Plasma exchange is a highly efficient technique to remove circulating autoantibodies and other humoral factors rapidly from the vascular compartment. It was the first effective acute treatment for peripheral disorders such as Guillain-Barré syndrome and myasthenia gravis before intravenous immunoglobulin became available. The recent recognition of rapidly progressive severe antibody-mediated central nervous system disorders, such as neuromyelitis optica spectrum disorders and anti-N-methyl-D-aspartate-receptor encephalitis, has renewed interest in using plasma exchange for their acute treatment also. In this review we explain the principles and technical aspects of plasma exchange, review its current indications, and discuss the implications for its provision in the UK.

We found variation in knowledge of cost. Formal education about costs and working in a private practice setting were associated with increased cost consciousness.

Pyridoxine-dependent epilepsy (PDE) is a genetic metabolic disease caused by inborn errors affecting vitamin B6 metabolism, which typically presents with neonatal seizures resistant to antiepileptic drugs (AEDs). Treatment with pyridoxine terminates seizures and prevents neurological decline. We describe a case in which the diagnosis was established at the age of 22 years. Birth and development were normal, but there was a history of three isolated tonic-clonic seizures during childhood and adolescence. At the age of 18 years, she developed frequent focal motor seizures, many evolving into tonic-clonic seizures. Electroencephalography identified a focus in the posterior right hemisphere, but magnetic resonance imaging of the brain was normal. Over the next 3 years, she was hospitalized with uncontrolled seizures on six occasions and spent a total of 121 days in intensive care. The seizures proved resistant to 12 different AEDs. Exome sequencing revealed two pathogenic mutations in ALDH7A1. Since starting on pyridoxine 50 mg once daily, she has been seizure-free, all AEDs have been withdrawn, and cognition has improved to premorbid levels.This case illustrates the importance of considering PDE in drug-resistant epilepsy in adults. K E Y W O R D Sgenetic, metabolic, pyridoxine e2 | OSMAN et Al.

In a great majority of patients with systemic lupus erythematosus (SLE) lymphocytotoxic antibodies (LCA) are detected. The reported prevalence depends on the methods of detection and definition of test-positivity. The pathogenetic role of LCA remains unclear. Different reports show that LCA can have an influence on mononuclear cell function, e.g., on production of interferon, or specifically on T-cell function. In several studies T-cell specificity of LCA cannot be shown. Sometimes an overlap between LCA and anti-B2M antibodies is found, suggesting that LCA have anti-B2M activity. Anti-B2M activity might have an impact on T-cell as well as on B cell function. Next to the anti-B2M activity, binding to nuclear material is claimed. Investigating the role of LCA in respect to lymphopenia, a direct relationship cannot be found; however, LCA might induce interferon production, resulting in lymphopenia. Several studies show or claim a relationship between the presence of LCA and neurological manifestations in SLE patients; the results, however, remain questionable due to the difference in detection methods as well as in definition of central nervous system (CNS) involvement. In other studies, an increased incidence of LCA is reported in relatives of SLE patients as well. In addition, LCA are reported in quite a lot of other diseases such as rheumatoid arthritis, ankylosing spondylitis, malignancies and viral illnesses. This latter association has led to the assumption that LCA might have a virus-related origin.