Pantothenate Kinase Associated Neurodegeneration: A Case Report

Authors

  • 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

DOI:

https://doi.org/10.3126/jnps.v32i1.5333

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

Downloads

Download data is not yet available.
Abstract
768
PDF
769

Downloads

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

Issue

Vol. 32 No. 1 (2012)

Section

Brief Reports/Case Reports/Case Series

License

Authors who publish with this journal agree to the following terms:

Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.

Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).