| Age of onset for female and male patients (mean±SD) | 40±10 years in female | 49 years / female |
| 47±11 years in male |
| Disease duration (mean±SD) | 6.8±5.4 years | 12 years |
| Mode of inheritance | Autosomal dominant inheritance or sporadic occurrence | Not definite |
| Father: dementia and gait disturbance |
| Sister: gait disturbance |
| Clinical features (prevalence, %) | | |
| Cognitive impairment | 0.94 | Present |
| K-MMSE; 25/30 initially |
| Psychiatric symptoms | 75% | Present |
| Anxiety, depression, apathy, indifference, abulia, irritability, disinhibition, distraction, etc. | Depression → aggressive behavior |
| Parkinsonism | 61% | Present |
| Resting tremor, rigidity, bradykinesia, postural instability | Postural instability, bradykinesia (left dominant) → rigidity↑ |
| Pyramidal signs | 57% | Present |
| Hyperreflexia, spasticity, increased tone in extremities, pseudobular palsy | Hyperreflexia, spasticity |
| Seizures | 32% | Present |
| Other clinical features | Dysarthria (34%) | Present |
| Dysphagia (17%) | Present |
| Ataxia (27%) | Present |
| Sensory disturbance (10%) | Allodynia |
| Peripheral neuropathy (2%) | History of premature ovarian failure |
| Voiding difficulty |
| MRI findings (prevalence, %) | | |
| Bilateral white matter lesions | 69% | Present |
| Thining of corpus callosum | 49% | Present |
| Calcification in the white matter | 14% | Absent |
