Myopathies Flashcards by Marco Cicero

Elderly gentleman, unable to raise arms above shoulder level (proximal
weakness), triceps and biceps reflex extinguished, finger flexor reflex present. It is:
a. myopathy, EMG required

a. myopathy, EMG required

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Which enzyme is defective in McArdle’s disease?
a. acid phosphatase
b. myophosphorylase
c. Phosphofructokinase
d. glc-6-phosphatase

b. myophosphorylase

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Which muscular dystrophy is a ‘triplet’ disease?
a. Duchenne
b. Emery-dreifussova
c. myotonic
d. oculopharyngeal

c. myotonic
d. oculopharyngeal

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Polymyositis is not treated with:
a. corticosteroids (methylprednisolone)
b. interferon beta
c. methotrexate
d. human immunoglobulins

b. interferon beta

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Acid maltase deficiency causes:
a. neuropathy
b. encephalopathy
c. myopathy
d. all of the above

c. myopathy

Pompe’s disease

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What is not mitochondrial hereditary myopathy?
a. Centronuclear myopathy.

a. Centronuclear myopathy.

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Which muscles are not affected in polymyositis?
a. shoulder muscles
b. facial muscles
c. pelvic muscles
d. bulbar muscles

b. facial muscles
d. bulbar muscles

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A patient with a rash on her face and wasting in her arms and legs is likely
to have:
a. myasthenia gravis
b. dermatomyositis
c. rhabdomyolysis due to chronic alcoholism

b. dermatomyositis

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What can trigger paroxysmal hypokalaemic paralysis?
a. pie
b. beef soup
c. grilled squid
d. lettuce

a. pie

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What is not characteristic of hypokalaemic periodic paralysis:
a. paralysis lasting more than 24h

a. paralysis lasting more than 24h

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Which enzyme is elevated in Duchenne muscular dystrophy?
a. creatine phosphokinase (CK)

a. creatine phosphokinase (CK)

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Muscle diseases most commonly affect:
a. foot extensors
b. small muscles of the arms
c. facial muscles
d. muscles of the shoulder and pelvic girdle

d. muscles of the shoulder and pelvic girdle

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In facioscapulohumeral muscular dystrophy, strength is preserved:
a. biceps
b. deltoida
c. facial muscles
d. serratus anterior

b. deltoida

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Emery-Dreyfuss dystrophy is characterised by:
a. spinal and elbow contractures
b. cataracts
c. distal muscle atrophy
d. normal CK values

a. spinal and elbow contractures

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What’s not typical of myotonic dystrophy:
a. distal muscle group involvement
b. frontal baldness
c. delayed relaxation during muscle contraction
d. elbow contractures

d. elbow contractures

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Congenital myopathies are not:
a. nonmalignant myopathy
b. centronuclear myopathy
c. congenital myotonic dystrophy
d. myopathy with adhesions

c. congenital myotonic dystrophy

This is actually hereditary

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Which characteristic applies most to myopathies?
a. muscle pain
b. proximal muscle involvement

b. proximal muscle involvement

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Which tests are not usually used to diagnose muscular dystrophies?
a. ECG
b. lumbar puncture
c. blood count

b. lumbar puncture

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It has the worst prognosis:
a. Becker muscular dystrophy
b. Duchenne muscular dystrophy
c. myotonic dystrophy

b. Duchenne muscular dystrophy

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What is not the case for the patient in the picture? (some sort of Dystrophy patient)
a. has a normal ECG
b. has endocrine disorders
c. has cataracts
d. his disease is hereditary

a. has a normal ECG

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Hypertrophy in Duchenne muscular dystrophy:
a. Pseudohypertrophy of the calves due to accumulation of fat and
connective tissue.

a. Pseudohypertrophy of the calves due to accumulation of fat and
connective tissue.

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Which of the following is not a neuromuscular junction disease?
a. myasthenia gravis
b. botulism
c. Lambert - Eaton’s syndrome
d. congenital acetylcholine receptor deficiency
e. Becker muscular dystrophy

e. Becker muscular dystrophy

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How is Duchenne inherited?
a. X-linked recessive

a. X-linked recessive

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What is the first test for suspected Duchenne in a 3-year-old?
a. Genetic testing.

a. Genetic testing.

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We have a pregnant woman who is about to give birth to a baby boy. Her brother has Duchenne muscular dystrophy. Genetic analysis has shown no deletion... What do we say to the pregnant woman? a. Duchenne muscular dystrophy is inherited differently and the son is not at risk. b. A boy is more likely to have Duchenne muscular dystrophy, but we don't know the genetic test. c. The boy is more likely to have Duchenne muscular dystrophy and the mother can be offered indirect genetic testing

c. The boy is more likely to have Duchenne muscular dystrophy and the mother can be offered indirect genetic testing

The worst prognosis for a child with which dystrophy? a. Duchenne dystrophy b. Becker's dystrophy c. congenital dystrophy

a. Duchenne dystrophy

Four pictures of one child picking himself up off the ground and climbing on his thighs. It's about: a. Gowers' sign.

a. Gowers' sign.

Description of a 51-year-old woman, weakness in the arms, bradycardia. What is it most likely? a. Duchenne muscular dystrophy b. Becker muscular dystrophy c. myotonic dystrophy d. ALS

c. myotonic dystrophy

Patient cannot abduct the arms at the shoulder (proximal weakness), biceps, triceps and brachioradialis reflexes extinguished, peripheral reflexes normal, normal finger movements, no sensory disturbances. What is wrong? a. polyneuropathy b. plexopathy c. radiculopathy d. myopathy

d. myopathy

Hypokalaemic periodic paralysis is triggered by: a. Sunday beef soup recipe by Valentina Novak b. steak tartare prepared by Luka Novak c. Original Bled slice d. Organic banana from Ecuador

c. Original Bled slice

In Duchenne muscular dystrophy, it is defective: a. actin b. myosin c. dystrophin

c. dystrophin

The patient is unable to abduct the upper limb above 90°, the biceps and triceps reflexes are extinguished and the strength of the distal arm muscles is unaffected. There are no other peculiarities in the status. What is the most likely diagnosis? a. radiculopathy b. plexopathy c. myopathy d. F-M contact

c. myopathy

In Duchenne muscular dystrophy, hypertrophy occurs: a. Everywhere b. nowhere c. only in the shins d. in the thighs only e. only in shoulders

c. only in the shins

What helps us least in diagnosing myopathy: a. disability in the family b. time of onset of symptoms c. pattern of affected d. Sensory disturbances

d. Sensory disturbances

A shot of the patient's hand clenched into a fist and unable to relax immediately. She was able to do so after some time. What is it? a. Myotonia b. dystrophy

a. Myotonia

Who will die in young adulthood? a. The boy from Duchenne.

a. The boy from Duchenne.

The gold standard of myopathies: a. EMG b. muscle biopsy c. serum CK determination

b. muscle biopsy

A 3-year-old suspected of Duchenne dystrophy, which diagnostic method should be used? a. Genetic testing

a. Genetic testing

What is not characteristic of acid maltase deficiency? a. Motor peripheral nerves are most affected b. The disease is treated by replacing the enzyme c. Respiratory failure may occur d. Proximal muscle weakness e. The childhood form is called Pompe disease

a. Motor peripheral nerves are most affected

What is not part of the clinical picture of myotonic dystrophy a. ptosis b. ECG changes c. frontal baldness d. diabetes e. all of the above

e. all of the above

Which test would confirm myositis? a. with EMG b. by determining serum CK levels c. with muscle biopsy d. through genetic testing

c. with muscle biopsy

Which enzyme is defective in Pompe disease: a. acid maltase b. myophosphorylase c. alpha-galactosidase

a. acid maltase

Which disease is treated with enzyme replacement therapy? a. McArdle's disease b. Pompe disease c. Duchenne muscular dystrophy d. spinal muscular atrophy

b. Pompe disease

Which antibodies cause Lambert-Eaton syndrome? a. anti-AChR on the postsynaptic membrane b. against voltage-dependent Ca channels on the presynaptic membrane c. against voltage-dependent Ca channels on the postsynaptic membrane

b. against voltage-dependent Ca channels on the presynaptic membrane

Lambert-Eaton syndrome is: a. 50% of cases are paraneoplastic b. Presynaptic calcium channel antibodies are present c. presence of antibodies against presynaptic Ach channels

b. Presynaptic calcium channel antibodies are present

Male, 69 years old, muscle weakness, fatigue, ptosis of the left eye, swallowing disorder, nasal speech, most probably lesion: a. Neuromuscular contact b. multiple sclerosis c. peripheral neuropathy of the Guillain-Barré type d. Cerebellar nerve nuclei III, IV, IX, X

a. Neuromuscular contact

The standard on contact M-M disease - what does it indicate? a. Multiple elicitation of a response.

a. Multiple elicitation of a response.

Side effects of anticholinesterases include: a. dry mouth b. visual impairment c. closure d. cramps and diarrhoea

d. cramps and diarrhoea

Not so for Lambert-Eaton syndrome: a. anti-VGCC antibodies b. sometimes paraneoplastic c. aggravation with prolonged irritation of the muscle d. is rare

c. aggravation with prolonged irritation of the muscle

A 27-year-old clerk has 6 months of double images in the evening, accompanied by drooping eyelids. On examination, bilateral ptosis is noted, which worsens after the patient stares upwards for a minute. Eyeball movement is restricted. The rest of the examination is normal. Your diagnosis: a. mitochondrial myopathy b. oculopharyngeal muscular dystrophy c. CIDP d. myasthenia gravis

d. myasthenia gravis

Myasthenia gravis - circle the incorrect statement: a. 10% have antibodies to ACh receptors in their blood

a. 10% have antibodies to ACh receptors in their blood

In the diagnosis of myasthenia gravis we will do: a. EMG b. anti-AChR antibody test c. edrophonium test d. all of the above

d. all of the above

Who can have MG: a. patient with an enlarged thymus b. with antibodies against ACh receptors c. patient responding to endrophony d. a patient suffering from muscle weakness and fatigue e. all of the above

e. all of the above

Which medicines can worsen the symptoms of myasthenia gravis: a. aminoglycoside antibiotics b. dopamine antagonists c. Serotonin uptake inhibitors d. noradrenaline uptake inhibitors

a. aminoglycoside antibiotics

Which muscles are most commonly affected in myasthenia gravis? a. proximal b. Neck c. backs d. distal

a. proximal

Myasthenia gravis is characterised by: a. wave 3 b. wave 9 and that there is an increase in potential, or first a decrease then an increase... c. reducing potential

c. reducing potential

In myasthenia gravis, the first symptom is most often: a. limb muscle disability b. Dysphagia c. eye muscle involvement

c. eye muscle involvement

Which tumour is associated with myasthenia gravis? a. thyroid carcinoma b. carcinoma of the lung c. lymphoma d. benign thymoma e. malignant thymoma

d. benign thymoma

Myasthenia gravis syndrome is not characteristic: a. outer eye muscle involvement b. muscle fatigue c. improving symptoms after an AChE inhibitor d. distal muscle group involvement

a. outer eye muscle involvement d. distal muscle group involvement

Generalised MG, no impairment found: a. eye muscles b. respiratory muscles c. distal limb muscles

c. distal limb muscles

Myasthenia gravis also affects: a. respiratory muscles b. distal limb muscles c. bulbarne

a. respiratory muscles

What does an electroencephalogram show in myasthenia gravis? a. Amplified 3rd wave and low 9th wave b. low 3rd wave and amplified 9th wave

a. Amplified 3rd wave and low 9th wave

. The most sensitive test for myasthenia gravis is: a. micro EMG b. refrigeration test c. Antibodies to AChR d. edrophonium test

a. micro EMG

Myasthenia gravis, what you give for fast action: a. AchE inhibitor

a. AchE inhibitor

Which imaging do you do at MG? a. CT of the thorax

a. CT of the thorax

Which is the most sensitive test for diagnosing myasthenia gravis? a. anti-AChR antibodies b. multiple elicitation of responses c. edrophonium test d. refrigeration test

b. multiple elicitation of responses

In myasthenia gravis, we will see: a. lively reflections b. weakened reflexes, which are strengthened after several repetitions c. normal reflexes that dry up after several repetitions

c. normal reflexes that dry up after several repetitions

One statistical question related to myasthenia gravis (circle the wrong one): a. has a bipolar age distribution b. more than 10% have a familial inheritance c. up to 10% have nicotinic receptor antibodies d. something gender-related

c. up to 10% have nicotinic receptor antibodies

The diagnostic method of choice for myasthenia gravis is: a. Sensory conductivity test b. motor conduction test c. repetitive nerve stimulation

c. repetitive nerve stimulation

Sometimes cancer is detected because of paraneoplastic syndrome. How often? a. 1% b. 30% c. 60% d. 99%

a. 1%

Which antibodies are found in myasthenia gravis? a. against acetylcholine receptors b. against aquaporin 4 c. anti-ganglioside d. against voltage-dependent calcium channels

a. against acetylcholine receptors

What is not used in the treatment of myasthenia gravis? a. acetylcholinesterase inhibitors b. corticosteroids c. azathioprine d. plasmapheresis e. IVIG f. we use all of the above

f. we use all of the above

A 21-year-old maid complains of occasional double vision in the afternoon. She tires more quickly when doing physical tasks. On examination: her left eyelid is drooping and she occasionally notices this herself. Strength is good, lively symmetrical myotatic reflexes are present. What is wrong with her? a. Myasthenia gravis

a. Myasthenia gravis

A smoker who drinks 6 beers a day says he finds it difficult to shave in the morning, says his hand is more awkward, then gets better towards the end of the day and continues to do physical work all day. At the end of the day he says that he gets tired and his wife notices that he has ptosis of one eye several times. On examination, you find that the reflexes have withered: a. myasthenia gravis syndrome b. peripheral polyneuropathy c. myasthenia gravis

a. myasthenia gravis syndrome

Which medicine is not used to treat myasthenia gravis? a. pyridostigmine b. glatiramer acetate c. methylprednisolone d. azathioprine e. all of the above

b. glatiramer acetate

What we are least likely to see in a person with myasthenia gravis: a. proximal arm weakness b. distal arm weakness c. weakness of the bulbar muscles d. weakness of the respiratory muscles e. weakness of the muscles that move the eyeballs

b. distal arm weakness

In myasthenia gravis syndrome, we are looking for antibodies against: a. nicotinic AChR b. Muscarine AChR c. against muscle fibres d. against all of the above

d. against all of the above