Video 1
L Hydroxyglutaric aciduria segment. Patient manifesting rest, postural and intentional tremor in both upper extremities, dysarthria, dysdiadochokinesia, and unstable gait with dragging of the legs.
Figure 1
Axial brain MRI sequences. (A) FLAIR sequence shows bilateral subcortical white matter hyperintensities in frontal – parietal and temporal lobes. (B) T2WI with bilateral hyperintensities of dentate nuclei, and (C) T2WI showing bilateral hyperintensities in basal ganglia. Note in (A) and (C) the sparing of both thalami.
Video 2
L2 hydroxyglutaric aciduria segment 2. Improvement of upper extremities tremor and gait with the use of levocarnitine and vitamin supplements.