Cerebral blood flow in Sneddon syndrome

Sneddon syndrome is defined as a clinical entity consisting of livedo racemosa generalisata (LRG) and cerebrovascular lesions, which often lead to physical and mental handicaps. Four patients with LRG and the suspected diagnosis of Sneddon syndrome had HMPAO-SPECT studies. The patients underwent CT and/or MR brain imaging and three patients had Duplex sonography of the cerebral arteries (TCD). Brain SPECT was abnormal in all patients, whereas CT/MRI revealed a cerebral lesion in only one 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 syndrome at an early stage.