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Ataxic Disorders- Part 2
See all quizzes of Ataxic Disorders at here:
1 Which of the following is a CAG repeat disease ?
A. Machado-Joseph Disease (MJD)
B. Huntington’s disease
C. Dentatorubral-pallidoluysian atrophy (DRPLA)
D. All of the above
2 Which of the following about increase in number of “nucleotide
repeats” is false ?
A. Length correlates with severity of disease
B. Repeat length increases from one generation to next
C. Leads to anticipation
D. None of the above
3 Dynamic mutation refers to ?
A. Decreasingly severe phenotype in next generation
B. Increasingly severe phenotype in next generation
C. Decreasingly severe genotype in next generation
D. Increasingly severe genotype in next generation
4 Which of the following Trinucleotide Repeat Disorders has
autosomal recessive inheritance ?
A. Spinocerebellar ataxia type 1 (SCA1)
B. Machado Joseph disease (MJD)
C. Dentorubral pallidoluysiane atrophy (DRPLA)
D. Friedreich ataxia (FRDA1)
5 Which of the following was also called olivopontocerebellar
atrophy ?
A. Spinocerebellar ataxia 1
B. Spinocerebellar ataxia 2
C. Spinocerebellar ataxia 3
D. Spinocerebellar ataxia 6
6 Which clinical phenotype of spinocerebellar ataxia has been
described in patients from India ?
A. Spinocerebellar ataxia 1
B. Spinocerebellar ataxia 2
C. Spinocerebellar ataxia 6
D. Spinocerebellar ataxia 7
7 Which out of the following is the most common inherited
autosomal dominant ataxia ?
A. Spinocerebellar ataxia 1
B. Spinocerebellar ataxia 2
C. Spinocerebellar ataxia 3
D. Spinocerebellar ataxia 6
8 Most common form of inherited ataxia is ?
A. Machado-Joseph Disease (MJD)
B. Friedreich’s Ataxia
C. Dentatorubropallidoluysian atrophy (DRPLA)
D. Episodic ataxia
9 Amyotrophic lateral sclerosis – parkinsonism – dystonia type
presentation is in which clinical type of MJD ?
A. Type I MJD
B. Type II MJD
C. Type III MJD
D. None of the above
10 Ataxic type presentation is seen in which type of MJD ?
A. Type I MJD
B. Type II MJD
C. Type III MJD
D. None of the above
11 Ataxic-amyotrophic type presentation is seen in which clinical
type of MJD ?
A. Type I MJD
B. Type II MJD
C. Type III MJD
D. None of the above
12 Which is the most common clinical type of MJD ?
A. Type I MJD
B. Type II MJD
C. Type III MJD
D. All of the above
13 The mean age of onset of symptoms in MJD is ?
A. 5 years
B. 10 years
C. 15 years
D. 25 years
14 Patients retain full intellectual function in which clinical type of
MJD ?
A. Type I MJD
B. Type II MJD
C. Type III MJD
D. All of the above
15Which of the following is spared in Machado-Joseph Disease
(MJD) ?
A. Inferior olives
B. Corpus striatum
C. Pars compacta of substantia nigra
D. Dentate nucleus of cerebellum
16 Which of the following spinocerebellar ataxias (SCAs) has pure
cerebellar presentation ?
A. SCA1
B. SCA2
C. SCA3
D. SCA5
17 ‘Retinal pigmentary degeneration’ occurs in which of the following
spinocerebellar ataxias (SCAs) ?
A. SCA1
B. SCA2
C. Machado-Joseph disease
D. SCA7
18 The color blindness in SCA7 is for which colour ?
A. Red-yellow
B. Green-yellow
C. Blue-yellow
D. Any of the above
19 Haw River syndrome relates to which of the following ?
A. Machado-Joseph Disease
B. Dentatorubropallidoluysian atrophy (DRPLA)
C. Friedreich’s Ataxia
D. Episodic Ataxia
20 Gene related to Dentatorubropallidoluysian Atrophy is ?
A. Dentatin
B. Quadrupin
C. Simplin
D. Atrophin 1
.
21 Which of the following spinocerebellar ataxias (SCAs) has
presentation similar to Huntington disease (HD) ?
A. SCA1
B. SCA2
C. SCA3
D. SCA17
22 Which of the following is not a potassium ion channelopathy ?
A. Episodic ataxia-1
B. Episodic ataxia-2
C. Benign neonatal familial convulsions
D. Jervell & Lange-Nielsen syndrome
23 Which of the following is not a sodium ion channelopathy ?
A. Paramyotonia congenita
B. Hyperkalemic periodic paralysis
C. Hypokalemic periodic paralysis
D. Generalized epilepsy with febrile convulsions plus
24 Which of the following is not a calcium ion channelopathy ?
A. Episodic ataxia-2
B. Spinocerebellar ataxia-6
C. Familial hemiplegic migraine
D. Autosomal dominant progressive deafness