Summary of the clinical features of patients with NIID
| Patient No. | ||||||||
|---|---|---|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | |
| Age at onset (yr) | 64 | 52 | 62 | 62 | 67 | 67 | 67 | 64 |
| Sex | M | F | F | M | F | M | F | M |
| Disease duration at MRI (yr) | 2 | 9 | 8 | 3 | 5 | 6 | 1 | 5 |
| Clinical manifestations | ||||||||
| Dementia | – | – | – | + | + | – | + | – |
| Delusion | – | – | + | – | – | – | – | – |
| Muscle weakness | – | – | – | – | – | – | – | – |
| Tremor | – | – | Postural | – | Postural | Postural | – | Resting, postural |
| Rigidity | – | – | – | – | – | – | – | – |
| Ataxia | – | – | – | + | + | – | – | + |
| Hyporeflexia or areflexia in DTR | + | + | + | + | + | + | + | + |
| Sensory disturbance | – | – | – | – | – | – | – | – |
| Urinary incontinence | – | – | – | + | + | – | – | – |
| Transient visual field abnormality | + | + | – | – | – | – | – | + |
| Transient hemiparesis | – | + | – | – | – | – | – | – |
| Transient abnormal behavior | – | + | – | + | – | + | – | – |
| Generalized convulsion | – | – | – | – | – | – | – | – |
| Disturbance of consciousness | – | – | – | – | – | + | + | + |
| Cognitive screening test | ||||||||
| MMSE | 30 | 29 | 30 | 14a | 30 | 28 | 24 | 29 |
| Frontal lobe function test | ||||||||
| FAB | 13b | 16 | 14b | 11b | 12b | 16 | 8b | NE |
| CSF | ||||||||
| Cell (mm3) | 3 | 2 | 4 | 1 | 1 | 6 | NE | NE |
| Protein (mg/dL) | 66 | 55 | 100 | 47 | 52 | 48 | NE | NE |