Pantothenate kinase-associated neurodegeneration: Clinical aspects, diangnosis and treatments

Razmeh, S. and Habibi, A.H. and Orooji, M. and Alizadeh, E. and Moradiankokhdan, K. and Razmeh, B. (2018) Pantothenate kinase-associated neurodegeneration: Clinical aspects, diangnosis and treatments. Neurology International, 10 (1). pp. 32-34.


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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 signs 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. © S. Razmeh et al.

Item Type: Article
Additional Information: cited By 0
Uncontrolled Keywords: baclofen; benzodiazepine derivative; botulinum toxin; cholinergic receptor blocking agent; clonidine; gabapentin; pantothenic acid; pregabalin; tetrabenazine, brain depth stimulation; clinical feature; diagnostic procedure; disease classification; human; iron chelation; neurodegeneration with brain iron accumulation; pallidotomy; pathophysiology; Review
Subjects: WK Endocrine System
Depositing User: eprints admin
Date Deposited: 19 Dec 2018 12:21
Last Modified: 14 Jul 2019 05:02

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