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What is Pantothenate Kinase associated neurodegeneration is: and explain in detail?
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).