Table 1 Clinical features of the affected family members.

From: Mutations in bassoon in individuals with familial and sporadic progressive supranuclear palsy-like syndrome

  Case 1 (III-2) Case 2 (III-1) Case 3 (II-3)
Onset age (years) 43 52 83
Primary symptom Dementia Dementia Dementia and anxiety
Disease duration (years) 18 12 9
Duration (years) from onset to gait disturbance 9 11 3
Rigidity of the neck +++ +++ +
Postural instability +++ +++ ++
Supranuclear palsy + + ±
Atrophy of the temporal lobe +++ +++ +
Atrophy of the brain stem +++ +++ ±
Hot cross bun sign + + ±
123I-IMP Decrease in the temporal lobe and brainstem Decrease in the temporal lobe and brainstem Decrease in the temporal lobe and brainstem
MIBG Normal Normal Normal