Table 1.
Clinical characteristics of 11 subjects with the ‘hot cross bun’ sign
ID | Sex | Age at onset (years) | Current age (years) | Time to MRI (years after symptom onset) | Parkinsonism | Cerebellar signs | Urinary symptoms | Erectile dysfunction | Orthostasis | Levodopa response | Diagnosis |
---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 55 | 60 | 2 | Yes | Yes | Frequency | Not documented | Not documented | No | Possible MSA |
2 | F | 59 | Dead at 71 | 9 | No | Yes | Incontinence | N/A | Not documented | N/A | Uncertain after autopsy |
3 | M | 60 | 65 | 5 | Yes | No | None | No | No | No | Probable DLB |
4 | M | 61 | 66 | 3 | Yes | No | Retention | Yes | Yes | Yes | Possible MSA |
5 | F | 36 | 40 | 2 | No | Yes | Urgency | N/A | Yes | N/A | Undefined hereditary ataxia |
6 | F | 58 | 69 | 8 | No | Yes | Incontinence | N/A | No | No | Probable MSA |
7 | F | 55 | 68 | 3 | No | Yes | Incontinence | N/A | Yes | N/A | Probable MSA |
8 | F | 53 | Dead at 65 | 4 | Yes | Yes | Incontinence and retention | N/A | Yes | No | Probable MSA |
9 | F | 66 | 68 | 0 | Yes | Yes | Incontinence | N/A | No | Yes | Probable MSA |
10 | M | 33 | 79 | 33 | No | Yes | None | Not documented | Not documented | N/A | Undefined hereditary ataxia |
11 | M | 30 | 68 | 36 | No | Yes | None | Yes | Not documented | N/A | Undefined hereditary ataxia |
MSA: multiple system atrophy, DLB: dementia with Lewy bodies.