title: Childhood stroke associated with protein C or S deficiency and primary antiphospholipid syndrome.
abstract: Two girls, 22 months and 12 years of age, presented with repeated cerebral infarctions in association with primary antiphospholipid syndrome. The younger patient also suffered from protein C deficiency, while the other one had protein S and complement C4 deficiencies. All other causes of cerebral infarction were excluded; however, vasculitis remains a possibility in one patient. Both girls developed spastic tetraparesis as a sequela of the previous infarctions. The two patients were treated with aspirin and prednisone, with remission of the infarctions during the next 8 months of observation. A primary deficiency of protein C or S is proposed which would produce cerebral thrombosis with exposure of phospholipids; this thrombosis then, like antigens, would generate antibodies acting on the thrombin-thrombomodulin complex, exacerbating the thrombotic process. The association of complement C4 deficiency is an additional risk factor.
authors.affiliation: Neuropediatric Unit, Military Hospital, Santiago, Chile.
authors.forename: M
authors.initials: M
authors.lastname: Devilat
authors.forename: M
authors.initials: M
authors.lastname: Toso
authors.forename: M
authors.initials: M
authors.lastname: Morales
mesh_terms: D016736:Antiphospholipid Syndrome / Q000150:complications*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002544:Cerebral Infarction / Q000209:etiology* / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D020151:Protein C Deficiency; D018455:Protein S Deficiency; D014057:Tomography, X-Ray Computed