| Typical Onset | Similar to typical CBS (mean age 60-68 years) |
| Asymmetric onset | symptoms begin on one side and remain more pronounced ipsilaterally |
| Cortical signs | ideomotor apraxia, cortical sensory loss, alien limb phenomenon |
| Extrapyramidal features | rigidity, bradykinesia, dystonia |
| Myoclonus | present in approximately 40-60% of cases |
| Simultanagnosia | inability to perceive more than one object at a time |
| Optic ataxia | misreaching for visual targets despite intact motor strength |
| Oculomotor apraxia | difficulty initiating voluntary saccades |
| Visual field defects | often contralateral to the more affected hemisphere |
| Agnosia | failure to recognize familiar objects or faces despite intact vision |
| Agraphia | writing difficulty |
| Acalculia | calculation impairment |
| Databases | OMIMOrphanetClinicalTrialsPubMed |