Fig. 2

a–o Radiological Findings of six individuals diagnosed with Biotin Thiamine Responsive Basal Ganglia Disease in Kuwait (Cases 4, 7, 9, 15, 19, 20): a Case 4. Plain brain computed tomography (CT) showing bilateral swelling and diffuse hypodensity of putamen. b Case 4. Follow up T2-weighted brain magnetic resonance imaging (MRI) revealing bilateral caudate, putamen and external capsule atrophy sparing globus pallidus and sub-insular regions, with multiple T2 hyperintense cystic foci of necrosis/injury. c Case 7. T1 post contrast brain MRI showing bilateral T1 faint enhancement in the caudates, peripheral putamen, thalami, and some occipital leptomeningeal regions. d Case 7. T2-FLAIR image revealing bright hyperintense lesions again in the basal ganglia and thalami with diffuse bilateral subcortical involvement. e Case 7. T1-weighted post contrast image showing faint subtle enhancement of the T2-FLAIR hyperintense lesion in the vermis; (f). g, h Case 9. T2-FLAIR brain MRI showing cortical and subcortical hyperintensities at both cerebral hemispheres and subtle cerebellar changes, as well as caudate and putamen bilaterally. i Case 9. T1-weighted brain MRI image correlated with T2-weighted image j showing the basal ganglia lesions as T1 hypointense and T2 hyperintense, k Case 15. Axial and l coronal T2 weighted MR images of the brain showing bilateral symmetric hyperintense signals in the midbrain/cerebral peduncles, as well as the basal ganglia and medial thalami There are multiple cortical/subcortical and bilateral sub-insular T2 hyperintensities. m Case 19. T2-FLAIR brain MRI showing multiple scattered hyperintense lesions at the cortical and subcortical cerebral parenchyma, as well as bilateral caudate, putamen and medial thalamic nuclei. n Some of these areas showed partial diffusion restriction on DWI. o Case 20. T2-weighted brain MRI showing hyperintensity of both putamina, representing atrophy with central necrosis/injury