Inverse bicontinuous cubic structures formed by lipids have been demonstrated in a wide variety of applications, from a host matrix for proteins for crystallisation, to templates for nanoscale structures. Recent work has focused on tuning their properties to realize such applications, often by manipulating the structure by introducing other lipids with different properties such as charge or packing. However, they are often prepared in the presence of solutions containing salt, counteracting the effects, for example, charged lipids, and fundamentally changing the structures obtained. Here, we demonstrate the delicate interplay between electrostatic swelling in bicontinuous structures formed by monoolein (MO) doped with both negatively charged dioleyl phosphatidylglycerol (DOPG), and zwitterionic dioleyl phosphatidylethanolamine (DOPE), with the addition of mono- and divalent salts. The effect of adding salt to the charged phase changes the structure from the primitive cubic () to the double diamond phase () whilst still allowing for modest increases in lattice parameter of up to a nanometer. Contrasting this, the addition of salts to the non-charged phase, has minimal effect on the lattice parameter but now the transition from the () to the inverse hexagonal phase (HII) is observed occurring at higher mole fractions of DOPE than in pure water.

BackgroundPre-treatment screening for IgA deficiency and close monitoring of full blood count (FBC) and urea and electrolytes (U&E) is recommended with intravenous immunoglobulin (IVIg) therapy in neurological diseases. AimsTo examine the frequency of biochemically defined and clinically significant episodes of treatment associated haemolysis, neutropenia, thrombocytopenia and acute renal impairment in a cohort of patients on maintenance Immunoglobulin (Ig) therapy for inflammatory neuropathy. MethodsA retrospective review of routine blood monitoring in a cohort of patients from two UK specialist peripheral nerve centres. Accepted definitions for clinically and biochemically significant haemolysis, neutropenia, thrombocytopenia and acute kidney injury (AKI) were used.Results 1919 infusion episodes in 90 patients were analysed. Age (mean(S.D))= 58.09(14.4)years, 63% male, 72% CIDP (28% MMN), 97% IVIg (3% SCIg). Dose= 1.57 (0.79) g/kg/month or 97.1(37.3)g/infusion, frequency: 3.9 (1.4) weeks.Relative IgA deficiency was noted in 2 individuals (prevalence: 2.2%, 95% C.I.: 0-5.2) who received a combined total of 38 infusions (3800g IVIg) without adverse event. No clinically significant episodes of haemolysis, neutropenia, thrombocytopenia or AKI occurred in relation to treatment. An asymptomatic drop>10g/L haemoglobin (Hb) occurred in 3.5% (95% CI: 2.7-4.3) of treatment episodes in 38 individuals, mean reduction: 17.7(7.4)g/L; lowest Hb: 86g/L. Lower pre-treatment haemoglobin correlated with risk of recurrent episodes Ig-related drop (p:0.007). Two patients with chronic renal failure (stage 1 and 3) had received 28 (IV) and 104 (SC) infusions respectively, (6416g Ig) without impact on estimated glomerular filtration rate (eGFR). ConclusionsNo clinically significant Ig-related events were identified in this representative cohort. We suggest annual screening or clinically indicated testing as safe and more appropriate in long-term Ig use. Screening for IgA deficiency and baseline FBC and U&E may be helpful in identifying those at greater risk of complication.

Background and purpose: This study was undertaken to explore associations between plasma neurofilament light chain (pNfL) concentration (pg/ml) and disease activity in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and examine the usefulness of pNfL concentrations in determining disease remission. Methods:We examined pNfL concentrations in treatment-naïve CIDP patients (n = 10) before and after intravenous immunoglobulin (IVIg) induction treatment, in pNfL concentrations in patients on maintenance IVIg treatment who had stable (n = 15) versus unstable disease (n = 9), and in clinically stable IVIg-treated patients (n = 10) in whom we suspended IVIg to determine disease activity and ongoing need for maintenance IVIg. pNfL concentrations in an age-matched healthy control group were measured for comparison.Results: Among treatment-naïve patients, pNfL concentration was higher in patients before IVIg treatment than healthy controls and subsequently reduced to be comparable to control group values after IVIg induction. Among CIDP patients on IVIg treatment, pNfL concentration was significantly higher in unstable patients than stable patients. A pNFL concentration > 16.6 pg/ml distinguished unstable treated CIDP from stable treated How to cite this article: Kapoor M, Carr A, Foiani M, et al. Association of plasma neurofilament light chain with disease activity in chronic inflammatory demyelinating polyradiculoneuropathy.

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Regular immunoglobulin treatment maintains strength and prevents disability in chronic inflammatory demyelinating polyneuropathy (CIDP). Discrimination between active disease, with optimum symptom control on treatment, and disease in remission not requiring treatment is essential for therapeutic decision-making and clinical trial design. To compare treatment cessation versus gradual dose reduction in assessment of disease activity (immunoglobulin dependence) in a cohort of stable CIDP patients on maintenance immunoglobulin treatment. An approach to restabilization of immunoglobulin-dependent individuals is also described. Retrospective review of IVIg cessation or gradual reduction in 33 patients with stable CIDP on maintenance IVIg. Demographic, clinical and treatment data were collected; clinical monitoring data were recorded prospectively as part of routine clinical practice. A total of 21/33 patients (62.6%) were immunoglobulin dependent, (gradual dose reduction:11, cessation:10). Mean change in Inflammatory Rasch-built Overall Disability Scale (I-RODS) (À15, standard deviation [SD] 16) and Medical Research Council Sum Score (MRC-SS) (À4, SD: 4) was clinically and statistically meaningful (>75% exceeded minimum clinically important differences). Mean time to deterioration was 5.0 (SD: 4.6) months, shorter in cessation group (3.5 months) than gradual reduction group (8.8 months). All patients were restabilized to previous baseline (M: 2.3, SD: 4.3 months), half within 1 week of retreatment. A total of 12 patients (37.4%) remained stable without treatment for ≥2 years (remission). A total of 50% were identified rapidly by cessation and 50% by gradual dose reduction requiring mean 4.8 (SD: 2.8) years follow-up and costing £113 623 per person Ig spend. No predictors of disease activity were identified. A treatment cessation trial with close clinical monitoring is an efficient, cost-effective and safe approach to assessing disease activity in CIDP.