Antiphospholipid antibodies and the brain: a consensus report.

This report discusses the difference between antiphospholipid antibodies (aPL) as a predictor for first and recurrent ischemic stroke, whether or not concomitant systemic lupus erythematosus (SLE) increases aPL-associated risk, and the association of aPL with other neurological manifestations. The neurological manifestations covered in this report were selected because they are among the most common, including cognitive dysfunction, headache, multiple sclerosis and seizures/epilepsy. Recommendations are made regarding further research that is needed to clarify remaining uncertainties.

Antiphospholipid syndrome and the brain in pediatric and adult patients.

The most common neurological manifestations of antiphospholipid syndrome (APS) in all age-groups include stroke and transient ischemic attacks due to arterial thromboses and cerebral ischemia. Antiphospholipid antibodies may cause additional non-criteria neurological impairments through vascular, neuroinflammatory and direct neuronal effects. Anti-aggregant or anticoagulant therapies are indicated for APS-related ischemic strokes. Treatment regimens for asymptomatic antibody-positive patients and those with refractory or recurrent disease remain controversial. There is scant literature on the epidemiology and therapy of neurological APS manifestations in pediatric patients. Assessments of modifiable cardiovascular and inherited thrombophilia risk factors are essential in patients with APS. There may be a role for novel neuroimaging modalities in quantifying APS-related microstructural brain damage. The clinical utility of statins, antimalarials, angiotensin-converting enzyme inhibitors, and thrombin inhibitors warrant further research.

Neurocognitive deficits and neuroimaging abnormalities are prevalent in children with lupus: clinical and research experiences at a US pediatric institution.

Neurocognitive impairments and neuroimaging abnormalities are frequently observed in adults with systemic lupus erythematosus. There is a paucity of similar data in childhood-onset disease. We hypothesized that neurocognitive and neuroimaging abnormalities would be prevalent in children undergoing neuropsychological evaluations. We reviewed patient neurocognitive evaluations performed at a large United States pediatric institution during the period 2001 to 2008. Records were retrieved from 24 children referred to neuropsychology due to clinical indications. Data from 15 children enrolled in a prospective structure-function association study were also analyzed. Subjects were predominantly African-American and Hispanic adolescent girls of average intelligence. aPL positivity and aspirin use was prevalent. Neurocognitive impairment was designated in 70.8% of retrospective, and 46.7% of prospective cohort patients. Deficits were seen at times of wellness, without previous neuropsychiatric lupus, and early in disease courses. Scores >1.5 standard deviations below published age-matched norms were common in tests of executive functioning, visual memory and visual-spatial planning. Features of depression were seen in 33.3% of the children in the retrospective cohort (clinical referrals). Cerebral and cerebellar volume loss was observed in a majority of blinded prospective cohort research magnetic resonance images (73.3% and 67.7% respectively). White matter hyperintensities were observed in retrospective and prospective cohort magnetic resonance images (36.6% and 46.7% respectively). Larger prospective studies that elucidate structure-function associations in children with systemic lupus erythematosus are planned.

Neurological manifestations of the antiphospholipid syndrome: risk assessments and evidence-based medicine.

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by autoantibody production and vascular thrombosis or pregnancy morbidity. Autoantibodies generated against phospholipid and phospholipid-binding proteins often impair phospholipid-dependent clotting assays (lupus anticoagulants). These autoantibodies activate endothelial cells, platelets and biochemical cascades and can exist in autoimmune disorders such as lupus. Consistently positive antibodies may worsen the severity of thrombo-occlusive disease. The most common neurological manifestations of APS include stroke and transient ischaemic attacks due to arterial thromboses. Antiphospholipid antibodies may cause additional neurological impairments through both vascular and immune mechanisms. Antiaggregant or anticoagulant therapies are indicated for APS-related ischaemic strokes. Treatment regimens for asymptomatic antibody-positive patients and those with refractory disease remain controversial. There is scant literature on neurological APS manifestations in paediatric patients. Assessment of traditional cardiovascular and inherited thrombophilia risk factors is essential in patients with APS. Modifiable risk factors and valvular heart disease may worsen thrombotic and cerebrovascular outcomes. Alternative therapies such as statins, anti-malarials, angiotensin-converting enzyme inhibitors and thrombin inhibitors warrant further research.

Single-photon-emission computed tomography (SPECT) in neurotologic assessment: a preliminary report.

The single-photon-emission computed tomography (SPECT) scan enables clinicians to probe dynamic and metabolic changes in brain tissue through measurement of regional cerebral blood flow (rCBF). Diagnostic benefits of the SPECT scan in clinical neurology have been demonstrated. SPECT scanning has been shown to be more sensitive than morphologic imaging techniques [magnetic resonance imaging (MRI) and computed tomography (CT)] in many conditions. However, the use of the scan in assessing neurotologic complaints remains inadequately investigated. Few studies have explored the value of SPECT in establishing the causes of dizziness, hearing loss, and tinnitus. We studied SPECT along with MRI, CT scan, electroencephalogram (EEG), and other evaluations in patients with these neurotologic complaints, SPECT abnormalities were more frequent and prominent than those visualized by other imaging modalities. Overall, 78% of SPECT scans revealed abnormalities. Abnormalities were found in 46% of MRIs, 40% of CTs, and 29% of EEGs. The disparity between SPECT scanning and other procedures was also seen once patients were divided by their chief complaints. This study illustrates the sensitivity of SPECT scanning in evaluating neurotologic complaints and highlights the need for additional research into the importance of SPECT scanning in comprehensive neurotologic evaluation.