Evoked potential studies in the antiphospholipid syndrome: differential diagnosis from multiple sclerosis.

BACKGROUND: The CNS manifestations of the antiphospholipid syndrome (APS) can mimic multiple sclerosis both clinically and radiologically. OBJECTIVE: To compare evoked potential studies in APS patients and patients with multiple sclerosis with similar neurological disability. METHODS: 30 APS patients with CNS manifestations and 33 patients with definite multiple sclerosis and similar neurological disability underwent studies of visual evoked potentials (VEP), somatosensory evoked potentials (SSEP) in the upper and lower limbs (UL, LL), and sympathetic skin responses (SSR) in the upper and lower limbs. RESULTS: The neurological manifestations in the APS patients included stroke (n = 17), transient ischaemic attacks (n = 10), and severe headache with multiple white matter lesions on brain MRI (n = 3). Abnormal SSEP (LL), and SSR (UL; LL) were seen in APS patients (37%, 27%, and 30%, respectively) but VEP and UL SSEP were rarely abnormal (10% and 6%, respectively in APS v 58% and 33% in multiple sclerosis; p = 0.0005, p = 0.008). Mean VEP latencies were more prolonged in multiple sclerosis (116 ms v 101 ms, p<0.001). Only one APS patient had abnormal findings in all three evoked potential studies, compared with seven patients in the multiple sclerosis group (p = 0.04) CONCLUSIONS: Abnormal VEPs are uncommon in APS in contrast to multiple sclerosis. Coexisting abnormalities in all other evoked potentials were similarly rare in APS. In patients with brain MRI findings compatible either with multiple sclerosis or APS, normal evoked potential tests, and especially a normal VEP, may support the diagnosis of APS.

The use of intravenous immunoglobulin as maintenance therapy in myasthenia gravis.

The standard therapy for myasthenia gravis (MG) includes steroids and immunosuppressants, which have delayed onset of action and significant side effects. Plasmapheresis and intravenous immunoglobulin have been used mostly for the treatment of severe exacerbations. In the present study we examined the use of intravenous immunoglobulin as maintenance treatment in MG. We included 11 patients with generalized myasthenia gravis. All had severe bulbar and respiratory involvement that required mechanical ventilation in three patients. Intravenous immunoglobulin treatment was initiated at a dose of 400 mg/kg/d for 5 days and followed by maintenance with 400 mg/kg once monthly. Regular medications were continued as necessary. There was significant improvement in all patients, and none required mechanical ventilation over the treatment period of 20.3 months +/- 8.3 (mean +/- SD, total patient years of treatment = 18.7). Steroid and pyridostigmine doses were reduced significantly and steroids were discontinued in two patients. There were no serious side effects related to intravenous immunoglobulin. These results suggest that intravenous immunoglobulin maintenance therapy is a valid modality in patients with MG.

[Prevalence of APO e4 allele in Israeli ethnic groups].

The 4 allele of the APOE gene, coding for apo-lipoprotein E, is the most common genetic risk factor for Alzheimer's disease and a significant risk factor for coronary atherosclerosis. There is therefore much interest in studying its frequency in different ethnic groups. We examined its frequency in Jews originating from Libya, Buchara and Ethiopia and in Jews of Sepharadi and Ashkenazi origins. Its frequency among Ethiopian immigrants was 0.27, significantly higher than in the other groups, in which the frequency was between 0.067 and 0.10. These differences in allele frequency may serve as a basis for future studies in Israel to assess the relative contributions of genetic and environmental factors to the incidence of dementia.