300 - AIIMS NOVEMBER 2009 MCQS with Answers - part 15

71q: A person has restricted eye movements in all direction and ptosis but no squint or diplopia. What could be the probable diagnosis?
a. Myasthenia gravis
b. Thyroid myopathy
c. CDEM
d. Multiple cranial nerve palsies

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72q: A patient was diagnosed with Kostmann syndrome. What is the treatment?
a. Anti-thymocyte globulin + cyclosporin
b. Anti-thymocyte globulin + cyclosporin + GM-CSF
c. G-CSF
d. GM-CSF

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*Congenital neutropenia (Kostmann syndrome) is a disease with mutations in the granulocyte colony-stimulating factor (G-CSF) receptor and, often, neutrophil elastase that may evolve into AML.

73q: NARP syndrome is ?
a. A glycogen storage disorder
b. A lipid storage disorder
c. A lysosomal storage disorder
d. Mitochondrial disorder

answer d.Mitochondrial disorder.

*The syndrome of Neuropathy, Ataxia and Retinitis Pigmentosa (NARP)is clinically heterogenous, but it is often characterised by a combination of sensory-motor neuropathy, cerebellar ataxia and night blindness.
*A maternally inherited condition, the NARP syndrome is associated with a mutation in the mitochondrial DNA(mtDNA) gene, MTATP6, coding for the subunit ATPase6.

74q: All are used in the treatment of visceral leishmaniasis except:
a. Sitamaquine
b. Paromomycin
c. Hydroxychloroquine
d. miltefosine

answer c. Hydroxychloroquine

75q: Stability of the ankle joint is maintained by all except
a. Cruciate ligaments
b. Collateral ligaments
c. Tendons of muscles passing the joint
d. close apposition of bones

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