Kouri & Oshima, et al. Table S1. Tau pathology in Case 1 Region
advertisement
Kouri & Oshima, et al. 1 Table S1. Tau pathology in Case 1 Region Temporal cortex Motor cortex Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Ventral thalamus Subthalamic nucleus Thalamic fasciculus Red nucleus Substantia nigra Oculomotor complex Midbrain tectum Locus ceruleus Pontine tegmentum Pontine base Medullary tegmentum Inferior olive Dentate nucleus Cerebellar white matter NFT & preNFT ++ +++ +++ ++ +++ +++ ++ +++ ++ +++ ++ + +++ +++ + +++ ++ +++ - Coiled bodies + + ++ + + + + + + + ++ +++ + ++ Astrocytic plaques ++ +++ +++ + - Tau+ threads + +++ +++ +++ +++ +++ +++ +++ ++ ++ +++ +++ +++ +++ +++ +++ +++ +++ ++ +++ Kouri & Oshima, et al. 2 Table S2. Tau pathology in Case 2 Region Temporal cortex Motor cortex Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Ventral thalamus Subthalamic nucleus Thalamic fasciculus Red nucleus Substantia nigra Oculomotor complex Midbrain tectum Locus ceruleus Pontine tegmentum Pontine base Medullary tegmentum Inferior olive Dentate nucleus Cerebellar white matter NFT & preNFT ++ +++ +++ ++ ++ +++ +++ + + ++ ++ ++ + ++ + ++ + ++ - Coiled bodies + + + + + + + + + + ++ + + + ++ Astrocytic plaques ++ +++ +++ - Tau+ threads + ++ +++ +++ ++ + +++ ++ ++ ++ +++ + +++ +++ +++ ++ ++ +++ +++ + Kouri & Oshima, et al. 3 Table S3. Tau pathology in Case 3 Region Temporal cortex Motor cortex Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Ventral thalamus Subthalamic nucleus Thalamic fasciculus Red nucleus Substantia nigra Oculomotor complex Midbrain tectum Locus ceruleus Pontine tegmentum Pontine base Medullary tegmentum Inferior olive Dentate nucleus Cerebellar white matter NFT & preNFT ++ ++ +++ +++ ++ +++ +++ +++ + +++ ++ + ++ +++ +++ +++ ++ +++ - Coiled bodies + + + + + + + ++ ++ Astrocytic plaques ++ +++ ++ + + ++ - Tau+ threads + ++ +++ +++ +++ ++ +++ +++ ++ ++ +++ +++ +++ +++ +++ +++ +++ +++ +++ +++ Kouri & Oshima, et al. 4 Table S4. TDP-43-positive neuronal cytoplasmic inclusions Region Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Amygdala Subthalamic nucleus Dentate of hippocampus Subiculum Red nucleus Substantia nigra Locus ceruleus Pontine tegmentum Pontine base Inferior olive Cerebellar white matter CBD-OPCA 1 CBD-OPCA 2 CBD-OPCA 3 CBD 1 CBD 2 CBD 3 CBD 4 ++ ++ + ++ ++ +++ ++ ++ ++ + ++ +++ - ++ +++ ++ +++ + +++ + + +++ +++ - + + + + + NA + ++ + + +++ +++ - + +++ + + ++ + + + - NA NA NA +++ + ++ + - + +++ + +++ ++ + +++ ++ + NA - ++ ++ + + + NA + - Kouri & Oshima, et al. 5 Table S5. TDP-43-positive glial inclusions Region Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Amygdala Subthalamic nucleus Dentate of hippocampus Subiculum Red nucleus Substantia nigra Locus ceruleus Pontine tegmentum Pontine base Inferior olive Cerebellar white matter CBD-OPCA 1 CBD-OPCA 2 CBD-OPCA 3 CBD 1 CBD 2 CBD 3 CBD 4 + ++ ++ ++ + + + ++ ++ + + ++ + ++ + + + ++ + ++ ++ ++ ++ ++ ++ ++ + ++ + + + ++ NA + + + + ++ ++ ++ + + + + - NA NA NA + + - ++ + + + ++ + + NA - + + NA + - Kouri & Oshima, et al. 6 Table S6. TDP-43-positive threads Region Caudate/putamen Globus pallidus Basal nucleus Hypothalamus Amygdala Subthalamic nucleus Dentate of hippocampus Subiculum Red nucleus Substantia nigra Locus ceruleus Pontine tegmentum Pontine base Inferior olive Cerebellar white matter CBD-OPCA 1 CBD-OPCA 2 CBD-OPCA 3 CBD 1 CBD 2 CBD 3 CBD 4 ++ + +++ +++ ++ + + ++ +++ + +++ +++ + - + ++ + + + + ++ + + +++ ++ + - ++ ++ + ++ + ++ NA + + + ++ +++ ++ - + + + + + - NA NA NA + ++ + ++ +++ + + + + ++ NA - ++ + NA + - + -