Background Voltage-gated potassium channel antibody-positive limbic encephalitis (VGKC+LE) frequently improves with immunotherapy, although the optimum regimen is unknown. The effectiveness of a combination immunomodulatory regimen was tested in consecutive VGKC+LE patients. Methods This was an open-label prospective study of nine VGKC+LE patients. All patients had plasma exchange (50 ml/kg), intravenous immunoglobulin (2 g/kg) and intravenous methylprednisolone (1 g×3), followed by maintenance oral prednisolone (1 mg/kg/day). Mycophenolate (2 g/day) was used in the first three patients. Assessments included serial clinical, cognitive, brain MRI and VGKC antibody testing. Results Within 1 week, seizures and hyponatraemia remitted in all affected patients. Cognitive function improved in all patients within 3 months. MRI appearances improved substantially within 9 months, with remission of inflammation in the majority of patients. All achieved immunological remission with normal VGKC antibody titres within 1–4 months. Major adverse events of therapy included one septicaemia and one thrombosis on plasma exchange and one death from sepsis after incidental bowel surgery. One patient remains in remission after 40 months of follow up, 26 months after being off all treatment. Conclusions Our immunotherapy regimen was effective for the treatment of the clinical, cognitive and immunological features of VGKC+LE. Radiological improvement was seen in the majority. Pending randomised controlled trials, this regimen is proposed for the treatment of VGKC+LE.

Background and Objectives Idiopathic intracranial hypertension (IIH) is associated with obesity, however there is a lack of clinical consensus on how to manage weight in IIH. The aim of this systematic review is to evaluate weight loss interventions in people with IIH to determine which intervention is superior in terms of weight loss, reduction in intracranial pressure, benefit to visual and headache outcomes, quality of life, and mental health. Methods A systematic review was carried out in accordance with PRISMA guidelines and registered with PROSPERO (CRD42023339569). MEDLINE and CINAHL were searched for relevant literature published from inception until 15^th December 2022. Screening and quality appraisal was conducted by two independent reviewers. Recommendations were graded using Scottish Intercollegiate Guidelines Network (SIGN) methodology. Results A total of 17 studies were included. Bariatric surgery resulted in 27.2-27.8kg weight loss at 24 months (Level 1- to 1++). Lifestyle weight management interventions resulted in between 1.4 to 15.7kg weight loss (Level 2+ to 1++). Bariatric surgery resulted in the greatest mean reduction in ICP (-11.9cm H₂O) at 24 months (Level 1++), followed by multi-component lifestyle intervention + acetazolamide (-11.2cm H₂O) at 6 months (Level 1+), and then a very low energy diet intervention (-8.0cm H₂O) at 3 months (Level 2++). The least ICP reduction was shown at 24 months after completing a 12-month multi-component lifestyle intervention (-3.5 cm H₂O) (Level 1++). Reduction in body weight was shown to be highly correlated with reduction in ICP (Level 2++ to 1++). Discussion Bariatric surgery should be considered for women with IIH and a BMI ≥35kg/m² since this had the most robust evidence for sustained weight management (Grade A). A multi-component lifestyle intervention (diet + physical activity + behaviour) had the most robust evidence for modest weight loss with a BMI <35kg/m² (Grade B). Longer term outcomes for weight management interventions in people with IIH are required, to determine if there is a superior weight loss intervention for IIH.

Background: The validity of the clinical dictum “the presence of spontaneous retinal venous pulsation (SVP) excludes raised intracranial pressure” has not been previously tested. We set out to determine the specificity and positive predictive value (PPV) of the presence of SVP to indicate normal intracranial pressure (ICP) in a routine clinical setting. Methods: We prospectively recruited patients undergoing lumbar puncture (LP), and 2 clinicians were blinded to the indications for LP and cerebrospinal fluid opening pressure (OP). Interobserver reliability was assessed. Results: There were 106 patients in our cohort with a median age of 44 years (range, 18–79 years) and median body mass index of 27.5 kg/m² (range, 18–48 kg/m²). SVP was present in 94 of 106 patients (88.7%). Thirteen of 106 (12.3%) patients had high OP (≥30 cmH₂O), and SVP was present in 11 of 13 patients (86%) with high OP. The sensitivity (95% confidence interval) of the presence of SVP to exclude raised ICP was 0.89 (0.88–0.92), specificity of 0.15 (0.05–0.37), PPV of 0.88 (0.87–0.9), and negative predictive value of 0.17 (0.05–0.4). Interobserver agreement was moderate for SVP (kappa = 0.42). Conclusions: Although the sensitivity and PPV of the presence of SVP to exclude raised ICP is high, it is not absolute. SVP can be seen in some patients with high ICP. Relying on the presence of SVP to exclude raised ICP may give a false sense of reassurance.

Background and Methods: To evaluate the value of brain biopsy for neurology patients in our unit, we conducted a retrospective audit of neurology patients referred for brain biopsies for non-neoplastic disease from 1993 to 2007. Results: 64 patients [median (range) age 51 (16–74) years] were included. The clinical presentation was diffuse encephalopathy for 40 patients, focal for 13 and multifocal for 11. The biopsy was diagnostic in 34 patients, abnormal but non-diagnostic in 21 and normal in 9. There was a statistically significant association between clinical presentation and biopsy result (p = 0.004); diagnostic biopsies were more common in patients with focal or multifocal clinical presentations. Twenty patients (31%) had alterations of management as a result of their brain biopsy, comprising specific treatment (11 patients) or prognosis/diagnosis of untreatable conditions (9 patients). Diagnoses of treatable conditions included Whipple’s disease, tuberculoma, progressive multifocal leukoencephalopathy, and neurosarcoidosis. Five patients (7.8%) had complications. Conclusions: Brain biopsy is useful and relatively safe in the management of neurology patients, with a diagnostic yield of 53% in our series; some led to significant changes in management, including treatment of infections. Patients with focal or multifocal presentation are more likely to yield a diagnostic biopsy result.

Background Ocular myasthenia gravis (OMG) is an autoimmune disorder resulting in ocular symptoms such as diplopia and ptosis. The proportion of patients who convert to secondary generalised myasthenia gravis (SGMG) reported in the literature has been varied. The aim of this systematic review was to determine the clinical characteristics of patients with OMG and determine on the proportion of SGMG conversion. Methods We conducted an electronic database search for randomised controlled trials, prospective non-randomised studies, observational studies and retrospective studies in EMBASE, CENTRAL, MEDLINE and Web of Science. We included studies with patients with OMG who initially presented with ocular symptoms and signs only who were seen in clinical practice, reporting on characteristics and the outcomes of SGMG. We excluded studies with paediatric and congenital myasthenia gravis populations. Eligibile studies included articles written in any language and containing data on patients with OMG. Main outcome measured was the proportion of patients with OMG who converted to SGMG and risk factors associated with secondary generalisation of OMG. Two independent reviewers screened titles and abstracts and extracted data from full texts, reporting findings according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Methodology was evaluated using the Joanna Briggs Institute critical appraisal forms. PROSPERO registration number: CRD2021285257 Results 31 studies were included in the quantitative and qualitative analysis. The proportion of generalisation ranged from 11 to 84%. The pooled proportion was 39% (95% CI 32%, 47%, I2=95·86%, p<0·001 unweighted, low certainty). Pooled risk ratio of female gender for conversion to SGMG was 1.06 (95%CI 0·96,1·17, I2=0% p=0·614, 21 studies included, very low certainty) and pooled risk ratio of AChR positivity was 1·30 (95%CI 1·05,1·56, I2=0% p=0·455, 16 studies included, very low certainty). Discussion Risk factors such as female gender and anti-AChR positivity have been identified as possible associations with SGMG but there are not enough quality observational studies. There is a need for a prospective global database of OMG patients, including all countries with different populations.