AI Article Synopsis
- GTPCH1-deficient dopa-responsive dystonia (DRD) is a condition that starts in childhood, causing issues with posture and movement, but can improve significantly with levodopa treatment.
- Researchers conducted a three-dimensional gait analysis (3DGA) on three patients receiving levodopa to measure various aspects of their walking patterns before and after treatment.
- Results showed that treatment could lead to improvements in some patients, particularly in foot alignment and gait consistency, although the effects varied individually, highlighting the need for tailored evaluations in treatment.
Article Abstract
Background: GTP-cyclohydrolase 1-deficient dopa-responsive dystonia (GTPCH1-deficient DRD) typically presents in childhood with dystonic posture of the lower extremities, gait impairment, and a significant response to levodopa. We performed three-dimensional gait analysis (3DGA) to quantitatively assess the gait characteristics and changes associated with levodopa treatment in patients with GTPCH1-deficient DRD.
Methods: Three levodopa-treated patients with GTPCH1-deficient DRD underwent 3DGA twice, longitudinally. Changes were evaluated for cadence; gait speed; step length; gait deviation index; kinematic data of the pelvis, hip, knee, and ankle joints; and foot progression angle.
Results: Levodopa treatment increased the cadence and gait speed in one of three patients and increased the gait deviation index in two of three patients. The kinematic data for each joint exhibited different characteristics, with some improvement observed in each of the three patients. There was consistent marked improvement in the abnormal foot progression angle; one patient had excessive external rotation of one foot, another had excessive bilateral internal rotation, and the other had excessive internal rotation of one foot and excessive external rotation of the opposite foot, all of which improved.
Conclusion: The 3DGA findings demonstrate that the gait pathology and recovery process in GTPCH1-deficient DRD vary from case to case. Changes in the foot progression angle and gait deviation index can enable the effects of treatment to be more easily evaluated.
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| http://dx.doi.org/10.1016/j.pediatrneurol.2024.03.006 | DOI Listing |
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Three-Dimensional Gait Analysis as a Biomarker for GTP Cyclohydrolase 1-Deficient Dopa-Responsive Dystonia.
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Article Synopsis
- GTPCH1-deficient dopa-responsive dystonia (DRD) is a condition that starts in childhood, causing issues with posture and movement, but can improve significantly with levodopa treatment.
- Researchers conducted a three-dimensional gait analysis (3DGA) on three patients receiving levodopa to measure various aspects of their walking patterns before and after treatment.
- Results showed that treatment could lead to improvements in some patients, particularly in foot alignment and gait consistency, although the effects varied individually, highlighting the need for tailored evaluations in treatment.
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J Neurol Neurosurg Psychiatry
August 2009
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, Queen Square, London WC1N 3BG, UK.
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View Article and Find Full Text PDFClinical and genetic features of DYT1 and DYT5.
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Article Synopsis
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