Weight Management Interventions for Adults With Idiopathic Intracranial Hypertension: A Systematic Review and Practice Recommendations.

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 was to evaluate weight loss interventions in people with IIH to determine which intervention is superior in terms of weight loss, reduction in intracranial pressure (ICP), benefit to visual and headache outcomes, quality of life, and mental health. METHODS: A systematic review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and registered with PROSPERO (CRD42023339569). MEDLINE and CINAHL were searched for relevant literature published from inception until December 15, 2022. Screening and quality appraisal was conducted by 2 independent reviewers. Recommendations were graded using Scottish Intercollegiate Guidelines Network methodology. RESULTS: A total of 17 studies were included. Bariatric surgery resulted in 27.2-27.8 kg weight loss at 24 months (Level 1- to 1++). Lifestyle weight management interventions resulted in between 1.4 and 15.7 kg weight loss (Level 2+ to 1++). Bariatric surgery resulted in the greatest mean reduction in ICP (-11.9 cm H2O) at 24 months (Level 1++), followed by multicomponent lifestyle intervention + acetazolamide (-11.2 cm H2O) at 6 months (Level 1+) and then a very low-energy diet intervention (-8.0 cm H2O) at 3 months (Level 2++). The least ICP reduction was shown at 24 months after completing a 12-month multicomponent 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 body mass index (BMI) ≥35 kg/m2 since this had the most robust evidence for sustained weight management (grade A). A multicomponent lifestyle intervention (diet + physical activity + behavior) had the most robust evidence for modest weight loss with a BMI <35 kg/m2 (grade B). Longer-term outcomes for weight management interventions in people with IIH are required to determine whether there is a superior weight loss intervention for IIH.

Clinical Characteristics Associated With Secondary Generalization in Patients With Ocular Myasthenia Gravis: A Systematic Review and Meta-analysis.

BACKGROUND AND OBJECTIVES: 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 generalized 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 the proportion of SGMG conversion. METHODS: We conducted an electronic database search for randomized controlled trials, prospective nonrandomized 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 and were seen in clinical practice, reporting on the characteristics and outcomes of SGMG. We excluded studies with pediatric and congenital myasthenia gravis populations. Eligible studies included articles written in any language and containing data on patients with OMG. The main outcome measured was the proportion of patients with OMG who converted to SGMG and risk factors associated with secondary generalization of OMG. Two independent reviewers screened titles and abstracts and extracted data from full texts, reporting findings according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The methodology was evaluated using the Joanna Briggs Institute critical appraisal forms. PROSPERO registration number: CRD2021285257. RESULTS: Thirty-one studies were included in the quantitative and qualitative analysis. The proportion of generalization ranged from 11% to 84%. The pooled proportion was 39% (95% CI 32%-47%, I 2 = 95.86%, p < 0.001 unweighted, low certainty). The pooled risk ratio of female sex for conversion to SGMG was 1.06 (95% CI 0.96-1.17, I 2 = 0% p = 0.614, 21 studies included, very low certainty), and the pooled risk ratio of acetylcholine receptor (AChR) positivity was 1.30 (95% CI 1.05-1.56, I 2 = 0% p = 0.455, 16 studies included, very low certainty). DISCUSSION: Risk factors such as female sex and anti-AChR positivity have been identified to have possible associations with SGMG, but there are not enough quality observational studies. There is a need for a prospective global database of patients with OMG, including all countries with different populations.

Anticholinergic syndrome: blurred vision and headache.

A young woman presented with blurred vision due to anticholinergic syndrome. We highlight the importance of considering this condition in the context of multiple medications and increased anticholinergic burden. The documented pupil abnormality gives an opportunity to review the syndrome of the reverse (inverse) Argyll Robertson pupil (preserved pupil light response with loss of accommodation). We review other situations in which the reverse Argyll Robertson pupil may occur and its possible mechanism in this case.

Prevalence and utility of overnight pulse oximetry as a screening tool for obstructive sleep apnoea in newly diagnosed idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP). Obstructive sleep apnoea (OSA) has been shown to cause episodic rises in ICP and is frequently reported in patients with IIH. The aim of this study is to identify the prevalence of OSA in a cohort of IIH patients. METHODS: We conducted a retrospective case notes review as part of a service evaluation of newly diagnosed IIH patients who were all referred for OSA screening with overnight pulse oximetry. The 3% oxygen desaturation index (3% ODI) was used to evaluate the presence and severity of OSA. The clinical outcomes of patients who received continuous positive airway pressure (CPAP) therapy as treatment for OSA were reviewed. RESULTS: In our cohort of newly diagnosed IIH patients, the yield of overnight pulse oximetry as a screening tool was 48.6% for OSA and 15.3% for moderate to severe OSA. We found that age (p = 0.0008), BMI (p < 0.0001), vitamin B12 (p = 0.0183), and a higher Epworth Sleep Score (p = 0.0269) correlated with more severe OSA. Eleven (10%) patients had CPAP therapy and those with good adherence alongside weight loss or medical therapy found improvements in symptoms of raised ICP. CONCLUSION: We report the largest series of consecutive IIH patients screened for OSA using overnight pulse oximetry. The high rate of OSA highlights a potential role for the recognition and management of OSA in the IIH patient cohort. Further studies on the potential contribution of OSA as a cause of raised ICP in the IIH cohort is warranted.

Clinical Signs in Neuro-Ophthalmology: Eye Signs in Myasthenia Gravis.

A hallmark of myasthenia gravis (MG) is the variability and fatigability of striated muscles. The majority of people with MG have eye symptoms of ptosis and diplopia. This paper outlines the eye signs in MG, including practical tips on the examination technique relevant to MG, and pitfalls to avoid.

Preliminary Findings of a Dedicated Ocular Myasthenia Gravis Rating Scale: The OMGRate.

There is a pressing need for a robust rating scale for ocular myasthenia gravis (OMG). Rating scales for myasthenia gravis (MG) research have a predominant focus on generalised disease. We present results of the first dedicated rating scale for OMG: the ocular myasthenia gravis rating scale (OMGRate). The OMGRate was developed through an international collaboration between neuromuscular and neuro-ophthalmology experts in OMG. It comprises two components: a physician- examination (OMGRate-e) and a patient questionnaire (OMGRate-q).. The OMGRate was prospectively validated in patients attending a neuro-ophthalmology clinic from April 2017 to October 2018. External validity and reliability of OMGRate were evaluated using validated MG rating scales: the Myasthenia Gravis Composite (MGC), the Myasthenia Gravis Quality of Life (MG-QOL), and the ocular component from the Myasthenia Gravis Impairment Index questionnaire (MGII). Two hundred and eleven assessments were completed in 104 patients (67 males, mean age 55 y, range 18-86 y). There was very good external validity of the OMGRate: good correlation between OMGRate-e and MGC (r = 0.64, 95% confidence intervals [CI] 0.54-0.74, p < .0001); excellent correlation between OMGRate-q and MGII (r = 0.85, 95% CI 0.78-0.91, p < .0001) and good correlation between OMGRate and MG-QOL (r = 0.68, 95% CI 0.60-0.77, p < .0001). A higher correlation of OMGRate and MG-QOL compared with MGC and MG-QOL (r = 0.47, 95% CI 0.34-0.59, p < .0001) suggests that OMGRate is better able to capture significant QOL information in patients with OMG. It had excellent reliability with an intraclass correlation coefficient of 0.83 (95% CI 0.67-0.92). Feedback from examiners and patients indicated that the OMGRate was easy to use. In conclusion, OMGRate is an easy-to-use, valid and reliable rating scale for monitoring the severity of OMG.

Pituitary apoplexy initially mistaken for bacterial meningitis.

We presented a case of a 62-year-old man whose initial clinical picture was suggestive of bacterial meningitis, but instead had pituitary apoplexy. We highlighted how pituitary apoplexy can mimic bacterial meningitis, learning points on how clinical assessment can aid earlier diagnosis and the importance of considering this differential diagnosis, particularly with the associated morbidity and mortality if missed.

The clinical validity of the spontaneous retinal venous pulsation.

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/m (range, 18-48 kg/m). 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.

The electrocardiograph (ECG) in a first seizure clinic.

An electrocardiograph (ECG) is recommended as an investigation in all patients with seizures or suspected seizures. However the incidence of abnormal ECGs in patients presenting to the first seizure clinic is unknown. This study examines 161 patients in a first seizure clinic and describes the ECG abnormalities found. Although 17 patients were found to have abnormal ECGs only one was found to have significant cardiac disease. We conclude that serious ECG abnormalities are rare but other abnormalities are common and that close cooperation between cardiology and neurology is important.