Pediatric Annals

SELF-ASSESSMENT QUIZ: Muscle Diseases in Children

Abstract

1 . In order to arrive at a correct diagnosis in children with muscle disease, what five steps should always be taken?

A. __________

B. __________

C. __________

D. __________

E. __________

2. An infant is very hypotonic following birth. Which of the following could be considered among the possible diagnoses?

A. Cerebral hypoxia

B. Cerebral palsy

C. Duchenne's muscular dystrophy

D. Spinal muscular atrophy

3. A new mother reports that her baby moved very little while in utero. When born, the baby was very "floppy." Physical examination demonstrated a lack of deep tendon relexes. What would be a tentative diagnosis?

A. Cerebral palsy __________

B. Hypothyroidism __________

C. Spinal muscular atrophy __________

D. Myasthenia gravis __________

4. Which of the following is correct? Of the tests for muscle enzymes, a very high CPK is important because:

A. It usually excludes neurogenic disease __________

B. It is a common finding in Duchenne's muscular dystrophy __________

C. It is not uncommon in muscular atrophy __________

D. It is common in myasthenia gravis syndromes __________

5. A seven-year-old child has had general malaise, fatigue, a low-grade fever, and increasing weakness for four to five weeks. Concurrent with these symptoms there has appeared a dull reddish rash over the knees, elbows, knuckles, and the upper face. Which of the following would you consider a most probable diagnosis?

A. Spinal muscular atrophy - __________

B. Lupus erythematosus __________

C. Dermatomyositis __________

D. Duchenne's muscular dystrophy __________

6. Which of the following steps would be most important in confirming your diagnosis?

A. Electromyography __________

B. Muscle chemistry __________

C. Muscle biopsy __________

D. LE preparations __________

7. An experienced neurologist has completed his studies on a child with muscular weakness. Enzyme studies show an increase of serum CPK activity. In spite of this, he makes a diagnosis of neurogenic muscle disease. On which of the following did he base his diagnosis?

A. A study of biopsy specimen __________

B Characteristic electromyographic studies __________

C. Genetic studies __________

8. In diagnostic studies on a "floppy" newborn, when should CPK enzyme studies be undertaken?

A. Immediately __________

B. After 1 week __________

C. After 6 to 1 0 weeks __________

9. Duchenne's muscular dystrophy is occasionally transmitted as an autosomal recessive trait, although a carrier state cannot be uniformly detected. In what way may evidence of a carrier state in the parents be gained?

A. Through a careful family history __________

B. Through muscle biopsies of the parents __________

C. Through electromyographic studies of both parents __________

D. Through CPK enzyme studies of the parents __________

10. A newborn has generalized hypotonia, weakness, arthrogryposis of the legs, ptosis, and cataracts. Which of the following would be the cause?

A. Cerebral palsy __________

B. Duchenne's muscular dystrophy __________

C. Spinal muscular atrophy __________

D Myotonic dystrophy __________

11. With the generalized weakness caused by Duchenne's muscular dystrophy, what Is the most common cause of death?

A. Congestive heart failure __________

B. Pneumonia __________

C Gastrointestinal problems __________

Answers to Quiz: Muscle Diseases in Children

1. A. Interpretation of signs and symptoms.

B. Genetic study of child's family.

C. Biochemical studies.

D. Electrodiagnostic studies.

E. Muscle biopsy.

2. A, B, D.

3. C.

4. A.

5. C.

6. A.

7. B.

8. C.

9. A.

10. D.

11. B.…

1 . In order to arrive at a correct diagnosis in children with muscle disease, what five steps should always be taken?

A. __________

B. __________

C. __________

D. __________

E. __________

2. An infant is very hypotonic following birth. Which of the following could be considered among the possible diagnoses?

A. Cerebral hypoxia

B. Cerebral palsy

C. Duchenne's muscular dystrophy

D. Spinal muscular atrophy

3. A new mother reports that her baby moved very little while in utero. When born, the baby was very "floppy." Physical examination demonstrated a lack of deep tendon relexes. What would be a tentative diagnosis?

A. Cerebral palsy __________

B. Hypothyroidism __________

C. Spinal muscular atrophy __________

D. Myasthenia gravis __________

4. Which of the following is correct? Of the tests for muscle enzymes, a very high CPK is important because:

A. It usually excludes neurogenic disease __________

B. It is a common finding in Duchenne's muscular dystrophy __________

C. It is not uncommon in muscular atrophy __________

D. It is common in myasthenia gravis syndromes __________

5. A seven-year-old child has had general malaise, fatigue, a low-grade fever, and increasing weakness for four to five weeks. Concurrent with these symptoms there has appeared a dull reddish rash over the knees, elbows, knuckles, and the upper face. Which of the following would you consider a most probable diagnosis?

A. Spinal muscular atrophy - __________

B. Lupus erythematosus __________

C. Dermatomyositis __________

D. Duchenne's muscular dystrophy __________

6. Which of the following steps would be most important in confirming your diagnosis?

A. Electromyography __________

B. Muscle chemistry __________

C. Muscle biopsy __________

D. LE preparations __________

7. An experienced neurologist has completed his studies on a child with muscular weakness. Enzyme studies show an increase of serum CPK activity. In spite of this, he makes a diagnosis of neurogenic muscle disease. On which of the following did he base his diagnosis?

A. A study of biopsy specimen __________

B Characteristic electromyographic studies __________

C. Genetic studies __________

8. In diagnostic studies on a "floppy" newborn, when should CPK enzyme studies be undertaken?

A. Immediately __________

B. After 1 week __________

C. After 6 to 1 0 weeks __________

9. Duchenne's muscular dystrophy is occasionally transmitted as an autosomal recessive trait, although a carrier state cannot be uniformly detected. In what way may evidence of a carrier state in the parents be gained?

A. Through a careful family history __________

B. Through muscle biopsies of the parents __________

C. Through electromyographic studies of both parents __________

D. Through CPK enzyme studies of the parents __________

10. A newborn has generalized hypotonia, weakness, arthrogryposis of the legs, ptosis, and cataracts. Which of the following would be the cause?

A. Cerebral palsy __________

B. Duchenne's muscular dystrophy __________

C. Spinal muscular atrophy __________

D Myotonic dystrophy __________

11. With the generalized weakness caused by Duchenne's muscular dystrophy, what Is the most common cause of death?

A. Congestive heart failure __________

B. Pneumonia __________

C Gastrointestinal problems __________

Answers to Quiz: Muscle Diseases in Children

1. A. Interpretation of signs and symptoms.

B. Genetic study of child's family.

C. Biochemical studies.

D. Electrodiagnostic studies.

E. Muscle biopsy.

2. A, B, D.

3. C.

4. A.

5. C.

6. A.

7. B.

8. C.

9. A.

10. D.

11. B.

10.3928/0090-4481-19770301-10

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