Table 1

Summary of clinical and MRI findings of patients from the first group

Patient No Age at onset Main clinical features Other clinical features MRI Possible diagnosis
H564Chorea, dementia, depressionCorticosubcortical atrophyHD-like
H4847Chorea, dementia, behav disturbObsessive featuresCaudate atrophyHD-like
H10062Chorea, dementiaFrontal release signsCorticosubcortical atrophyHD-like
H10266Chorea, dementiaFrontal release signsLentiform nucleus atrophyHD-like
Corticosubcortical atrophy,
H12256Chorea, dementia, depressionpallidal hyperintensitiesHD-like
H15440Chorea, dementia, depressionFrontal release signsNDHD-like
Behav disturb
H2660Chorea, dementia, behav disturbAtaxiaCerebellar and caudal atrophyHD-like/cerebellar disorder
H13930Choreaoathetosis, dysarthria,Pyramidal signs, hypertension,NDHD-like
dementia, behav disturb.Angor, ischaemic cardiopathy
H15064Chorea, dementia, behav disturbCortical and cerebellar atrophyHD-like/cerebellar disorder
H15546Chorea, dementia, behav disturbAtaxiaCortical and cerebellar atrophyHD-like/cerebellar disorder
H16549Chorea, dementiaAtaxiaHD-like/cerebellar disorder

  • behav disturb=Behavioural disturbances; ND=not determined.