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Herwin Speckter 1 Article
Changes in Cerebral Gray and White Matter in Patients with Pantothenate Kinase-Associated Neurodegeneration: A Long-Term Magnetic Resonance Imaging Follow-Up Study
Pedro Roa-Sanchez, Pamela Bido, Jairo Oviedo, Hans-Jürgen Huppertz, Herwin Speckter, Peter Stoeter
J Mov Disord. 2021;14(2):148-152.   Published online May 26, 2021
DOI: https://doi.org/10.14802/jmd.20102
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  • 3 Crossref
AbstractAbstract PDFSupplementary Material
Objective
To determine the volume changes in gray and white matter during a long-term follow-up in patients suffering from pantothenate kinase-associated neurodegeneration (PKAN).
Methods
Magnetic resonance imaging was repeated in 13 patients and 14 age-matched controls after a mean interval of more than 7 years. T1-weighted sequences were evaluated by fully automated atlas-based volumetry, compared between groups and correlated with disease progression.
Results
The patients did not show generalized cerebral atrophy but did show a significantly faster volume reduction in the globus pallidus during follow-up (between -0.96% and -1.02% per year, p < 0.05 adjusted for false discovery rate) than controls, which was significantly related to the progression in their dystonia scores (p = 0.032).
Conclusion
The volume loss in the globus pallidus over time—together with the accumulation of iron known as the “tiger’s eye”—supports the pathophysiologic concept of this nucleus as a center of inhibition and its severe malfunction in PKAN.

Citations

Citations to this article as recorded by  
  • Pantothenate Kinase Activation Restores Brain Coenzyme A in a Mouse Model of Pantothenate Kinase-Associated Neurodegeneration
    Chitra Subramanian, Matthew W. Frank, Rajaa Sukhun, Christopher E. Henry, Anna Wade, Mallory E. Harden, Satish Rao, Rajendra Tangallapally, Mi-Kyung Yun, Stephen W. White, Richard E. Lee, Uma Sinha, Charles O. Rock, Suzanne Jackowski
    Journal of Pharmacology and Experimental Therapeutics.2024; 388(1): 171.     CrossRef
  • Cerebral and cerebellar white matter tract alterations in patients with Pantothenate Kinase-Associated Neurodegeneration (PKAN)
    Diones Rivera, Pedro Roa-Sanchez, Pamela Bidó, Herwin Speckter, Jairo Oviedo, Peter Stoeter
    Parkinsonism & Related Disorders.2022; 98: 1.     CrossRef
  • Long-Term Neuroradiological and Clinical Evaluation of NBIA Patients Treated with a Deferiprone Based Iron-Chelation Therapy
    Nicola Romano, Giammarco Baiardi, Valeria Maria Pinto, Sabrina Quintino, Barbara Gianesin, Riccardo Sasso, Andrea Diociasi, Francesca Mattioli, Roberta Marchese, Giovanni Abbruzzese, Antonio Castaldi, Gian Luca Forni
    Journal of Clinical Medicine.2022; 11(15): 4524.     CrossRef

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