with Duchenne dystrophy. She has genetic testing and is known to be a carrier of the gene. A blood test may exhibit substantial elevations in her serum of which of the following? a. Ammonia b. Myoglobin c. Phosphofructokinase d. Creatine phosphokinase (CPK) e. Hexosaminidase 12. Duchenne dystrophy affects approximately what percentage of infants? a. 1 in 3,000 infants b. 1 in 3,000 male infants c. 1 in 30,000 infants d. 1 in 30,000 male infants e. 1 in 50,000 infants 13. A 2-year-old male child has recently been diagnosed with muscular dystrophy. The parents are highly educated people, but not in the medical field. They have many specific and detailed questions. For a female child to have Duchenne dystrophy, she must have which of the following? a. Turner syndrome (XO) b. Klinefelter syndrome (XXY) c. Two affected parents d. An affected father e. An affected brother 14. A biopsy is obtained from a clinically affected muscle in a person with several months of progressive weakness. The pathologist reports that there are numerous abnormally small muscle fibers intermingled with hypertrophied muscle fibers. The normal mosaic of muscle fiber types is disrupted. There is no significant inflammatory infiltrate. This pathologic description is most consistent with which of the following? a. Disuse atrophy b. Denervation atrophy c. Muscular dystrophy d. Polymyositis e. Hypoxic damage 15. A 52-year-old left-handed woman says that she has a history of myasthenia gravis. When asked about details of the history she says that she was weak. With further prompting the patient becomes belligerent and says that she does not remember any further details. Which of the following is the most common manifestation of muscle weakness with myasthenia gravis? a. Diaphragmatic weakness b. Wristdrop c. Footdrop d. Ocular muscle weakness e. Dysphagia 16. A patient with amyotrophic lateral sclerosis dies within 9 months of his initial evaluation. An autopsy is performed, but only the central nervous system (CNS) can be examined. Examination of the spinal cord would be expected to reveal degeneration of which of the following? a. Dorsal root ganglia b. Posterior columns c. Spinothalamic tracts d. Corticospinal tracts e. Spinocerebellar tracts 17. The shortest life expectancy is associated with which clinical sign in amyotrophic lateral sclerosis? a. Atrophy of the interossei b. Atrophy of the gastrocnemius c. Fasciculations in the lumbrical muscles d. Atrophy of the pectoralis muscles e. Fasciculations in the tongue (18~20 common topic) Male, 35 years old, weight loss, fatigue, afraid of hot, hand tremors 2 months, night suddenly appeared lower extremity flaccid paralysis, emergency check: conscious, 40/80mmHg, heartbeat 108/min, the law is neat, slightly enlarged thyroid. No vascular murmur. 18. Causes the patient to be the direct cause of the double lower limb soft paralysis may be a. Cerebral embolism b. Motor neuron disease c. Myasthenia gravis d. Respiratory base poisoning e. Blood potassium anomaly 19. In order to clear the diagnosis, which inspection item should be the first to carry out? a. Head CT, blood glucose determination b. Electromyography and blood electrolyte determination c. Chest CT and blood anti acetylcholine receptor antibody determination d. Blood gas analysis and blood electrolyte determination e. Determination of serum electrolytes and thyroid function 20. Which of the emergency treatment of the patient should be? a. Spironolactone b. Correcting electrolyte disorder c. Intravenous infusion of potassium chloride and insulin d. Glucocorticoid therapy e. Dehydration and reduction of intracranial pressure Part Two: Fill in the blanks 1. Commonly used antibiotics in the treatment of myasthenia gravis, the anticholinesterase drug has _________ 2. The pathogenesis of myasthenia gravis is related to _________ 3. Myasthenia gravis involved muscle often: _________ 4. The main death cause of myasthenia gravis: _________ 5. Tests for the diagnosis of myasthenia gravis include: _________ _________ 6. Myasthenia gravis is divided into three types: _________ _________ _________ 7. Myasthenia gravis is a major cumulative neuromuscular joint _________ on post synaptic membrane of autoimmune diseases 8. The skeletal muscle fatigue characteristics of Myasthenia gravis is: _________ 9. Type hypo kalmia periodic paralysis of the main features are: _________ _________ 10. The complications of myasthenia gravis: _________ _________ _________ Part Three: Definitions 1. Muscle tone 2. Spasticity 3. Periodic paralysis 4. Myasthenia gravis (MG) 5. Muscular dystrophies Part Four: General questions 1. Grading muscle power 2. What are cerebellar dysfunction signs? 3. Presenting symptoms in myasthenia gravis 4. How to diagnosis myasthenia gravis? 5. The subdivided of muscular dystrophies? Part Five: Case analysis A 30-year-old female plastic surgery resident presents with a 1-month history of intermittent ptosis (droopiness of the eyelids) and fatigue. She has been on call every third night over the past 2 months and has been attributing her fatigue to her hectic call schedule. However she became concerned when she acutely developed ptosis last month after being on call. She went home and went to sleep, and by morning her ptosis had resolved. Her 6-year-old triplets have pointed out to her that she can’t keep up with them when they’re riding their bicycles. She had experienced three more episodes of ptosis over the past month. They all have occurred while she has been post call and have improved by the morning. Today for the first-time she developed ptosis while performing a complicated facial lift. Her attending asked her to stop assisting in surgery and to immediately seek medical evaluation. She has not experienced diplopia, dysarthria, dysphagia, difficulty walking up stairs, difficulty blow-drying her hair, or shortness of breath. She had always been healthy until now. Her neurologic examination is notable for normal mental status and speech. Her cranial nerve examination reveals bilateral ptosis on primary gaze, which worsens with sustained upward gaze for 90 seconds. Extraocular muscles are intact as is her facial strength. Her motor strength is normal with the exception of 4+/5 in the deltoid muscles bilaterally. On repetitive testing of the right iliopsoas muscle fatigability is elicited, which improves after 2 minutes of rest. Her sensory examination and deep tendon reflexes are normal. 1. What is the most likely diagnosis? 2. What is the best test to confirm the diagnosis? 3. What is the next step in therapy? Answers Part One: Choice question 1 to 5: a d c d a 6 to 10: d b e c d 11 to 15: d b a b d 16 to 20: d e e e b Part Two: Fill in the blanks 1. Pyridostigmine bromide 2. The AChR damage induced by auto-antibodies. 3. One side or both sides of the eye muscle paralysis 4. Respiratory muscle involvement 5. Jolly test, anti-drug test 6. Myasthenic crisis, cholinergic crisis, brittle crisis. 7. Acetylcholine receptors 8. Light night heavy in the morning. 9. Slow skeletal muscle recurrent palsy, attack lower serum potassium. 10. Myasthenia gravis crisis, respiratory failure, muscular dystrophy. Part Three: Definitions 1. Muscle tone: For clinical purposes, tone can be defined as the resistance of muscle to passive movement of a joint. Tone depends on the degree of muscle contraction and on the mechanical properties of muscle and connective tissue. 2. Spasticity: Consists of a fincrease in tone that affects different muscle groups to different extents. 3. Periodic paralysis: Periodic paralysis is a group of rare genetic diseases that lead to weakness or paralysis (rarely death) from common triggers such as cold, heat, high carbohydrate meals, not eating, stress or excitement and physical activity of any kind. The underlying mechanism of these diseases are malfunctions in the ion channels in skeletal musclecell membranes that allow electrically charged ions to leak in or out of the muscle cell, causing the cell to depolarize and become unable to move (a channelopathy). 4. Myasthenia gravis (MG): Myasthenia gravis is a neuromuscular disease that leads to fluctuating muscle weakness and fatigue. In the most common cases, muscle weakness is caused by circulating antibodies that block nicotinic acetylcholine receptors at the postsynaptic neuromuscular junction. 5. Muscular dystrophies: The muscular dystrophies are a group of inherited myopathic disorders chare]acterized by progressive muscle weakness and wasting. Part Four: General questions 1. Grading muscle power: 5 normal powers 4 Active movements against resistance and gravity 3 Active movements against gtavity but not resistance 2 Active movements possible only with gtavity eliminated 1 Flicker or trace of contration 0 No contraction 2. What are cerebellar dysfunction signs? Hypotonia Depressed or pendular tendon reflexes Ataxia Gait disorder Imbalance of station Disturbances of eye movement Dysarthria 3. Presenting symptoms in myasthenia gravis Diplopia Ptosis Dysarthria Lower extremity weakness Generalized weakness Dysphagia Upper extremity weakness Masticatory weakness 4. How to diagnosis myasthenia gravis? The diagnosis of myasthenia gravis can generally be confirmed by the benefit that follows administration of anticholinesterase drugs; the power of affected muscles is influenced at a dose that has no effect on normal muscles and slight, if any, effect on muscles weakened by other causes. 5. The subdivided of muscular dystrophies: Duchenne dystrophy Becker dystrophy Gmery-Dreifuss muscular dystrophy Limb-Girdle dystrophy Facioscapulohumeral dystrophy Distal myopathy Ocular dystrophy Oculopharyngeal dystrophy Paraspinal dystrophy Part Five: Case analysis Most likely diagnosis: Myasthenia gravis Best confirmatory test: Antiacetylcholine receptor antibodies Next step in therapy: Acetylcholinesterase inhibitors (pyridostigmine) and immunosuppression