Article Figures & Data
Figures
- Figure 1. Neuroimaging findings in chorea-achantocytosis
Axial T2-weighted FLAIR MRI of patient 3 (at age 46 years) shows a pronounced atrophy of the caudate nuclei and putamina and increased signal intensity from the putamina (arrows) (A) DaTscan SPECT and fluorodeoxyglucose PET in the same patient at age 43 years demonstrate very low DAT density in both putamen (B, arrows) respectively severe hypometabolism in the striata (C, arrows). DAT = dopamine transporter; FLAIR = fluid-attenuated inversion recovery.
Tables
Video 1
These recordings illustrate the course of disease for patient 1. Segment 1, recorded at age 39 years, displays feeding dystonia but normal speech. In addition, there is mild slowness when he performs alternating hand movements and finger taps. Intermittent truncal chorea is also mild. Dystonia in the right leg, and induced by walking, is also evident. Walking backward overcomes leg dystonia completely. Five years later (segment 2), the patient is basically bound to a wheelchair and displays dystonic movements in his right arm induced by action. Truncal chorea is more pronounced, and the patient displays marked bradykinesia. In addition, the patient can barely perform alternating hand movements, and his speech is barely intelligible. However, he is able to navigate an electric wheelchair. Buckling of the right leg is pronounced, and the patient needs constant support to walk even very short distances.Download Supplementary Video 1 via http://dx.doi.org/10.1212/000426_Video_1
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