Neurológia pre prax 1/2009

Antiphospholipid syndrome, a syndrome which can mimic multiple sclerosis

Antiphospholipid syndrome (APS, also called as Hughes syndrome) is a relatively common autoimmune disorder characterized by recurrent arterial or venous thromboses and/or pregnancy-related complications with a characteristic finding of antiphospholipid antibodies. To diagnose APS, the following criteria must be met: one clinical criterion (i. e., the presence of thrombosis or repeated abortions or preeclampsia) and one laboratory criterion, i. e., the demonstration of some of the antiphospholipid antibodies within a minimal time interval of 12 weeks between individual laboratory tests. Antiphospholipid antibodies interfere with the homeostasis of blood coagulation and cause hypercoagulation state by a variety of mechanisms. When APS occurs in patients with rheumatic disease, it is referred to as secondary APS. The term „primary antiphospholipid syndrome“ (PAPS) is used when no other underlying disease is found. Within APS, CNS can be involved as well. If this involement is characterized by recurrent arterial occlusions with attack–like course, it can mimic for example multiple scelrosis (MS) and establishment of correct diagnosis can be difficult. The following case report of a woman, who has been treated with MS for 10 years, is an evidence thereof. During severe brainstem attack, due to signs of multiorgan involvment a systemic autoimmune disease was considered, with final conclusion of PAPS, which mimicked MS. The recognition of the correct diagnosis is of major relevance to treatment planning and secondary prevention of further thrombotic complications.

Keywords: primary antiphospholipid syndrome, secondary antiphospholipid syndrome, Sneddon syndrome, multiple sclerosis.