TDP-43 (bvFTD)151 | Orbital gyri, gyrus rectus and amygdala. | Pattern I |
Middle frontal and anterior cingulate gyrus, anteromedial temporal lobe areas, superior and medial temporal gyri, striatum, red nucleus, thalamus, precerebellar nuclei. | Pattern II | |
Motor cortex, bulbar somatomotor neurons and the spinal cord anterior horn. | Pattern III | |
Visual cortex. | Pattern IV | |
TDP-43 (ALS)152 | Agranular motor cortex, brainstem motor nuclei of cranial nerves V, VII and X–XII, and spinal cord a-motoneurons. | Stage 1 |
Prefrontal neocortex (middle frontal gyrus), brainstem reticular formation, precerebellar nuclei and the red nucleus. | Stage 2 | |
Prefrontal (gyrus rectus and orbital gyri), and then postcentral neocortex and striatum. | Stage 3 | |
Anteromedial portions of the temporal lobe, including the hippocampus. | Stage 4 | |
TDP-43 (AD)154 | Amygdala. | Stage 1 |
Entorhinal cortex and subiculum. | Stage 2 | |
Dentate gyrus of the hippocampus and occipitotemporal cortex. | Stage 3 | |
Insular cortex, ventral striatum, basal forebrain and inferior temporal cortex. | Stage 4 | |
Substantia nigra, inferior olive and midbrain tectum. | Stage 5 | |
Basal ganglia and middle frontal cortex. | Stage 6 | |
aSyn (MSA-P)156 | Striatum, lentiform nucleus, substantia nigra, brainstem white matter tracts, cerebellar subcortical white matter, motor cortex, mid-frontal cortex and sensory cortex. | Phase 1 |
Spinal cord and thalamus. | Phase 2 | |
Hippocampus and amygdala. | Phase 3 | |
Visual cortex. | Phase 4 | |
aSyn (MSA-C)155 | Cerebellum and cerebellar brainstem connectivities. | Phase 1 |
Pyramidal and extrapyramidal white matter. | Phase 2 | |
Neocortex and basal ganglia grey matter. | Phase 3 | |
Amygdala and hippocampus. | Phase 4 | |
aSyn (Lewy)72 | Dorsal IX/X motor nucleus and/or intermediate reticular zone (medulla oblongata). | Stage 1 |
Caudal raphe nuclei, gigantocellular reticular nucleus, coeruleus–subcoeruleus complex (pons). | Stage 2 | |
Pars compacta of the substantia nigra (midbrain). | Stage 3 | |
Temporal mesocortex (transentorhinal region, amygdala) and allocortex (CA2-plexus). | Stage 4 | |
High-order sensory association areas of the neocortex and prefrontal neocortex. | Stage 5 | |
First-order sensory association areas of the neocortex and premotor areas, mild changes in primary sensory areas and the primary motor field. | Stage 6 |
AD, Alzheimer’s disease; ALS, amyotrophic lateral sclerosis; C, cerebellar type; CA, Cornu Ammonis; MSA, multiple system atrophy; P, parkinsonian type; TDP-43, Transactive response (TAR) DNAbinding protein 43; aSyn, α-synuclein; bvFTD, behavioural variant of frontotemporal dementia.