|
KMID : 1197720210140020148
|
|
대한파킨슨병 및 이상운동질환 학회지
2021 Volume.14 No. 2 p.148 ~ p.152
|
|
|
Changes in Cerebral Gray and White Matter in Patients with Pantothenate Kinase-Associated Neurodegeneration: A Long-Term Magnetic Resonance Imaging Follow-Up Study
|
|
Roa-Sanchez Pedro
Bido Pamela
Oviedo Jairo
Huppertz Hans-Jurgen
Speckter Herwin
Stoeter Peter
|
|
|
Abstract
|
|
|
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”ㅡsupports the pathophysiologic concept of this nucleus as a center of inhibition and its severe malfunction in PKAN.
|
|
|
KEYWORD
|
|
|
Cerebral gray and white matter volume, Long-term follow-up, Pantothenate kinase-associated neurodegeneration
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
|
|