Table 2 Clinical features of FTLD-FUS
| Â | BIBD | NIFID | aFTLD-U | Unclassified | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
|  | 〈with chorea〉 | 〈without chorea〉 |  |  |  |  | ||||
Case No. | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 |
Sex | F | F | F | M | M | M | F | M | M | F |
Onset (age) | 32 | 44 | 56 | 57 | 40 | 34 | 67 | 29 | 39 | 30 |
Duration (y) | 7 | 3.3 | 12 | 6 | 7 | 6.3 | 5.7 | 8 | 13 | 15 |
Family history | No | No | No | No | No | No | No | No | No | No |
Initial symptoms | Apathy, Behavioral abnormality | Apathy, Polyphagia, Disinhibition | Behavioral abnormality, memory impairment | Obsessive behaviors | Disinhibition | Weakness in the left hand, dysarthria | Dysarthria | Disinhibition | Apathy, Behavioral abnormality | Behavioral abnormality, memory impairment |
Prominent features | Behavioral abnormality | Behavioral abnormality | Behavioral abnormality | Behavioral abnormality | Behavioral abnormality | Motor neuron signs | Pseudobulbar palsy, nonfluent aphasia | Behavioral abnormality | Behavioral abnormality | Behavioral abnormality |
Clinical diagnosis | bvFTD | bvFTD | bvFTD | bvFTD | bvFTD | ALS with dementia | CBD | bvFTD | bvFTD | bvFTD |
Psychiatric and behavioral symptoms | ||||||||||
Apathy | + | + | + | + | + | + | + | - | + | + |
Disinhibition | + | + | + | - | + | - | + | + | + | + |
Stereotypy | + | + | + | + | + | - | + | + | + | + |
Wandering | + | + | - | - | - | - | + | + | + | + |
Altered dietary habits, polyphagia | + | + | + | - | - | - | + | + | + | - |
Memory impairment | + | + | + | + | - | - | - | - | + | + |
Hypersexuality | + | + | - | - | + | - | + | + | + | - |
Oral tendency | + | + | - | + | - | - | - | + | + | - |
Perseverations | + | + | + | ? | - | ? | + | ? | - | - |
Language and speech | ||||||||||
Reduced speech output | + | + | + | + | + | + | + | + | + | + |
Dysarthria | - | - | - | - | - | + | - | - | - | + |
Verbal stereotypies | + | - | + | + | - | ? | + | ? | + | ? |
Semantic errors | - | - | - | ? | - | ? | - | ? | - | - |
Echolalia | + | + | + | ? | ? | ? | + | ? | - | - |
Neurological signs | ||||||||||
Involuntary movement | ||||||||||
 Onset (age) | 36 | 46 | 65 | - | - | - | - | - | - | - |
 Chorea | + | + | + |  |  |  |  |  |  |  |
 Athetosis | + | - | + |  |  |  |  |  |  |  |
 Ballismus | + | - | - |  |  |  |  |  |  |  |
Lack of normal muscular tonus | + | + | + | - | - | - | - | - | - | - |
Upper motor signs | - | - | - | - | - | + | + | + | - | - |
Lower motor signs | - | - | - | - | - | + | + | - | - | - |
Dysphagia | - | - | - | - | + | + | + | + | + | + |
Gait disturbance | + | + | + | + | + | + | + | + | + | + |
Parkinsonism-early | No | No | No | Moderate | No | Moderate | Mild | No | No | Mild |
Parkinsonism-late | No | No | Mild | Severe | Severe | Severe | Severe | Severe | Moderate | Severe |
Investigations | ||||||||||
CT/MRI (atrophy) | FT(L = R) | na | na | na | FT(L = R) | na | FT(L > R) | na | FT(L = R) | na |
EEG | Normal | Slow dominant rhythm | Slow dominant rhythm | na | Normal | na | Normal | na | Slow dominant rhythm | na |
EMG | Normal | na | na | na | na | na | na | na | na | na |