Sneddon syndrome is defined as a clinical entity consisting of livedo
racemosa generalisata (LRG) and cerebrovascular lesions, which often l
ead to physical and mental handicaps. Four patients with LRG and the s
uspected diagnosis of Sneddon syndrome had HMPAO-SPECT studies. The pa
tients underwent CT and/or MR brain imaging and three patients had Dup
lex sonography of the cerebral arteries (TCD). Brain SPECT was abnorma
l in all patients, whereas CT/MRI revealed a cerebral lesion in only o
ne patient and all TCD studies were normal. HMPAO-SPECT is valuable in
detecting disturbed regional cerebral blood flow before irreversible
ischemic insults occur, thus allowing the diagnosis of Sneddon syndrom
e at an early stage.