Myopathies Flashcards
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
Which enzyme is defective in McArdle’s disease?
a. acid phosphatase
b. myophosphorylase
c. Phosphofructokinase
d. glc-6-phosphatase
b. myophosphorylase
Which muscular dystrophy is a ‘triplet’ disease?
a. Duchenne
b. Emery-dreifussova
c. myotonic
d. oculopharyngeal
c. myotonic
d. oculopharyngeal
Polymyositis is not treated with:
a. corticosteroids (methylprednisolone)
b. interferon beta
c. methotrexate
d. human immunoglobulins
b. interferon beta
Acid maltase deficiency causes:
a. neuropathy
b. encephalopathy
c. myopathy
d. all of the above
c. myopathy
Pompe’s disease
What is not mitochondrial hereditary myopathy?
a. Centronuclear myopathy.
a. Centronuclear myopathy.
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
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
What can trigger paroxysmal hypokalaemic paralysis?
a. pie
b. beef soup
c. grilled squid
d. lettuce
a. pie
What is not characteristic of hypokalaemic periodic paralysis:
a. paralysis lasting more than 24h
a. paralysis lasting more than 24h
Which enzyme is elevated in Duchenne muscular dystrophy?
a. creatine phosphokinase (CK)
a. creatine phosphokinase (CK)
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
In facioscapulohumeral muscular dystrophy, strength is preserved:
a. biceps
b. deltoida
c. facial muscles
d. serratus anterior
b. deltoida
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
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
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
Which characteristic applies most to myopathies?
a. muscle pain
b. proximal muscle involvement
b. proximal muscle involvement
Which tests are not usually used to diagnose muscular dystrophies?
a. ECG
b. lumbar puncture
c. blood count
b. lumbar puncture
It has the worst prognosis:
a. Becker muscular dystrophy
b. Duchenne muscular dystrophy
c. myotonic dystrophy
b. Duchenne muscular dystrophy
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
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.
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
How is Duchenne inherited?
a. X-linked recessive
a. X-linked recessive
What is the first test for suspected Duchenne in a 3-year-old?
a. Genetic testing.
a. Genetic testing.
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