Acta Neuropathol (2010) 120:33–41 Table 1 Clinical symptoms in aFTLD-U cases ID Sex Diagnosis Behavioural/personality UBC1 F UBC2 F UBC3 F UBC4 F UBC5 M UBC6 F MUC1 M MUC2 F MUC3 F MUC4 M MUC5 F MUC6 M MUC7 F MUC8 M MUC9 M UR1 M UR2 F UR3 F UR4 F UT1 M UTSW1 M NWU1 M NWU2 F UP1 F UP2 M UP3 M UNSW1 F UNSW2 M MC1 M MC2 F MC3 M MC4 M MC5 M UCL1 M bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD Dem bvFTD Dem bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD bvFTD % with symptom 1 1 1 1 1 1 1 1 0 1 1 0 1 1 1 1 1 0 1 1 1 1 0 0 0 1 0 74 1 0 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 0 1 1 1 1 1 1 0 1 1 91 1 0 1 1 1 0 0 1 1 1 1 1 1 1 0 1 1 1 1 1 1 1 1 0 1 1 81 1 0 0 0 0 0 0 1 0 0 1 1 0 1 1 1 1 1 1 1 1 1 0 1 1 1 1 0 61 0 1 1 0 1 0 0 1 1 0 0 1 1 1 0 0 0 0 1 1 1 0 1 52 0 1 0 0 1 0 0 0 0 1 0 1 0 0 0 0 0 0 0 0 0 0 18 0 0 0 0 0 1 0 1 0 0 0 0 0 0 0 0 0 1 0 0 0 1 18 Psychiatric Cognitive Motor Apathy Disinh Abnormal Compulsions Inappropriate Delusions Hallucinations Aphasia eating sexual Parkinsonism MND Cases of aFTLD-U were scored for presence of the symptoms indicated 0 absent, 1 present, information not available, bvFTD behavioural variant FTD, Dem unspeciﬁed dementia, Disinh disinhibition a One patient exhibited tongue fasciculation, dysphagia and muscle hypotonia but there was insufﬁcient evidence to diagnose MND these cases form a clinically homogeneous group. 94% (32/ 34) of aFTLD-U patients received an initial diagnosis of bvFTD (Table 1). The other two patients, for whom there was little clinical information available, were described as having ‘unspeciﬁed dementia’. Patients were typically described as showing a decline in social conduct, self- neglect and disinhibition, with aggression as a common feature. Inappropriate sexual behaviour was widely repor- ted, in 52% of cases (12/23) (Table 1). Dietary changes, most commonly overeating, were observed in 81% of cases (21/26) with hyperorality a feature of several. Strikingly, psychotic symptoms, either hallucinations or delusions, were observed in 36% of aFTLD-U patients (8/22), and not comorbid in any case. Motor symptoms 0 0 0 0 0 0 1 1 1 0 0 0 0 0 0 0 1 0 1 0 0 0 1 1 0 0 0 0 0 24 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1 0 0 0 0 0 0 0 3 37 0 0 0 0 0 0 0 0 0 0 0a 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 123