Table 2.
Summary of characteristics of Parkinsonism and motor and non-motor manifestations other movement disorders seen in patients carrying autosomal dominant genes mutations for FTD associated with Parkinsonism
| MAPT | PGRN | C9ORF72 | CHMP2B | TARDBP | VCP | FUS | |
|---|---|---|---|---|---|---|---|
| Parkinsonism | |||||||
| Bradykinesia | +++ | +++ | ++ | + | ++ | ++ | ++ |
| Rigidity | +++ | +++ | ++ | ++ | ++ | + | + |
| Postural instability | ++ | ++ | + | − | − | − | − |
| Resting tremor | +/− | +/− | +/− | − | + | +/− | − |
| Gait impairment | + | + | + | + | − | − | |
| PSP phenotype | |||||||
| Supranuclear gaze palsy | ++ | + | + | + | − | − | + |
| Difficulty initiating saccades | ++ | + | + | + | − | − | + |
| CBS phenotype | |||||||
| Apraxia | + | ++ | + | ++ | − | ++ | + |
| Cortical sensory loss | − | + | + | − | − | − | − |
| Alien limb phenomenon | − | + | + | − | − | − | + |
| Other movement disorders | |||||||
| Tremor (except resting tremor) | +/− | − | − | − | − | − | − |
| Dyskinesia | − | − | − | + | − | + | − |
| Dystonia | − | + | + | + | + | + | − |
| Myoclonus | + | + | − | + | − | + | − |
| Chorea | − | − | − | − | − | − | +/− |
| Tics | − | − | − | − | + | − | − |
| RBD | − | − | − | − | + | +/− | − |
| Restless legs syndrome | − | − | − | − | − | − | − |
| Asymmetry | − | ++ | + | − | + | − | − |
| Levodopa responsiveness | |||||||
| Might be temporarily effective | Usually not effective | Might be temporarily effective | UKN | Effective | Might be effective | UKN | |
CBS=corticobasal syndrome; PSP=progressive supranuclear palsy; RBD=REM behavioral sleep disorder; UKN=unknown; (−)=not present/not assessed; (+/−) =infrequent; (+)=present in some cases; (++)=moderate frequency; (+++)=very frequent