Therapy of Sneddon syndrome.
We report the case of a young woman with progressive cognitive decline and epilepsy. She showed ischemic cerebrovascular disease and proximal livedo racemosa. Antiphospholipid antibody (aPL) could not be detected and there were no microemboli on continuous transcranial Doppler ultrasonography monitoring. Histology of cerebral vessels showed intimal hyperplasia in small leptomeningeal venous vessels and micronecrosis of grey and white matter. We subsequently made the diagnosis of aPL-negative Sneddon Syndrome (SNS). Anticoagulation with warfarin could not be initiated because of a drug-resistant epilepsy with the risk of falls and subsequent bleeding; immunosuppression with steroids and azathioprine was ineffective, as was initial antiplatelet therapy with clopidogrel alone. However, when we intensified antiplatelet therapy by combining clopidogrel and ASS, a slowing of disease progression, as assessed by neuropsychological testing and magnetic resonance imaging, was noted on a follow-up after 6 months. Therapeutic options in SNS in both aPL-positive and aPL-negative patients with SNS are discussed.
Flöel
Agnes
A
Department of Neurology, University of Münster, Münster, Germany. floeel@uni-muenster.de
Imai
Tanya
T
Lohmann
Hubertus
H
Bethke
Florian
F
Sunderkötter
Cord
C
Droste
Dirk W
DW
D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D000927:Anticonvulsants / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use; D000077144:Clopidogrel; D004359:Drug Therapy, Combination; D004827:Epilepsy / Q000150:complications / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D018860:Sneddon Syndrome / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D013988:Ticlopidine / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D014635:Valproic Acid / Q000627:therapeutic use
D017152:Antibodies, Antiphospholipid; D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D010975:Platelet Aggregation Inhibitors; D014635:Valproic Acid; D000077144:Clopidogrel; D001379:Azathioprine; D013988:Ticlopidine; D001241:Aspirin