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Neurology International
Volume 10
Issue 1
10.4081/ni.2018.7516
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Neurology International is published by MDPI from Volume 12 Issue 3 (2020). Previous articles were published by another publisher in Open Access under a CC-BY licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Review

Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments

by
Saeed Razmeh
1,*,
Amir Hassan Habibi
2,
Maryam Orooji
2,
Elham Alizadeh
2,
Karim Moradiankokhdan
3 and
Behroz Razmeh
3
1
Yasuj University of Medical Sciences, Yasuj, Iran
2
Iran University of Medical Sciences, Tehran, Iran
3
Kermanshah University of Medical Sciences, Kermanshah, Iran
*
Author to whom correspondence should be addressed.
Neurol. Int. 2018, 10(1), 7516; https://doi.org/10.4081/ni.2018.7516
Submission received: 30 November 2017 / Revised: 29 December 2017 / Accepted: 4 February 2018 / Published: 4 April 2018

Abstract

Pantothenate Kinase-Associated Neurodegeneration (PKAN) is an autosomal recessive disorder characterized by a mutation in the PANK2 gene. The clinical presentation may range from only speech disorder to severe generalized dystonia, spasticity, Visual loss, dysphagia and dementia. The hallmark of this disease is eyes of the tiger sign in the medial aspect of bilateral globus pallidus on T2-weighted MRI that is a hyperintense lesion surrounded by hypointensity. Common treatments for PKAN disease include anticholinergics, botulinum toxin, Oral and Intrathecal baclofen, Iron chelation drugs and surgical procedures such as ablative pallidotomy or thalamotomy, Deep brain stimulation. There are many controversies about the pathogenesis and treatment of this disease, and in recent years interesting studies have been done on PKAN disease and other similar diseases. This review summarizes the clinical presentation, etiology, imaging modalities and treatment.
Keywords: Pantothenate Kinase-Associated Neurodegeneration; Neurodegeneration with brain iron accumulation Pantothenate Kinase-Associated Neurodegeneration; Neurodegeneration with brain iron accumulation

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MDPI and ACS Style

Razmeh, S.; Habibi, A.H.; Orooji, M.; Alizadeh, E.; Moradiankokhdan, K.; Razmeh, B. Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments. Neurol. Int. 2018, 10, 7516. https://doi.org/10.4081/ni.2018.7516

AMA Style

Razmeh S, Habibi AH, Orooji M, Alizadeh E, Moradiankokhdan K, Razmeh B. Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments. Neurology International. 2018; 10(1):7516. https://doi.org/10.4081/ni.2018.7516

Chicago/Turabian Style

Razmeh, Saeed, Amir Hassan Habibi, Maryam Orooji, Elham Alizadeh, Karim Moradiankokhdan, and Behroz Razmeh. 2018. "Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments" Neurology International 10, no. 1: 7516. https://doi.org/10.4081/ni.2018.7516

APA Style

Razmeh, S., Habibi, A. H., Orooji, M., Alizadeh, E., Moradiankokhdan, K., & Razmeh, B. (2018). Pantothenate Kinase-Associated Neurodegeneration: Clinical Aspects, Diagnosis and Treatments. Neurology International, 10(1), 7516. https://doi.org/10.4081/ni.2018.7516

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