[MCQ] Muscular Dystrophies- Part 1

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Muscular Dystrophies- Part 1

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Question 1

Clinical findings of a myopathy include all except ?

A	Proximal limb weakness
B	Symmetric limb weakness
C	Depressed reflexes
D	Preserved sensation

Question 1 Explanation:

Clinical findings of a myopathy are proximal, symmetric limb weakness (arms or legs) with preserved reflexes and sensation.

Question 2

Which of the following is not a cause of intermittent weakness of muscles ?

A	Myasthenia gravis
B	Polymyositis
C	Paramyotonia congenita
D	Carnitine palmitoyltransferase deficiency

Question 2 Explanation:

Disorders causing intermittent muscle weakness include myasthenia gravis, periodic paralyses (hypokalemic, hyperkalemic, and paramyotonia congenita), and metabolic energy deficiencies of glycolysis (myophosphorylase deficiency), fatty acid utilization (carnitine palmitoyltransferase deficiency), and some mitochondrial myopathies

Question 3

Which of the following is accompanied by myoglobinuria ?

A	Myasthenia gravis
B	Polymyositis
C	Paramyotonia congenita
D	Carnitine palmitoyltransferase deficiency

Question 4

Which of the following can have a presentation of “dropped head syndrome” ?

A	Myasthenia gravis
B	Amyotrophic lateral sclerosis
C	Hyperparathyroidism
D	All of the above

Question 4 Explanation:

Dropped head syndrome indicates selective neck extensor muscle weakness. Neuromuscular diseases associated with this pattern of weakness include myasthenia gravis, amyotrophic lateral sclerosis, late-onset nemaline myopathy, hyperparathyroidism, focal myositis, and some forms of inclusion body myopathy.

Question 5

Pathologic fatigability occurs in which of the following ?

A	Disorders of neuromuscular transmission
B	Disorders of defects in glycolysis
C	Chronic myopathies
D	All of the above

Question 5 Explanation:

Pathologic fatigability refers to inability to maintain or sustain a force. It occurs in disorders of neuromuscular transmission and in disorders altering energy production (glycolysis, lipid metabolism, or mitochondrial energy production), chronic myopathies.

Question 6

Drugs that cause true myalgia include ?

A	Cimetidine
B	Labetalol
C	Statins
D	All of the above

Question 6 Explanation:

Drugs that cause true myalgia include Cimetidine, Cocaine, Cyclosporine, Danazol, Emetine, Gold, Heroin, Labetalol, Methadone, d-Penicillamine, Statins, l-Tryptophan, Zidovudine.

Question 7

Which of the following is a painful muscle condition ?

A	Paramyotonia congenita
B	Amyotrophic lateral sclerosis
C	Fibromyalgia
D	All of the above

Question 7 Explanation:

Painful muscle conditions not associated with muscle weakness include fibromyalgia and polymyalgia rheumatica

Question 8

Which of the following is associated with polymyalgia rheumatica ?

A	Takayasu’s arteritis
B	Henoch-Schönlein purpura
C	Temporal arteritis
D	Granulomatosis with polyangiitis (Wegener’s)

Question 8 Explanation:

Giant cell arteritis, also referred to as cranial arteritis or temporal arteritis, is an inflammatory disorder of medium- and large-sized arteries, may accompany polymyalgia rheumatica.

Question 9

Muscle cramps often occur in which of the following neurogenic disorders ?

A	Motor neuron disease
B	Radiculopathies
C	Polyneuropathies
D	All of the above

Question 9 Explanation:

Muscle cramps often occur in neurogenic disorders, especially motor neuron disease, radiculopathies, and polyneuropathies. They are not a feature of most primary muscle diseases

Question 10

Which of the following enzymes is not found in muscles ?

A	Creatine kinase (CK)
B	Aldolase
C	Lactic dehydrogenase (LDH)
D	Gamma-glutamyl transferase (GGT)

Question 10 Explanation:

Creatine kinase (CK), aspartate aminotransferase (AST), alanine aminotransferase (ALT), aldolase, and lactic dehydrogenase (LDH) are enzymes sharing an origin in both muscle and liver. Gammaglutamyl transferase (GGT) has a liver origin and is not found in muscle.

Question 11

Nationality of Guillaume Benjamin Amand Duchenne de Boulogne was ?

A	Polish
B	French
C	German
D	Danish

Question 12

Who discovered dystrophin gene ?

A	Little
B	Gowers
C	Kunkel
D	Meryon

Question 13

‘Gowers’ maneuver’ is diagnostic of ?

A	Duchenne Muscular Dystrophy (DMD)
B	Limb-girdle Muscular Dystrophy
C	Emery-Dreifuss Muscular Dystrophy
D	Facioscapulohumeral Muscular Dystrophy

Question 14

Patients with Duchenne dystrophy are typically in a wheelchair by the age of ?

A	8
B	10
C	12
D	14

Question 14 Explanation:

Patients with Duchenne dystrophy are typically in wheelchair by age of 12.

Question 15

Gowers’ sign is due to weakness of which group of muscles ?

A	Knee extensor muscles
B	Hip muscles
C	Anterior compartment of leg muscles
D	Posterior compartment of leg muscles

Question 15 Explanation:

Gowers’ sign refers to the inability to get up from the floor without climbing up the extremities. Weakness is in the hip, thigh, and trunk muscles

Question 16

In DMD, which of the following muscle groups are preferrentially involved ?

A	Distal limb muscles
B	Neck flexors
C	Neck extensors
D	Anterior abdominal muscles

Question 16 Explanation:

Loss of muscle strength is progressive, with predilection for proximal limb muscles and neck flexors.

Question 17

Which of the following about DMD is false ?

A	Due to mutation in dystrophin gene
B	Dystrophin is the largest identified human gene
C	Dystrophin is on X chromosome
D	Mostly due to gene duplication

Question 17 Explanation:

DMD is caused by a mutation of gene encoding 427-kDa dystrophin protein on the inner surface of sarcolemma. Dystrophin gene is one of the largest identified human genes (>2000 kb) on the short arm of X chromosome (Xp21). Most common gene mutation is a deletion. Less often, Duchenne’s dystrophy is caused by a gene duplication or point mutation

Question 18

Which of the following is an uncommon cause of death in Duchenne’s muscular dystrophy ?

A	Pulmonary infection
B	Aspiration of food
C	Acute gastric dilation
D	Cardiac cause (cardiomyopathy)

Question 18 Explanation:

By age 16–18 years, patients of DMD are predisposed to serious, sometimes fatal pulmonary infections. Other causes of death include aspiration of food and acute gastric dilation. Cardiac

Question 19

Which of the following statements about serum CK levels in DMD is false ?

A	Invariably elevated
B	Serum CK levels are abnormal at birth
C	Decline late in the disease
D	None of the above

Question 19 Explanation:

In DMD, serum CK levels are invariably elevated (20 to 100 times). Levels are abnormal at birth but decline late in the disease because of inactivity & loss of muscle mass.

Question 20

Dystrophin binds to which of the following in sarcolemma ?

A	Laminin
B	Alpha-dystroglycan
C	F-actin
D	Caveolin-3

Question 20 Explanation:

Dystrophin is part of a large complex of sarcolemmal proteins and glycoproteins. Dystrophin binds to F-actin at its amino terminus and to beta-dystroglycan at the carboxyl terminus.

Question 21

In essence, the key defect in DMD due to abnormal dystrophin protein is ?

A	Physical weakening of the sarcolemma
B	Disruption of Golgi apparatus
C	Abnormal mitochondria
D	Abnormal extracellular matrix (ECM)

Question 21 Explanation:

Dystrophin-glycoprotein complex confer stability to the sarcolemma. Disruption of dystrophinglycoprotein complexes weakens sarcolemma, causing membrane tears and a cascade of events leading to muscle fiber necrosis and eventually muscular dystrophy.

Question 22

Which of the following significantly slows progression of Duchenne’s dystrophy ?

A	Azathioprine
B	Glucocorticoids
C	Cyclosporine
D	IVIg

Question 22 Explanation:

Glucocorticoids (prednisone 0.75 mg/kg per day), significantly slows progression of Duchenne’s dystrophy for up to 3 years.

Question 23

Becker’s Muscular Dystrophy resembles which of the following muscular dystrophy ?

A	Duchenne’s muscular dystrophy
B	Limb-Girdle Muscular Dystrophy
C	Emery-Dreifuss Muscular Dystrophy
D	Myotonic Dystrophy

Question 23 Explanation:

Becker’s muscular dystrophy is a less severe and 10 times less frequent form of X-linked recessive Duchenne’s muscular dystrophy.

Question 24

Most patients with Becker’s muscular dystrophy first experience difficulties at what age ?

A	Birth
B	1 - 2 years
C	3 - 5 years
D	5 - 15 years

Question 24 Explanation:

Most patients with Becker’s dystrophy first experience difficulties between ages 5 and 15 years.

Question 25

Calpains are Ca++ dependent ?

A	Cysteine proteases
B	Arginine proteases
C	Methionine proteases
D	Leucine proteases

Question 26

Tissue-specific calpains have been implicated in ?

A	Diabetes
B	Multiple sclerosis
C	Limb-girdle muscular dystrophy type 2A (LGMD2A)
D	All of the above

Question 27

Which of the following statements about Calpain is false ?

A	Calpain activity is regulated by calpastatin
B	Calpains are involved in apoptosis
C	Calpain is a cytoplasmic protease
D	None of the above

Question 28

LGMD2A is caused by gene mutations in ?

A	Calpain-1
B	Calpain-2
C	Calpain-3
D	Calpain-4

Question 29

LGMD2B is due to defect in ?

A	Dysferlin
B	Emerin
C	Telethonin
D	Titin

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See all quizzes of Muscular Dystrophies- Part 1 here:

1 Clinical findings of a myopathy include all except ?
A. Proximal limb weakness
B. Symmetric limb weakness
C. Depressed reflexes
D. Preserved sensation

2 Which of the following is not a cause of intermittent weakness of
muscles ?
A. Myasthenia gravis
B. Polymyositis
C. Paramyotonia congenita
D. Carnitine palmitoyltransferase deficiency

3 Which of the following is accompanied by myoglobinuria ?
A. Myasthenia gravis
B. Polymyositis
C. Paramyotonia congenita
D. Carnitine palmitoyltransferase deficiency

4 Which of the following can have a presentation of “dropped head
syndrome” ?
A. Myasthenia gravis
B. Amyotrophic lateral sclerosis
C. Hyperparathyroidism
D. All of the above

5 Pathologic fatigability occurs in which of the following ?
A. Disorders of neuromuscular transmission
B. Disorders of defects in glycolysis
C. Chronic myopathies
D. All of the above

6 Drugs that cause true myalgia include ?
A. Cimetidine
B. Labetalol
C. Statins
D. All of the above

7 Which of the following is a painful muscle condition ?
A. Paramyotonia congenita
B. Amyotrophic lateral sclerosis
C. Fibromyalgia
D. All of the above

8 Which of the following is associated with polymyalgia
rheumatica ?
A. Takayasu’s arteritis
B. Henoch-Schönlein purpura
C. Temporal arteritis
D. Granulomatosis with polyangiitis (Wegener’s)

9 Muscle cramps often occur in which of the following neurogenic
disorders ?
A. Motor neuron disease
B. Radiculopathies
C. Polyneuropathies
D. All of the above

10 Which of the following enzymes is not found in muscles ?
A. Creatine kinase (CK)
B. Aldolase
C. Lactic dehydrogenase (LDH)
D. Gamma-glutamyl transferase (GGT)

11 Nationality of Guillaume Benjamin Amand Duchenne de Boulogne
was ?
A. Polish
B. French
C. German
D. Danish
12 Who discovered dystrophin gene ?
A. Little
B. Gowers
C. Kunkel
D. Meryon
13 Duchenne Muscular dystrophy is present at ?
A. Birth
B. 1 – 2 years
C. 3 – 5 years
D. 5 – 7 years

14 ‘Gowers’ maneuver’ is diagnostic of ?
A. Duchenne Muscular Dystrophy (DMD)
B. Limb-girdle Muscular Dystrophy
C. Emery-Dreifuss Muscular Dystrophy
D. Facioscapulohumeral Muscular Dystrophy
15 Patients with Duchenne dystrophy are typically in a wheelchair by
the age of ?
A. 8
B. 10
C. 12
D. 14

16 Gowers’ sign is due to weakness of which group of muscles ?
A. Knee extensor muscles
B. Hip muscles
C. Anterior compartment of leg muscles
D. Posterior compartment of leg muscles

17 In DMD, which of the following muscle groups are preferrentially
involved ?
A. Distal limb muscles
B. Neck flexors
C. Neck extensors
D. Anterior abdominal muscles

18 Which of the following about DMD is false ?
A. Due to mutation in dystrophin gene
B. Dystrophin is the largest identified human gene
C. Dystrophin is on X chromosome
D. Mostly due to gene duplication

19 Which of the following is an uncommon cause of death in
Duchenne’s muscular dystrophy ?
A. Pulmonary infection
B. Aspiration of food
C. Acute gastric dilation
D. Cardiac cause (cardiomyopathy)

20 Which of the following statements about serum CK levels in DMD
is false ?
A. Invariably elevated
B. Serum CK levels are abnormal at birth
C. Decline late in the disease
D. None of the above

21 Dystrophin binds to which of the following in sarcolemma ?
A. Laminin
B. Alpha-dystroglycan
C. F-actin
D. Caveolin-3

22 In essence, the key defect in DMD due to abnormal dystrophin
protein is ?
A. Physical weakening of the sarcolemma
B. Disruption of Golgi apparatus
C. Abnormal mitochondria
D. Abnormal extracellular matrix (ECM)

23 Which of the following significantly slows progression of
Duchenne’s dystrophy ?
A. Azathioprine
B. Glucocorticoids
C. Cyclosporine
D. IVIg

24 Becker’s Muscular Dystrophy resembles which of the following
muscular dystrophy ?
A. Duchenne’s muscular dystrophy
B. Limb-Girdle Muscular Dystrophy
C. Emery-Dreifuss Muscular Dystrophy
D. Myotonic Dystrophy

25 Most patients with Becker’s muscular dystrophy first experience
difficulties at what age ?
A. Birth
B. 1 – 2 years
C. 3 – 5 years
D. 5 – 15 years
26 Calpains are Ca++ dependent ?
A. Cysteine proteases
B. Arginine proteases
C. Methionine proteases
D. Leucine proteases
27 Tissue-specific calpains have been implicated in ?
A. Diabetes
B. Multiple sclerosis
C. Limb-girdle muscular dystrophy type 2A (LGMD2A)
D. All of the above
28 Which of the following statements about Calpain is false ?
A. Calpain activity is regulated by calpastatin
B. Calpains are involved in apoptosis
C. Calpain is a cytoplasmic protease
D. None of the above
29 LGMD2A is caused by gene mutations in ?
A. Calpain-1
B. Calpain-2
C. Calpain-3
D. Calpain-4
30 LGMD2B is due to defect in ? 0
A. Dysferlin
B. Emerin
C. Telethonin
D. Titin

Muscular Dystrophies

[MCQ] Muscular Dystrophies- Part 1

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Muscular Dystrophies- Part 1

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[MCQ] Muscular Dystrophies- Part 2

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