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Biochemistry Nutrition & Digestion d01b21e0

Pantothenate Kinase associated neurodegeneration is:

A
Wiison's disease
B
Hallervorden-Spatz syndrome
C
McLeod syndrome
D
Lesch Nyhan syndrome
High-Yield Explanation
Ans. B. Hallervorden-Spatz syndromePantothenate kinase associated neuro degeneration (PKAN) (formerly Hallervorden-Spatz syndrome)Rare autosomal recessive neuro degenerative disorderChorea, dystonia, parkinsonian features, pyramidal tract features and MRMRI-decreased T2 signal in the globus pallidus and substantianigra, "eye of the tiger" sign (hyperintense area within the hypointense area)Sometimes acanthocytosisNeuropathologic examination indicates excessive accumulation of iron-containing pigments in the globuspallidus and substantianigra.Similar disorders are grouped as neurodegeneration with brain iron accumulation (NBIA).

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