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Table 2 Clinical features of FTLD-FUS

From: Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration

  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
  1. +, present; -, absent; na, not available