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Table 1: 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 14^{^a} 30 28 24 29
Frontal lobe function test 
    FAB 13^{^b} 16 14^{^b} 11^{^b} 12^{^b} 16 8^{^b} NE
CSF 
    Cell (mm³) 3 2 4 1 1 6 NE NE
    Protein (mg/dL) 66 55 100 47 52 48 NE NE
Note:—DTR indicates deep tendon reflex; –, absence of the manifestation; +, presence of the manifestation; NE, not examined.
↵a Decreased MMSE score below a cutoff score of 24.
↵b Decreased FAB score below the published age-matched average.