Cerebral microembolism in patients with Sneddon's syndrome
M. Sitzer, D. Sohngen, M. Siebler, C. Specker, J. Rademacher, I. Janda, A. Aulich and H. Steinmetz
Department of Neurology, Heinrich-Heine-University, Dusseldorf, Germany.
BACKGROUND: The pathogenesis of Sneddon's syndrome is unclear. This study
addresses the question whether cerebral thromboembolism may be involved in
the pathogenesis of the neurologic complications of the disorder. The study
consisted of 13 patients with Sneddon's syndrome defined by both
generalized livedo reticularis and a history of one or more cerebrovascular
ischemic events; none had clinical or Doppler ultrasonographic evidence of
atherosclerosis. METHODS: Transcranial Doppler microembolic monitoring of
the middle cerebral artery; blood screening for antiphospholipid antibodies
(lupus anticoagulant, anticardiolipin antibodies). RESULTS: Five patients
(38%) showed clinically silent microembolism at transcranial Doppler
monitoring, with individual microembolic event rates of the middle cerebral
artery between 2 per hour and 33 per hour. In this group, the time since
the last ischemic symptom was significantly shorter than in the eight
patients without microemboli. Antiphospholipid antibodies were detected in
three patients (23%), all of whom belonged to the microemboli-positive
group. CONCLUSIONS: These data suggest that the detectability of both
clinically silent cerebral microembolism and antiphospholipid antibodies
may provide paraclinical evidence of active disease in patients with
Sneddon's syndrome. The results support the notion that an immune-mediated
prothrombotic state facilitating the formation of arterial thrombi with
subsequent cerebral embolization, and/or triggering in situ thrombosis of
cerebral vessels, plays a pathogenetic role in the neurologic
manifestations of this disorder.