Sunday, December 02, 2007

Dementia mimicking FTD with striatal hypermetabolic

state and responsive to steroids.

Leger GC,Johnson N,Horowitz SW , et al. Dememtia like presentation of striatal hypermetabolic state with antistriatal antibodies responsive to steroids. Arch Neurol 2004; 61: 757-757.

Case presentation of a 48 year old woman with a one year history of progressive changes in pesonality and attention, originally diagnosed with a frontal dementia. PET showed a hypermetabolic lesion in the left striatum and plasma antistriatal antibodies were found. Treatment with corticosteroids resulted in a return of her premorbid state. PET scan and titers of antibody resolved. the differential diagnosis in this case was Syndenham's chorea and theantiphospholipid antibody state.

the phenotype of Sydenhams is usuallykids with onset of chorea, facial grimacing, hypotonia, and loss of fine control. Elevated ASO titers are present. Mood changes and OCD behavior may occur. Epitypes of IgM ASO titers cros react with striatum. It typically is self-limited and immune modulation may be useful. SLE occassionally occurs.