Pantothenate Kinase Associated Neurodegeneration: A Case Report

  • S Rahar Senior Resident, Department of Ophthalmology, Safdarjung Hospital and Vardhaman Mahaveer Medical College, New Delhi
  • A Chaudhary Junior Specialist, Department of Paediatrics, Sri Kalyan Hospital, Sikar, Rajasthan
Keywords: Eye of tiger sign, NBIA, Neuroregeneration, Iron Accumulation, Hallevorden Spatz Syndrome

Abstract

Pantothenate kinase-associated neurodegeneration (PKAN) is a form of neurodegeneration with brain iron accumulation (NBIA), formerly called Hallervorden-Spatz syndrome. PKAN is the first described inborn error of coenzyme A metabolism. PKAN encompasses two clinical subtypes, classic and atypical. We here report an eight year girl diagnosed as classic PKAN. We also review the literature about PKAN.

Key words: Eye of tiger sign; NBIA; Neuroregeneration; Iron Accumulation; Hallevorden Spatz Syndrome

DOI: http://dx.doi.org/10.3126/jnps.v32i1.5333  

J. Nepal Paediatr. Soc. Vol.32(1) 2012 90-92

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Abstract
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Published
2012-02-29
How to Cite
Rahar, S., & Chaudhary, A. (2012). Pantothenate Kinase Associated Neurodegeneration: A Case Report. Journal of Nepal Paediatric Society, 32(1), 90-92. https://doi.org/10.3126/jnps.v32i1.5333
Section
Brief Reports/Case Reports/Case Series