[MCQ] Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases

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Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases

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Question 1

Lou Gehrig’s disease is the other name of ?

A	Amyotrophic lateral sclerosis
B	Myasthenia gravis
C	Multiple sclerosis
D	Subacute combined degeneration of cord

Question 1 Explanation:

ALS is often called Lou Gehrig’s disease after Lou Gehrig (1903-1941), a hall-of-fame baseball player for New York Yankees who suffered with ALS. People in England and Australia call ALS as Motor Neurone Disease (MND). The French refer to it as Maladie de Charcot, after the French doctor Jean-Martin Charcot, who first wrote about ALS in 1869.

Question 2

Acute sporadic motor neuron diseases include ?

A	Poliomyelitis
B	Herpes zoster
C	Coxsackie virus
D	All of the above

Question 2 Explanation:

Acute sporadic motor neuron diseases include Poliomyelitis, Herpes zoster and Coxsackie virus.

Question 3

Chronic sporadic motor neuron disease with predominant involvement of upper motor neurons is ?

A	Amyotrophic lateral sclerosis
B	Multifocal motor neuropathy with conduction block
C	Motor neuropathy with paraproteinemia
D	Primary lateral sclerosis

Question 3 Explanation:

Chronic sporadic MND with predominant involvement of UMN is primary lateral sclerosis.

Question 4

Chronic sporadic motor neuron disease with predominant involvement of lower motor neurons are ?

A	Multifocal motor neuropathy with conduction block
B	Motor neuropathy with paraproteinemia or cancer
C	Motor-predominant peripheral neuropathies
D	All of the above

Question 4 Explanation:

In Amyotrophic lateral sclerosis, both upper & lower motor neurons are involved. In Primary lateral sclerosis, predominantly upper motor neurons are involved. In Multifocal motor neuropathy with conduction block, Motor neuropathy with paraproteinemia or cancer and Motor-predominant peripheral neuropathies, predominantly lower motor neurons are affected.

Question 5

Diseases that affect only upper motor neurons innervating the brainstem and spinal cord are ?

A	Pseudobulbar palsy
B	Primary lateral sclerosis (PLS)
C	Familial spastic paraplegia (FSP)
D	All of the above

Question 5 Explanation:

Pseudobulbar palsy, primary lateral sclerosis (PLS), and familial spastic paraplegia (FSP) affect only upper motor neurons innervating the brainstem and spinal cord

Question 6

In amyotrophic lateral sclerosis, the structures that are left intact include ?

A	Sensory apparatus
B	Centers for control & coordination of movement
C	Centers for cognitive processes
D	All of the above

Question 6 Explanation:

A remarkable feature of ALS is the selectivity of neuronal cell death. Entire sensory apparatus, regulatory mechanisms for the control and coordination of movement and the components of brain that are needed for cognitive processes remain intact.

Question 7

In amyotrophic lateral sclerosis, the structures that are left intact include ?

A	Motor neurons for ocular motility
B	Parasympathetic neurons in sacral spinal cord
C	Sensory apparatus
D	All of the above

Question 7 Explanation:

In ALS, within motor system, there is selectivity of involvement. Motor neurons required for ocular motility remain unaffected, as do the parasympathetic neurons in the sacral spinal cord (the nucleus of Onufrowicz, or Onuf) that innervate the sphincters of the bowel and bladder.

Question 8

In ALS, the cause leading to death is ?

A	Infections
B	Depression and suicide
C	Respiratory paralysis
D	Cardiovascular event

Question 8 Explanation:

In ALS, the cause leading to death is respiratory paralysis

Question 9

Which of the following is not a feature of ALS ?

A	Absence of pain or of sensory changes
B	Normal bowel and bladder function
C	Normal roentgenographic studies of spine
D	Raised proteins in cerebrospinal fluid (CSF)

Question 9 Explanation:

Absence of pain or of sensory changes, normal bowel and bladder function, normal roentgenographic studies of the spine, and normal cerebrospinal fluid (CSF) all favor ALS.

Question 10

Which of the following antibiotics has neuroprotective properties ?

A	Azithromycin
B	Minocycline
C	Spiramycin
D	Vancomycin

Question 10 Explanation:

Minocycline is a second-generation tetracycline with neuroprotective properties. Minocycline inhibits ischemia-induced up-regulation of nitric oxide synthase, caspase 1, and reactive microgliosis. Neuroprotection by minocycline has been observed in Huntington’s disease, ALS, brain injury, Parkinson’s disease, and multiple sclerosis

Question 11

Multifocal motor neuropathy with conduction block (MMCB) evolves in association with which of the following ?

A	Tuberculosis
B	Lymphoma
C	Syphilis
D	All of the above

Question 11 Explanation:

MMCB is a diffuse, lower motor axonal neuropathy mimicking ALS which sometimes evolves in association with lymphoma or multiple myeloma.

Question 12

Intraneuronal inclusions “Bunina bodies” are a pathognomonic feature of ?

A	Motor neuron disease
B	Multiple sclerosis
C	Subacute combined degeneration of cord
D	Myasthenia gravis

Question 13

Differential diagnosis of amyotrophic lateral sclerosis is ?

A	Compression of cervical spinal cord
B	Thyrotoxicosis
C	Chronic lead poisoning
D	All of the above

Question 13 Explanation:

D/D of ALS includes cervical spinal cord compression, cervicomedullary junction tumors, cervical or foramen magnum tumors, cervical spondylosis, chronic lead poisoning, thyrotoxicosis, hexosaminidase A, or alpha-glucosidase deficiency.

Question 14

Riluzole - a glutamate antagonist is useful in treatment of ?

A	Amyotrophic lateral sclerosis
B	Multiple sclerosis
C	Subacute combined degeneration of cord
D	Myasthenia gravis

Question 14 Explanation:

Riluzole is approved for ALS. Riluzole reduces excitotoxicity by diminishing glutamate release.

Question 15

Which of the following is false for Kennedy’s Disease ?

A	X linked spinobulbar muscular atrophy
B	Lower motor neuron disorder
C	Gynecomastia and reduced fertility
D	None of the above

Question 15 Explanation:

Azoospermia & male-factor infertility occurs in association with mild loss of function mutations in androgen receptor. Trinucleotide (CAG) repeat expansion, from a mean of 22 repeats to greater than 40 repeats, within a highly polymorphic region of androgen receptor is associated with spinal & bulbar muscular atrophy (Kennedy disease). These patients may show evidence of partial androgen insensitivity in adolescence or adulthood (gynecomastia).

Question 16

Werdnig-Hoffmann Disease is the name given to ?

A	SMA I
B	SMA II
C	SMA III
D	None of the above

Question 17

Kugelberg-Welander disease is the name given to ?

A	SMA I
B	SMA II
C	SMA III
D	None of the above

Question 18

Which out of the following carries the worst prognosis ?

A	SMA I
B	SMA II
C	SMA III
D	None of the above

Question 18 Explanation:

Infantile SMA or SMA I is known as Werdnig-Hoffmann disease with earliest onset & most rapidly fatal course. Chronic childhood SMA (SMA II) begins later in childhood & evolves with slowly progressive course. Juvenile SMA (SMA III or Kugelberg-Welander disease) manifests in late childhood & has a slow course. EP studies distinguish SMA III from myopathic syndromes

Question 19

Which of the following is false about spinal muscular atrophy ?

A	Genetic lower motor neuron disease
B	Early onset
C	Locus on chromosome 5
D	None of the above

Question 20

Which of the following is false about spinal muscular atrophy ?

A	Extensive loss of large motor neurons
B	Evidence of denervation atrophy on muscle biopsy
C	Electrophysiologic evidence of denervation
D	None of the above

Question 21

Which of the following is false about ‘Multifocal Motor Neuropathy with Conduction Block’ (MMCB) ?

A	Elevated serum titers of mono- & polyclonal antibodies to ganglioside GM1
B	No corticospinal tract signs
C	Respond to intravenous immunoglobulin therapy
D	None of the above

Question 22

Which of the following is true for Fazio-Londe syndrome ?

A	ALS variant
B	Juvenile onset
C	Involves musculature innervated by brainstem mainly
D	All of the above

Question 22 Explanation:

Fazio-Londe syndrome is an ALS variant of juvenile onset that involves mainly the musculature innervated by the brainstem.

Question 23

Which of the following is false for Familial Spastic Paraplegia (FSP) in adults ?

A	X linked transmission
B	Presents in third or fourth decade
C	Progressive spastic weakness in lower extremities
D	Long survival

Question 23 Explanation:

In its pure form, most adult-onset FSP are transmitted as an autosomal dominant trait. FSP has long survival because respiratory function is spared.

Question 24

Which of the following is false for Familial Spastic Paraplegia (FSP) ?

A	Sexual function preserved
B	Spastin and atlastin gene mutations common
C	Degeneration of corticospinal tracts
D	None of the above

Question 24 Explanation:

In FSP, sexual function tends to be preserved. Gene implicated is spastin & atlastin in dominantly inherited and childhood-onset dominant FSP respectively. Degeneration of corticospinal tracts occurs in FSP, most severe at more caudal levels in spinal cord.

Question 25

Which of the following is a disorder of CNS myelin ?

A	Fragile X syndrome
B	Pelizaeus-Merzbacher syndrome
C	Prader-Willi syndrome
D	Rett syndrome

Question 25 Explanation:

Pelizaeus-Merzbacher disease is a widespread disorder of CNS myelin.

Question 26

Protein restricted to CNS myelin is which of the following ?

A	Proteolipid protein (PLP)
B	P0
C	PMP22
D	GM1

Question 27

Protein restricted to PNS myelin is which of the following ?

A	Proteolipid protein (PLP)
B	P0
C	Myelin oligodendrocyte glycoprotein (MOG)
D	GM1

Question 28

Protein in both CNS & PNS myelin is ?

A	Proteolipid protein (PLP)
B	P0
C	Myelin oligodendrocyte glycoprotein (MOG)
D	GM1

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See all quizzes of Ataxic Disorders at here:

1 Lou Gehrig’s disease is the other name of ?
A. Amyotrophic lateral sclerosis
B. Myasthenia gravis
C. Multiple sclerosis
D. Subacute combined degeneration of cord

2 Acute sporadic motor neuron diseases include ?
A. Poliomyelitis
B. Herpes zoster
C. Coxsackie virus
D. All of the above

3 Chronic sporadic motor neuron disease with predominant
involvement of upper motor neurons is ?
A. Amyotrophic lateral sclerosis
B. Multifocal motor neuropathy with conduction block
C. Motor neuropathy with paraproteinemia
D. Primary lateral sclerosis

4 Chronic sporadic motor neuron disease with predominant
involvement of lower motor neurons are ?
A. Multifocal motor neuropathy with conduction block
B. Motor neuropathy with paraproteinemia or cancer
C. Motor-predominant peripheral neuropathies
D. All of the above

5 Diseases that affect only upper motor neurons innervating the
brainstem and spinal cord are ?
A. Pseudobulbar palsy
B. Primary lateral sclerosis (PLS)
C. Familial spastic paraplegia (FSP)
D. All of the above

7 In amyotrophic lateral sclerosis, the structures that are left intact
include ?
A. Sensory apparatus
B. Centers for control & coordination of movement
C. Centers for cognitive processes
D. All of the above

8 In amyotrophic lateral sclerosis, the structures that are left intact
include ?
A. Motor neurons for ocular motility
B. Parasympathetic neurons in sacral spinal cord
C. Sensory apparatus
D. All of the above

9 In ALS, the cause leading to death is ?
A. Infections
B. Depression and suicide
C. Respiratory paralysis
D. Cardiovascular event

10 Which of the following is not a feature of ALS ?
A. Absence of pain or of sensory changes
B. Normal bowel and bladder function
C. Normal roentgenographic studies of spine
D. Raised proteins in cerebrospinal fluid (CSF)

11 Which of the following antibiotics has neuroprotective properties ?
A. Azithromycin
B. Minocycline
C. Spiramycin
D. Vancomycin

12 Multifocal motor neuropathy with conduction block (MMCB)
evolves in association with which of the following ?
A. Tuberculosis
B. Lymphoma
C. Syphilis
D. All of the above

13 Intraneuronal inclusions “Bunina bodies” are a pathognomonic
feature of ?
A. Motor neuron disease
B. Multiple sclerosis
C. Subacute combined degeneration of cord
D. Myasthenia gravis
14 Differential diagnosis of amyotrophic lateral sclerosis is ?
A. Compression of cervical spinal cord
B. Thyrotoxicosis
C. Chronic lead poisoning
D. All of the above

15 Riluzole – a glutamate antagonist is useful in treatment of ?
A. Amyotrophic lateral sclerosis
B. Multiple sclerosis
C. Subacute combined degeneration of cord
D. Myasthenia gravis

16 Which of the following is false for Kennedy’s Disease ?
A. X linked spinobulbar muscular atrophy
B. Lower motor neuron disorder
C. Gynecomastia and reduced fertility
D. None of the above

17 Werdnig-Hoffmann Disease is the name given to ?
A. SMA I
B. SMA II
C. SMA III
D. None of the above
18 Kugelberg-Welander disease is the name given to ?
A. SMA I
B. SMA II
C. SMA III
D. None of the above
19 Which out of the following carries the worst prognosis ?
A. SMA I
B. SMA II
C. SMA III
D. None of the above

20 Which of the following is false about spinal muscular atrophy ?
A. Genetic lower motor neuron disease
B. Early onset
C. Locus on chromosome 5
D. None of the above
21 Which of the following is false about spinal muscular atrophy ?
A. Extensive loss of large motor neurons
B. Evidence of denervation atrophy on muscle biopsy
C. Electrophysiologic evidence of denervation
D. None of the above
22 Which of the following is false about ‘Multifocal Motor Neuropathy
with Conduction Block’ (MMCB) ?
A. Elevated serum titers of mono- & polyclonal antibodies to
ganglioside GM1
B. No corticospinal tract signs
C. Respond to intravenous immunoglobulin therapy
D. None of the above
23 Which of the following is true for Fazio-Londe syndrome ?
A. ALS variant
B. Juvenile onset
C. Involves musculature innervated by brainstem mainly
D. All of the above

24 Which of the following is false for Familial Spastic Paraplegia
(FSP) in adults ?
A. X linked transmission
B. Presents in third or fourth decade
C. Progressive spastic weakness in lower extremities
D. Long survival

25Which of the following is false for Familial Spastic Paraplegia
(FSP) ?
A. Sexual function preserved
B. Spastin and atlastin gene mutations common
C. Degeneration of corticospinal tracts
D. None of the above

26 Which of the following is a disorder of CNS myelin ?
A. Fragile X syndrome
B. Pelizaeus-Merzbacher syndrome
C. Prader-Willi syndrome
D. Rett syndrome

27 Protein restricted to CNS myelin is which of the following ?
A. Proteolipid protein (PLP)
B. P0
C. PMP22
D. GM1
28 Protein restricted to PNS myelin is which of the following ?
A. Proteolipid protein (PLP)
B. P0
C. Myelin oligodendrocyte glycoprotein (MOG)
D. GM1
29 Protein in both CNS & PNS myelin is ?
A. Proteolipid protein (PLP)
B. P0
C. Myelin oligodendrocyte glycoprotein (MOG)
D. GM1

Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases

[MCQ] Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases

I. Start the exam by click the “Start” button

Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases

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