| Hyperreflexia | Deep tendon reflexes increased on affected side |
| Babinski sign | May be present in advanced cases |
| Clonus | Ankle clonus may be elicitable |
| Frontal release signs | Primitive reflexes may emerge |
| Motor cortex (precentral gyrus) | Primary cortical motor neuron degeneration |
| Corticospinal tract | Degeneration of descending motor pathways |
| Basal ganglia | Particularly the putamen and globus pallidus |
| Subcortical white matter | Including the internal capsule |
| 4R Tau pathology | Corticobasal degeneration with predominant tau deposition |
| TDP-43 pathology | Some cases show TDP-43 inclusions |
| Alzheimer's disease pathology | A smaller subset with AD-type pathology may present with this phenotype |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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