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Introduction/Aims: In myasthenia gravis, prolonged muscle denervation causes muscle atrophy. We re-visited this observation using a biomarker hypothesis. We tested if serum neurofilament heavy chain levels, a biomarker for axonal degeneration, were elevated in myasthenia gravis. Methods: We enrolled 70 patients with isolated ocular myasthenia gravis and 74 controls recruited from patients in the emergency department. Demographic data were collected alongside serum samples. Serum samples were analyzed by enzyme-linked immunosorbent assay (ELISA) for the neurofilament heavy chain (NfH-SMI35). The statistical analyses included group comparisons, receiver operator characteristic (ROC) curves, area under the curve (AUC), sensitivity, specificity, and positive and negative predictive values. Results: Serum neurofilament heavy chain levels were significantly (p < 0.0001) higher in individuals with myasthenia gravis (0.19 ng/mL) than in healthy control subjects (0.07 ng/mL). A ROC AUC optimized cutoff level of 0.06 ng/mL gave a diagnostic sensitivity of 82%, specificity of 76%, positive predictive value of 0.77 and a negative predictive value of 0.81. Discussion: The increase of serum neurofilament heavy chain levels in myasthenia gravis is consistent with observations of muscle denervation. We suggest that there is ongoing remodeling of the neuromuscular junction in myasthenia gravis. Longitudinal quantification of neurofilament isoform levels will be needed to investigate the prognostic value and potentially guide treatment decisions.

We came together in March 2021 for a joyous celebration of Dr Gordon Plant's incredible career. This is a report of the meeting tidings.

BACKGROUND There is currently no prognostic test to determine the risk of developing generalized myasthenia gravis (GMG) risk in patients who first present with ocular disease. Most studies that report risk factors are flawed by the inclusion of patients on immunosuppression, which is likely to reduce the risk. OBJECTIVE To create a prognostic score to predict the risk of GMG. METHODS Multicenter retrospective cohort of patients with ocular myasthenia gravis for minimum 3 months, untreated with immunosuppression for minimum 2 years or until GMG onset. RESULTS One hundred one (57 female) patients were included, with median follow-up of 8.4 years (2-42) from disease onset. Thirty-one developed GMG at median of 1.31 years (3.5 months-20.2 years); 19 occurred within 2 years. Univariable logistic regression analysis produced 3 significant predictors (P < 0.10), adjusted odds ratios in a multivariable logistic model (χ P = 0.01) with multiple imputations for missing data: seropositivity, 5.64 (95% CI, 1.45-21.97); presence of 1 or more comorbidities including autoimmune disorders, 6.49 (95% CI, 0.78-53.90); thymic hyperplasia, 5.41 (95% CI, 0.39-75.43). Prognostic score was derived from the coefficients of the logistic model: sum of the points (1 point for the presence of each of the above predictive factors), classified "low risk" if ≤1 and "high risk" if ≥2. Predicted probabilities were 0.07 (SD, 0.03) for low risk and 0.39 (SD, 0.09) for high risk. Negative predictive value was 91% (95% CI, 79-98), positive predictive value was 38% (95% CI, 23-54), sensitivity was 79% (95% CI, 54-94), specificity was 63% (95% CI, 50-74), area under receiver operating characteristic curve was 0.74 (95% CI, 0.64-0.85). CONCLUSIONS In this preliminary study, we have shown by proof of principle that it is possible to stratify risk of GMG: an approach that may allow us to better counsel patients at diagnosis, complement decision-making, and move us toward addressing the question of modifying GMG risk in high-risk patients. Furthermore, the effect of comorbidities is novel and demands further elucidation.

We highlight an under-recognised cause of acquired esotropia with this prospective observational case series of adults with diplopia secondary to cerebellar dysfunction. We also show deterioration of cerebellar esotropia over time, which has not been previously described. Seven adults (four women) developed diplopia at a median age of 63 years (range: 31–75 years), as the initial manifestation of the underlying cerebellar disorder. Causes of cerebellar dysfunction were familial cerebellar ataxia of unknown mutation (two patients), idiopathic cerebellar ataxia (four patients), and spinocerebellar ataxia 3 (one patient). At onset, three patients had unilateral and four had bilateral lateral rectus under-action. These were initially diagnosed as lateral rectus paresis, but the diagnosis was revised, as our examination showed no slowing of abducting saccades assessed clinically and full abduction with gaze-evoked nystagmus. Esotropia was concomitant and worse for distance, although at onset one patient’s esotropia was equal for near and distance. There was a trend of worsening esotropia over time, following a median interval follow-up of 4 years (range: 1–18). All patients were first observed to have cerebellar eye signs after a median interval of 5 years (range: 1–30) from presentation, i.e., impaired pursuit (7/7 patients), gaze-evoked nystagmus (7/7), hypometric saccades (3/7), downbeat nystagmus (2/7), and skew deviation (4/7). Only two patients have not developed non-ocular cerebellar eye signs, after 5 and 8 years from diplopia onset, respectively; the other five patients had gait ataxia, which could be mild. The patients were successfully treated with prisms (7/7), botulinum toxin injections (1/7), and strabismus surgery (1/7).

We introduce the ABN Sustainability SIG. We present our aims & objectives, and practical ways of implementing sustainability strategies in Neurology.At the 2016 ABN annual meeting, a keynote speech by Dr. David Pencheon, then director of the National Health Service Sustainability Development Unit, highlighted the relevance and importance of Sustainability in Neurology. This planted the seed for our SIG’s formation. Initial interest was gathered from an ABN newsletter notice, via word-of-mouth and informal discussions at ABN annual meetings (2017, 2018). A series of teleconferences & email discussions enabled the formation of SIG byelaws and application to the ABN council.Our aims and objectivesTo be a positive force within the ABN to highlight issues surrounding global environmental sustainability.To provide a forum to consider the impact of choices made in neurology practice on global environmental sustainability. This will cover all aspects of neurology including, but not limited to, clinical practice, service provision, technological and digital developments, meetings and ABN resources and investments.To identify areas where the choice made could impact positively on global environmental sustainability and disseminate this information to the ABN membership to inform their decisions.

Ocular Myasthenia Gravis (OMG) causes ptosis and diplopia, which can be disabling. For this study, OMG is defined as MG patients who have ocular symptoms only and no generalised involvement. A robust way of assessing the severity of OMG symptoms is important for research, to assess treatment and outcome. The rating scales recommended for MG research have a predominant focus on generalised disease, and are insufficiently sensitive for OMG due to the limited number of ocular questions. This study aims to create a new rating scale for OMG that is sensitive, reliable and clinically usable. We present our proposal of such a rating scale, which incorporates physician- and patient-rated components. We report the preliminary results of this pilot observational cohort study, in 60 patients with OMG. We compare the results of this with the MG composite scale.Future work is planned to validate this rating scale and to develop this alongside the MG Impairment Index (MGII). For future phases of this study we plan to assess the usability of this rating scale by Neurologists without specialist Neuro-ophthalmology.We invite feedback from Neurologists at the ABN.

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.

Imagine you are assessing a patient with visual difficulties or optic disc swelling. After a bedside visual field examination with waggling fingers and even a red hatpin, you decide that there is an abnormality. After requesting quantified visual field tests, the patient returns with a black and white printout with numbers (eg, Humphrey fields) or coloured lines on a sheet (eg, Goldmann fields). Where is the report you ask? There is none! Static perimetry uses flashing stationary lights. This can be automated (eg, evenly spaced points on a grid) or manual (eg, as a small part of Goldmann test: detailed later). The Humphrey field analyser is by far the most commonly used for automated static perimetry, although there are also other machines such as Octopus and Henson. Later, we describe in detail the interpretation of Humphrey perimetry. Kinetic perimetry uses a moving illuminated target and is done either manually (eg, Goldmann) or on an automated machine (eg, Octopus). Goldmann machines are no longer manufactured, being slowly replaced by Octopus machines. Nevertheless, Goldmann remains the most commonly used kinetic perimetry, and so we use this here to illustrate interpretation of kinetic fields. The principles for interpreting Goldmann also apply to results from Octopus machines. It is beyond the scope of this paper to cover the neuroanatomical localisation of visual field defects. Instead we recommend two excellent recent reviews.1 ,2 Skilled interpretation of visual field tests requires a good grasp and application of this prior knowledge. ### Useful aspects of eye anatomy 1. The fovea is the area of greatest visual sensitivity, where the cone photoreceptor density is at its highest. The visual sensitivity slopes off further from the fovea. This drop in sensitivity can be visualised as a hill, with the fovea is at the peak (figure 1). Conventional perimetry is carried out under photopic (well …

The majority of patients with myasthenia gravis (MG) initially present with ocular symptoms. An unresolved question is whether there are clinical features at onset to guide clinicians to predict an individual patient's conversion risk from ocular MG (OMG) to generalized disease, or "secondary generalized MG" (SGMG), that is, a prognostic model. In light of the emerging theory that early corticosteroids may have a risk-modifying effect, the factors associated with secondary SGMG previously reported should be revisited. Studies showing potential risk-modifying effects of corticosteroids are useful, though flawed, owing to the heterogeneous retrospective studies and methods of reporting. Updates on other potential immunosuppressive agents are also discussed. Thymectomy in OMG has been recently reported in a few studies to be useful. MG associated with antibodies to muscle-specific kinase, usually associated with severe generalized MG, can cause a pure OMG syndrome. Recent serological developments in seronegative patients have also revealed antibodies to clustered anti-acetylcholine receptor and lipoprotein receptor-related protein-4.

A 52-year-old man with 3 years of progressive unsteadiness admitted to previous alcohol excess but denied ongoing consumption. Magnetic resonance spectroscopy (MRS) (figure) was performed looking for changes of subclinical hepatic encephalopathy, which was not found. Instead, …

Do you often get frustrated by the lack of neurological examination equipment on the wards? Are your residents’ backs breaking from lugging around their neurological “case”, weighing in excess of 5 kg?1 Is your ward manager complaining of the never-ending cost of repurchasing lost ophthalmoscopes? If so, read on … this solution might work for your unit! The Walton Centre is a stand-alone Neurology and Neurosurgery Hospital, with four in-patient wards. An audit showed that over a period of three years, 18 ophthalmoscopes were purchased at a total cost of £2283, 50 tuning forks at £1000 and 30 tendon hammers at £40. Despite this, at the time of the audit, there were only two tendon hammers, two ophthalmoscopes (one locked away in the ward sister’s office) and one otoscope, shared between the four wards; furthermore, these were only found after at least 30 …