Neurologic manifestations of the antiphospholipid syndrome

Abstract

Neurologic disorders are among the most prominent clinical manifestations associated with the antiphospholipid syndrome. Such neurologic disorders are predominantly related to focal central nervous system thrombo-occlusive events. This review summarizes the latest data regarding the clinical aspects of stroke and other neurologic manifestations associated with antiphospholipid antibodies.

Neurological manifestations of antiphospholipid syndrome

“…More than 40% of the patients with Sneddon’s syndrome have APS, which suggests that there is an association between these two conditions [48]. Although clinical and magnetic resonance imaging findings are similar in the two disease, the clinical course of Sneddon’s syndrome is generally worse, and patients with Sneddon’s syndrome present cognitive deterioration that is more pronounced, as well as a greater number of disabilities [16]. In addition, patients with Sneddon’s syndrome more often present leukoaraiosis and small lacunar infarcts, whereas patients with APS can present main cerebral artery territory infarction, such as middle cerebral artery territory infarction [49].…”

“…In lupus patients with convulsions, aPL impair the function of a gamma-aminobutyric acid receptor-mediated chloride channel in the myelin sheath [26] and that aPL can decrease the seizure threshold through a direct and reversible mechanism. It has been suggested that aPL play a direct role in the development of cognitive and behavioural Sneddon's syndrome -The clinical course of Sneddon's syndrome is generally worse, and patients with Sneddon's syndrome present greater cognitive deterioration [17] Myelopathy < 1% [2] There is a strong correlation between transverse myelitis and aPL in SLE [9] Convulsions 7% [2] It is possible that many cases of convulsions in aPL-positive patients are caused by ischaemic events [55] Chorea 1AE3% [2] It is a well-known phenomenon in patients with SLE and is strongly correlated with aPL positivity [16] Headache and migraine 20AE2% [2] Headache in APS is often untreatable, not responding to narcotics or analgesics and persisting for years before APS is diagnosed [9] Ocular syndromes 15-88% [9] Venous stasis retinopathy and neuro-ophthalmologic symptoms have been described in association with aPL positivity [77] Multiple sclerosis ⁄ multiple sclerosis-like expression -There are no definite diagnostic tests for multiple sclerosis and APS [10] Dementia 2AE5% [2] Factors associated with cognitive deterioration: persistently positive aPL levels; prednisone use; diabetes; high scores for depression; and low level of education [83] Guillain-Barré syndrome -Probably produced as a result of damage to myelin [86] Peripheral neuropathy Rare Peripheral nervous system involvement is rare in APS [87], being most commonly found in SLE and in vasculitis of small and medium-sized vessels deficit in patients with APS [27]. It is possible that the prothrombotic state associated with aPL positivity is responsible for changes in the cerebral microcirculation as the principal cause of certain types of neuropsychiatric manifestations observed in APS, including convulsions and cognitive dysfunction [9].…”

“…Chorea is a well-known phenomenon in patients with SLE and is strongly correlated with aPL positivity [16]. Chorea has been described in association with primary APS in a number of patients, many of whom were children [16]. Cervera et al [62] reviewed the clinical, radiological and immunological characteristics of 50 patients with chorea and APS, of whom 15% had SLE, 12% had lupus-like syndrome and 30% had primary APS.…”

“…Patients presenting with ischaemic neurological disease should be approached with a high degree of clinical suspicion for aPL positivity. Screening for aPL should be performed in patients with serological or clinical manifestations of APS (such as recurrent miscarriages, venous thrombosis and livedo reticularis), in young stroke patients, in any patient with recurrent stroke and in patients experiencing thrombo‐occlusive events of undetermined origin [16].…”

Antibodies directed against ribosomal P proteins cross-react with phospholipids

“…Anti-phospholipid antibody syndrome may appear as a stand-alone syndrome or associated with major CTDs (as SLE) and may comprise a number of neurological manifestations [6].…”