Abstract
Shahin Asadi* Author Affiliations Director in the Division of Medical Genetics and Molecular Pathology Research, Iran Received: October 24, 2019 | Published: November 01, 2019 Corresponding author: Shahin Asadi, Director in the Division of Medical Genetics and Molecular Pathology Research, Iran DOI: 10.26717/BJSTR.2019.22.003753
Highlights
Pantothenate kinase-associated neurodegeneration (PKAN), formerly called Hallervorden-Spatz syndrome, is a rare, inherited neurological movement disorder characterized by the progressive degeneration of specific regions in the central nervous system
PKAN is the most common type of neurodegeneration with brain iron accumulation (NBIA), a group of clinical disorders marked by progressive abnormal involuntary movements, alterations in muscle tone, and postural disturbances
PKAN is typically diagnosed by molecular genetic testing, most often after a characteristic finding on magnetic resonance imaging (MRI), called the “eye-of-the-tiger” sign, is detected
Summary
Pantothenate kinase-associated neurodegeneration (PKAN), formerly called Hallervorden-Spatz syndrome, is a rare, inherited neurological movement disorder characterized by the progressive degeneration of specific regions in the central nervous system (neurodegenerative disorder). PKAN is the most common type of neurodegeneration with brain iron accumulation (NBIA), a group of clinical disorders marked by progressive abnormal involuntary movements, alterations in muscle tone, and postural disturbances (extrapyramidal). PKAN is typically diagnosed by molecular genetic testing, most often after a characteristic finding on magnetic resonance imaging (MRI), called the “eye-of-the-tiger” sign, is detected. Hallervorden-Spatz syndrome, known as pantothenatedependent neurodegeneration, is a genetic disorder in the nervous system that is characterized by progressive motor impairment and usually begins in childhood.
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