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Neurological manifestations of primary antiphospholipid antibody syndrome: a prospective study.

The primary antiphospholipid syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features, and a variety of other nonthrombotic neurologic syndromes. The association of neurologic manifestations in primary APS has not been clearly studied from the Indian subcontinent. A study was done to determine the association of neurologic manifestations in primary APS in South Indian patients presenting to our institution, a tertiary care referral center, between January 2004 and December 2005. Ethical committee approval of the institution was obtained. There were 20 patients of primary APS with neurologic manifestations during this period. The diagnosis of primary APS syndrome was made based on the presence of clinical criteria of arterial or venous thrombosis and laboratory diagnosis based on positive lupus anticoagulant or elevation of anticardiolipin antibodies (IgM and/or IgG) in moderate to high titers repeated twice 6 weeks apart. Patients with proven positive antiphospholipid antibody were also investigated for other procoagulant states, which could coexist with primary APS such as antithrombin, protein C, protein S, and serum homocysteine. Factor V Leiden mutation analysis was not done in view of the rarity of its presentation in the Indian population. The diagnosis of the neurologic manifestation was made by computerized tomography, magnetic resonance imaging, and magnetic resonance angiography. Carotid angiography was not done to prove thrombosis, but the diagnosis made by magnetic resonance angiography. Only proven primary APSs with neurologic manifestations were included in our study. All patients with connective tissue disorders were excluded. Case definitions of primary APS as proposed by the International consensus statement on preliminary classification criteria for definite APS was followed. Among the 20 patients who were diagnosed having primary APS, there was female preponderance with 12 women and 8 men. The mean age of presentation in men was 25.5 5.2 years (range, 13–40 years) and in women was 26.5 6.3 years (range, 12–43 years). There was no mortality in our series. In our study, 13 patients (65%) had hemiparesis, 2 patients (10%) presented with cerebellar involvement, 2 patients (10%) had headache, 1 patient (5%) pseudotumor cerebri, 1 patient (5%) migraine. One patient (5%) presented with ocular symptoms and 1 patient (5%) presented with memory disturbances with gradually deteriorating cognitive dysfunction and 1 more patient (5%) presented with neuropsychiatric manifestations (Table 1). The commonest presentation in our series was cortical venous thrombosis in 7 patients (35%). There were 6 women, 5 with postpartum onset with a mean age of 24 years and 1 man aged 22 years. The mean time of onset of thrombosis in the postpartum state was 8 days. The thrombosis was most commonly noted in the superior saggital sinus, seen in 5 patients (25%), followed by sigmoid sinus thrombosis in 2 patients (5%). IgM acL was elevated in moderate to high titers in all the 7 patients with a mean value of 28 MPL Units. Cerebral venous thrombosis (CVT) has been reported to be an uncommon manifestation in APS. CVT may be encountered in a variety of hypercoagulable states, and had been reported more frequently in young women, often during pregnancy and the puerperium or associated with oral contraceptive pills. There are only rare isolated reports of CVT in the presence of APS, but it was Deschiens et al. who postulated a role of APS as a risk factor for CVT, possibly in association with other potential thrombophilic risk factors such as activated protein C resistance due to factor V Leiden mutation. Reports from India of the high incidence of CVT has been highlighted in a study by Christopher et al, where elevated levels of aCL was found to be present in 22.6% of CVT patients among the 31 patients studied. Similar observations have not been observed in any other study from India. In our series, contrary to world literature, there have been more cases of cortical venous thrombosis (35%). The possible reason could be that we in India see more cases of CVT From the Institutes of *Internal Medicine, and †Neurology, Madras Medical College, Chennai. Reprints: Dr. N. Udayakumar, MD, Resident in Internal Medicine, Institute of Internal Medicine, Madras Medical College, Chennai 600 003. E-mail: udhaykumar81@yahoo.co.in. Copyright © 2007 by Lippincott Williams & Wilkins ISSN: 1076-1608/07/1304-0238 DOI: 10.1097/RHU.0b013e318124fddf