Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions, with a hereditary transmission and unknown etiopathogenesis. A number of reports have documented a link between antiphospholipid antibodies and Sneddon's syndrome with different results. The present work was designed to sequentially study antiphospholipid antibodies in a patient with Sneddon's syndrome and her family and their potential role in thrombotic events. We used cardiolipin and a mixture of phospholipids from rabbit brain as antigen for antiphospholipid assays to determine diagnostic usefulness. A patient with Sneddon's syndrome and 12 available family members belonging to three generations were evaluated to determine the presence of antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) during vascular thrombotic events and asymptomatic periods. Our results support a temporal relation between thrombotic events in Sneddon's syndrome and lupus anticoagulant; anticardiolipin antibodies remained invariable. Our index case patient and her father could be diagnosed as having primary antiphospholipid antibody syndrome. Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. The use of a mixture of phospholipids as antigen could present some advantages in serological studies performed in antiphospholipid syndromes.

The authors report a patient with partial and secondarily generalized status epilepticus who required 70 days of general anesthesia for seizure control. Although antiepileptic medications failed to control the seizures, they resolved with plasma exchange. The patient's serum reacted with rat cerebral cortex, hippocampus, and cerebellum, but not with cells expressing the glutamate receptor GluR3. These findings suggest an immune response against neuronal antigens other than GluR3.

A case of an intracerebral "primary" arachnoidal cyst of the left fronto-parietal region is reported. No communication with the ventricular system or with subarachnoidal space was found at the first surgical procedure. It contained genuine choroid plexus and clinically behaved as an expansive intracranial mass.