[MCQ] Peripheral Neuropathy - Part 2

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Peripheral Neuropathy- Part 2

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Question 1

Which of the following proteins is restricted to CNS myelin ?

A	P0 protein
B	PMP22
C	Proteolipid protein (PLP)
D	Myelin-associated glycoprotein (MAG)

Question 1 Explanation:

Protein restricted to CNS myelin are proteolipid protein (PLP) and myelin oligodendrocyte glycoprotein (MOG) while that in PNS myelin are P0, PMP22 and Cx32. Proteins present in both CNS & PNS are GM1, myelin-associated glycoprotein (MAG) and GQ1b.

Question 2

Which of the following is false about Schmidt-Lanterman incisures ?

A	Noncompacted regions of myelin
B	Contains cytoplasm
C	Transport metabolic substances across myelin sheath
D	None of the above

Question 2 Explanation:

Schwann cells form myelin sheath around peripheral nerve axons as either highly compacted membrane or noncompacted cytoplasmic regions (Schmidt-Lanterman incisures or SLI). SLI are important for transport of metabolic substances across myelin sheath and for metabolic maintenance & longitudinal growth of sheath.

Question 3

Palpably thickened nerves in CMT1 is due to increase in ?

A	Hyaline
B	Mucopolysaccharides
C	Collagen
D	Glycogen

Question 3 Explanation:

In CMT1, increased collagen between layers of supernumerary Schwann cells leads to palpably thickened nerves

Question 4

Which of the following is false about myelin ?

A	It speeds impulse conduction
B	Single oligodendrocyte ensheaths multiple CNS axons
C	Each Schwann cell myelinates a single PNS axon
D	It is a protein-rich material

Question 4 Explanation:

Myelin is a lipid-rich material formed by a spiraling process of the membrane of myelinating cell around the axon.

Question 5

Pelizaeus Merzbacher disease is caused by mutations in gene for ?

A	Proteolipid protein (PLP)
B	P0 protein
C	Myelin oligodendrocyte glycoprotein (MOG)
D	Myelin basic protein (MBP)

Question 5 Explanation:

Pelizaeus-Merzbacher syndrome is a dysmyelinating X-linked allelic disorder of CNS, caused by mutations in gene for proteolipid protein (PLP) that promotes extracellular compaction between adjacent myelin lamellae.

Question 6

Which of the following is called tomaculous neuropathy ?

A	DéJerine-Sottas Syndrome (DSS)
B	Hereditary neuropathy with liability to pressure palsies
C	Roussy-LéVy Syndrome
D	Congenital Hypomyelinating Neuropathy (CHN)

Question 6 Explanation:

Autosomal dominant disorder Hereditary neuropathy with liability to pressure palsies (HNPP) is also called tomaculous neuropathy. Tomaculae are sausage-shaped bodies that indicate segmental demyelination

Question 7

Which of the following is false about Charcot-Marie-Tooth Disease (CMT) ?

A	Heritable neuromuscular disorder
B	Chronic distal sensory and motor neuropathy
C	CMT does not reduce the life span
D	None of the above

Question 7 Explanation:

CMT is the most common heritable neuromuscular disorder. It is a chronic distal sensory & motor neuropathy. Pes cavus & hammer toes indicate that neuropathy dates from early life. CMT does not reduce life span & rarely involves respiratory muscles.

Question 8

Which of the following is the most common type of hereditary neuropathy ?

A	Hereditary Neuralgic Amyotrophy (HNA)
B	Hereditary Neuropathy with Liability to Pressure Palsies (HNPP)
C	Charcot-Marie-Tooth (CMT) disease
D	Hereditary Sensory and Autonomic Neuropathy (HSAN)

Question 8 Explanation:

Charcot-Marie-Tooth (CMT) disease is a syndrome of several genetically distinct disorders. It is the most common type of hereditary neuropathy

Question 9

Various subtypes of CMT are classified according to ?

A	Predominant pathology
B	Nerve conduction velocities
C	Inheritance pattern
D	All of the above

Question 9 Explanation:

Various subtypes of CMT are classified according to the nerve conduction velocities and predominant pathology (demyelination or axonal degeneration), inheritance pattern (autosomal dominant, recessive, or X-linked), and the specific mutated genes.

Question 10

In CMT1, motor conduction velocities in the arms are slowed to ?

A	< 38 m/sec.
B	< 48 m/sec.
C	< 58 m/sec.
D	< 68 m/sec.

Question 10 Explanation:

By definition, motor conduction velocities in the arms are slowed to < 38 m/sec in CMT1 and are greater than 38 m/sec in CMT2.

Question 11

Transmission of Charcot-Marie-Tooth (CMT) neuropathy diseases is ?

A	Autosomal dominant
B	Autosomal recessive
C	X-linked
D	All of the above

Question 11 Explanation:

CMT is usually transmitted as autosomal dominant trait, as X-linked-dominant CMT in ~10%. Rare autosomal recessive forms (CMT4) have an early onset & are more severe than dominant types

Question 12

Inheritance of CMT1 is ?

A	Autosomal dominant
B	Autosomal recessive
C	X-linked
D	Any of the above

Question 12 Explanation:

Inheritance of CMT1 is autosomal dominant

Question 13

Which of the following about Charcot–Marie–Tooth disease is false ?

A	CMT1 and 2 are autosomal dominant
B	CMT1 is a demyelinating polyneuropathy
C	CMT2 is an axonal polyneuropathy
D	None of the above

Question 13 Explanation:

Charcot–Marie–Tooth disease type 1 is most common inherited demyelinating sensorimotor neuropathy, followed by type 2. Both are autosomal dominant. Type 1 is a demyelinating polyneuropathy, whereas type 2 is an axonal sensory polyneuropathy.

Question 14

Which of the following is an autosomal recessive neuropathy ?

A	CMT1
B	CMT2
C	CMT3
D	CMT4

Question 14 Explanation:

CMT1, CMT2 and CMT3 have autosomal dominant inheritance (with a few exceptions). CMT4 (genetically heterogenic) is an autosomal recessive neuropathy

Question 15

The ratio of occurrence of CMT1:CMT2 is approximately ?

A	1 : 1
B	2 : 1
C	3 : 1
D	4 : 1

Question 15 Explanation:

CMT1 is the most common form of hereditary neuropathy. The ratio of CMT1:CMT2 is ~2:1.

Question 16

CMT1 is which form of CMT ?

A	Demyelinating
B	Axonal
C	Intermediate
D	Any of the above

Question 17

CMT2 is which form of CMT ?

A	Demyelinating
B	Axonal
C	Intermediate
D	Any of the above

Question 17 Explanation:

Demyelinating forms of CMT are classified as CMT1, and axonal forms as CMT2. Nerve conduction velocities (NCVs) intermediate between CMT1 & CMT2 are classified as “intermediate CMT” and most of these are X-linked.

Question 18

Which of the following manifestation prompts suspicion of CMT1 ?

A	Bed sore
B	Numbness or tingling
C	Footdrop
D	Frequent falls

Question 18 Explanation:

CMT1 usually present in the first to third decade of life with distal leg weakness (footdrop). People with CMT generally do not complain of numbness or tingling, which helps in distinguishing CMT from acquired forms of neuropathy in which sensory symptoms usually predominate

Question 19

Which of the following manifestation prompts suspicion of CMT1 ?

A	Reduced sensation to all modalities
B	Reduced muscle stretch reflexes
C	Atrophy of muscles below knee
D	All of the above

Question 19 Explanation:

In CMT1, reduced sensation to all modalities is apparent on examination. Muscle stretch reflexes are absent or reduced. Atrophy of muscles below knee (particularly anterior compartment), leads to inverted champagne bottle legs.

Question 20

In CMT1, characteristic nerve biopsy appearance is ?

A	Mulberry appearance
B	Cabbage appearance
C	Potato peel appearance
D	Onion bulb appearance

Question 20 Explanation:

Nerve biopsies show evidence of repeated demyelination & remyelination. Supernumerary, concentrically arranged, Schwann cells attempting to remyelinate axons give a characteristic “onion bulb” appearance

Question 21

The most common form of CMT1 is ?

A	1A subtype
B	2A subtype
C	2B subtype
D	2C subtype

Question 21 Explanation:

CMT1A is the most common subtype of CMT1, representing 70% of cases.

Question 22

CMT 1A subtype is caused by ?

A	Duplication of PMP22 gene
B	Deletion in PMP22
C	Translocation in PMP22
D	All of the above

Question 22 Explanation:

CMT1A is caused by a 1.5-megabase (Mb) duplication within peripheral myelin protein-22 (PMP- 22) gene containing chromosome 17p11.2-12 resulting in patients having three copies of the PMP- 22 gene rather than two.

Question 23

CMT2 is caused by mutations for which gene ?

A	Alpha-galactosidase gene
B	ATP-binding cassette transporter 1 (ABC1) gene
C	Mitofusin 2 (MFN2) gene
D	Transthyretin (TTR) gene

Question 23 Explanation:

Most common cause of CMT2 is a mutation in the gene for mitofusin 2 (MFN2) which is localized to the outer mitochondrial membrane, where it regulates the mitochondrial network architecture by fusion of mitochondria.

Question 24

DéJerine-Sottas disease is also called ?

A	CMT type 1
B	CMT type 2
C	CMT type 3
D	CMT type 4

Question 24 Explanation:

CMT3 was originally described by Dejerine and Sottas as a hereditary demyelinating sensorimotor polyneuropathy presenting in infancy or early childhood

Question 25

DéJerine-Sottas syndrome is similar to ?

A	CMT2A
B	CMT2B
C	Congenital hypomyelinating neuropathy (CHN)
D	Roussy-LéVy Syndrome

Question 25 Explanation:

DéJerine-Sottas syndrome & Congenital hypomyelinating neuropathy are indistinguishable. Nerve biopsy can distinguish them

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See all quizzes of Peripheral Neuropathy here:

1 Which of the following proteins is restricted to CNS myelin ?
A. P0 protein
B. PMP22
C. Proteolipid protein (PLP)
D. Myelin-associated glycoprotein (MAG)

2 Which of the following is false about Schmidt-Lanterman incisures ?
A. Noncompacted regions of myelin
B. Contains cytoplasm
C. Transport metabolic substances across myelin sheath
D. None of the above

3 Palpably thickened nerves in CMT1 is due to increase in ?
A. Hyaline
B. Mucopolysaccharides
C. Collagen
D. Glycogen

4 Which of the following is false about myelin ?
A. It speeds impulse conduction
B. Single oligodendrocyte ensheaths multiple CNS axons
C. Each Schwann cell myelinates a single PNS axon
D. It is a protein-rich material

5 Pelizaeus Merzbacher disease is caused by mutations in gene for ?
A. Proteolipid protein (PLP)
B. P0 protein
C. Myelin oligodendrocyte glycoprotein (MOG)
D. Myelin basic protein (MBP)

6 Which of the following is called tomaculous neuropathy ?
A. DéJerine-Sottas Syndrome (DSS)
B. Hereditary neuropathy with liability to pressure palsies
C. Roussy-LéVy Syndrome
D. Congenital Hypomyelinating Neuropathy (CHN)

7 Which of the following is false about Charcot-Marie-Tooth Disease
(CMT) ?
A. Heritable neuromuscular disorder
B. Chronic distal sensory and motor neuropathy
C. CMT does not reduce the life span
D. None of the above

8 Which of the following is the most common type of hereditary
neuropathy ?
A. Hereditary Neuralgic Amyotrophy (HNA)
B. Hereditary Neuropathy with Liability to Pressure Palsies
(HNPP)
C. Charcot-Marie-Tooth (CMT) disease
D. Hereditary Sensory and Autonomic Neuropathy (HSAN)

9 Various subtypes of CMT are classified according to ?
Harrison’s 18th Ed. 3452
A. Predominant pathology
B. Nerve conduction velocities
C. Inheritance pattern
D. All of the above

10 In CMT1, motor conduction velocities in the arms are slowed to ?
A. < 38 m/sec.
B. < 48 m/sec.
C. < 58 m/sec.
D. < 68 m/sec.

11 Transmission of Charcot-Marie-Tooth (CMT) neuropathy diseases
is ?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked
D. All of the above

12 Inheritance of CMT1 is ?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked
D. Any of the above

13 Which of the following about Charcot–Marie–Tooth disease is
false ?
A. CMT1 and 2 are autosomal dominant
B. CMT1 is a demyelinating polyneuropathy
C. CMT2 is an axonal polyneuropathy
D. None of the above

14 Which of the following is an autosomal recessive neuropathy ?
Harrison’s 18th Ed. 3452
A. CMT1
B. CMT2
C. CMT3
D. CMT4

15 The ratio of occurrence of CMT1:CMT2 is approximately ?
A. 1 : 1
B. 2 : 1
C. 3 : 1
D. 4 : 1

16 CMT1 is which form of CMT ?
A. Demyelinating
B. Axonal
C. Intermediate
D. Any of the above
17 CMT2 is which form of CMT ?
A. Demyelinating
B. Axonal
C. Intermediate
D. Any of the above

18 Which of the following manifestation prompts suspicion of CMT1 ?
A. Bed sore
B. Numbness or tingling
C. Footdrop
D. Frequent falls

19 Which of the following manifestation prompts suspicion of CMT1 ?
A. Reduced sensation to all modalities
B. Reduced muscle stretch reflexes
C. Atrophy of muscles below knee
D. All of the above
.
20 In CMT1, characteristic nerve biopsy appearance is ?
Harrison’s 18th Ed. 3454
A. Mulberry appearance
B. Cabbage appearance
C. Potato peel appearance
D. Onion bulb appearance

21 The most common form of CMT1 is ?
A. 1A subtype
B. 2A subtype
C. 2B subtype
D. 2C subtype

22 CMT 1A subtype is caused by ?
A. Duplication of PMP22 gene
B. Deletion in PMP22
C. Translocation in PMP22
D. All of the above

23 Charcot-Marie-Tooth disease (CMT) type 1B is caused by mutations
in gene for ?
A. Proteolipid protein (PLP)
B. P0 protein
C. Myelin oligodendrocyte glycoprotein (MOG)
D. Myelin basic protein (MBP)

24 CMT2 is caused by mutations for which gene ?
A. Alpha-galactosidase gene
B. ATP-binding cassette transporter 1 (ABC1) gene
C. Mitofusin 2 (MFN2) gene
D. Transthyretin (TTR) gene

25 DéJerine-Sottas disease is also called ?
A. CMT type 1
B. CMT type 2
C. CMT type 3
D. CMT type 4

26 DéJerine-Sottas syndrome is similar to ?
A. CMT2A
B. CMT2B
C. Congenital hypomyelinating neuropathy (CHN)
D. Roussy-LéVy Syndrome

Peripheral Neuropathy

[MCQ] Peripheral Neuropathy – Part 2

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Peripheral Neuropathy- Part 2

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