Cemiplimab-Induced Hyperosmolar Hyperglycemic State With Concurrent Diabetic Ketoacidosis in a Patient Receiving Treatment for Cutaneous Squamous Cell Carcinoma.

The immune checkpoint inhibitor (ICI) cemiplimab is a human monoclonal antibody used in the treatment of locally advanced and metastatic cutaneous squamous cell carcinoma (CSCC) not amenable to surgery or radiation therapy. Although cemiplimab shows excellent efficacy with a good tolerability profile, it can cause side effects, including potentially life-threatening endocrinopathies. We discuss the case of a 77-year-old Caucasian female with CSCC treated with only three cycles of cemiplimab who presented with altered mental status and was found to have severe hyperglycemia, hyperosmolarity, ketonemia, glucosuria, and ketonuria concerning for hyperosmolar hyperglycemic syndrome (HHS) with concurrent diabetic ketoacidosis (DKA).

A natural history museum visitor survey of perception, attitude and knowledge (PAK) of microbes and antibiotics.

A kiosk-based survey at the American Museum of Natural History in New York City in 2016-2018 allowed us to assess public knowledge of antibiotics and public attitudes toward microbes in museum goers. Over 22,000 visitors from 172 countries and territories answered several carefully designed questions about microbes and antibiotics. These visitors also entered age, gender, and country demographic data that allowed for stratification along these demographic and geographic divisions.

A kiosk survey of perception, attitudes and knowledge (PAK) of Australians concerning microbes, antibiotics, probiotics and hygiene.

Issues Addressed: To obtain a baseline of public perception, attitudes and knowledge (PAK) of Australians about microbes, antibiotics and hygiene like hand washing and use of probiotics.

Methods: Using a kiosk-based survey method at the American Museum of Natural History (AMNH), we remotely assayed PAK of Australians through their interaction with the kiosk. The surveys we used had five and seven multiple answer questions and were analysed using standard comparative approaches.

Assessing Instructional Cognitive Load in the Context of Students' Psychological Challenge and Threat Orientations: A Multi-Level Latent Profile Analysis of Students and Classrooms.

To better understand instructional cognitive load, it is important to operationalize and assess it in novel ways that can reveal how different students perceive and experience this load as either challenging or threatening. The present study administered a recently developed instruction assessment tool-the Load Reduction Instruction Scale-Short (LRIS-S)-to = 2,071 students in 188 high school science classrooms. Multilevel latent profile analysis (LPA) was used to identify student and classroom profiles based on students' reports of instructional cognitive load (load reduction instruction, LRI; using the LRIS-S) and their accompanying psychological challenge orientations (self-efficacy and growth goals), and psychological threat orientations (anxiety and failure avoidance goals).

Boarding and Day School Students: A Large-Scale Multilevel Investigation of Academic Outcomes Among Students and Classrooms.

Boarding school is a major educational option for many students (e.g., students living in remote areas, or whose parents are working interstate or overseas, etc.

LGI1-antibody encephalitis is characterised by frequent, multifocal clinical and subclinical seizures.

Purpose: To describe clinical and electrographic characteristics of seizures LGI1-antibody encephalitis, and their correlations with two-year outcomes.

Methods: Video-electroencephalography recordings were performed on a cohort of 16 consecutive patients with LGI1-antibodies from two UK neuroscience-centers over five-years.

Results: From 14 of 16 patients (13 males; age-range 53-92years), 86 faciobrachial dystonic seizures were recorded at a median frequency of 0.

Prevalence of HPV Infection in Racial-Ethnic Subgroups of Head and Neck Cancer Patients.

The landscape of HPV infection in racial/ethnic subgroups of head and neck cancer (HNC) patients has not been evaluated carefully. In this study, a meta-analysis examined the prevalence of HPV in HNC patients of African ancestry. Additionally, a pooled analysis of subject-level data was also performed to investigate HPV prevalence and patterns of p16 (CDNK2A) expression amongst different racial groups.

The role of digital data entry in participatory environmental monitoring.

Many argue that monitoring conducted exclusively by scientists is insufficient to address ongoing environmental challenges. One solution entails the use of mobile digital devices in participatory monitoring (PM) programs. But how digital data entry affects programs with varying levels of stakeholder participation, from nonscientists collecting field data to nonscientists administering every step of a monitoring program, remains unclear.

Clinical features of the myasthenic syndrome arising from mutations in GMPPB.

Background: Congenital myasthenic syndrome (CMS) due to mutations in GMPPB has recently been reported confirming the importance of glycosylation for the integrity of neuromuscular transmission.

Methods: Review of case notes of patients with mutations in GMPPB to identify the associated clinical, neurophysiological, pathological and laboratory features. In addition, serum creatine kinase (CK) levels within the Oxford CMS cohort were retrospectively analysed to assess its usefulness in the differential diagnosis of this new entity.

Mutations in GMPPB cause congenital myasthenic syndrome and bridge myasthenic disorders with dystroglycanopathies.

Congenital myasthenic syndromes are inherited disorders that arise from impaired signal transmission at the neuromuscular junction. Mutations in at least 20 genes are known to lead to the onset of these conditions. Four of these, ALG2, ALG14, DPAGT1 and GFPT1, are involved in glycosylation.

Isolated paediatric neurosarcoidosis presenting as epilepsia partialis continua: a case report and review of literature.

Isolated paediatric neurosarcoidosis (IPN) is exceptionally rare and only seven cases have been reported so far in the literature. We report the clinical and radiological profile of a 7 year-old boy with epilepsia partialis continua (EPC) who was initially thought to have Acute Disseminated Encephalomyelitis (ADEM), but was subsequently found to have isolated neurosarcoidosis. Additionally, we performed a literature search on Medline and Embase and secondary sources of data such as reference list of articles reviewed.

Presence and pathogenic relevance of antibodies to clustered acetylcholine receptor in ocular and generalized myasthenia gravis.

Background: Clustered acetylcholine receptor antibodies (clustered AChR-Abs) have been detected in a proportion of patients with previously "seronegative" (SN) generalized myasthenia gravis (GMG), but their presence in patients with ocular MG (OMG) and their pathogenicity in vivo are unknown.

Objective: To test the presence of clustered AChR-Abs and their pathophysiologic properties in patients with SNMG.

Design: Screening and diagnostic tests.

Modern electroencephalography.

Electroencephalography (EEG) has been in continuous development over at least 70 years and is firmly established as a tool in the management of epilepsy. For a while, the technique fell into disregard because of difficulties with interpretation, specificity and sensitivity. Whilst clinicians have to be aware of these problems, they have been largely addressed by recent computer digitization of signals, which permits longer standard recordings and monitoring linked to a simultaneous video.

Correlating extent of neuromuscular instability with acetylcholine receptor antibodies.

In a retrospective study of 86 patients with myasthenia gravis (MG), we correlated the acetylcholine receptor (AChR) antibody titers with single-fiber EMG studies to explore whether a relationship exists between these parameters. We found that the AChR antibody titers correlated significantly with the mean of the mean consecutive difference of orbicularis oculi (OO, P<0.0001) and extensor digitorum communis (EDC, P<0.

Posterior interosseous nerve palsy in rheumatoid arthritis: case report and literature review.

Posterior interosseous nerve (PIN) palsy, presenting with a loss of digital extension, is a rare neurological complication of rheumatoid arthritis (RA). It may be caused by nerve entrapment, vasculitis or drug toxicity. There is no consensus regarding the treatment of PIN palsy in RA.

Clinical features of the DOK7 neuromuscular junction synaptopathy.

Mutations in DOK7 have recently been shown to underlie a recessive congenital myasthenic syndrome (CMS) associated with small simplified neuromuscular junctions ('synaptopathy') but normal acetylcholine receptor and acetylcholinesterase function. We identified DOK7 mutations in 27 patients from 24 kinships. Mutation 1124_1127dupTGCC was common, present in 20 out of 24 kinships.

Quantitative EMG of facial muscles in myasthenia patients with MuSK antibodies.

Objective: Our aim was to study the pathophysiological process leading to facial muscle atrophy in 13 patients with MuSK antibody positive myasthenia gravis (MuSK-MG), and to compare with findings from 12 acetylcholine receptor antibody positive myasthenia patients (AChR-MG), selected because they suffered from the same degree of disease severity and required similar treatment.

Methods: Motor unit action potential (MUAP) and interference pattern analysis from orbicularis oculi (O oculi) and orbicularis oris (O oris) muscles were studied using a concentric needle electrode, and compared with findings in 20 normal subjects, 6 patients receiving botulinum toxin injections (representing a neurogenic model) and 6 patients with a muscle dystrophy (representing a myopathic model). The techniques and control data have been reported previously.

The genetic structure of Australasian green turtles (Chelonia mydas): exploring the geographical scale of genetic exchange.

Ecological and genetic studies of marine turtles generally support the hypothesis of natal homing, but leave open the question of the geographical scale of genetic exchange and the capacity of turtles to shift breeding sites. Here we combine analyses of mitochondrial DNA (mtDNA) variation and recapture data to assess the geographical scale of individual breeding populations and the distribution of such populations through Australasia. We conducted multiscale assessments of mtDNA variation among 714 samples from 27 green turtle rookeries and of adult female dispersal among nesting sites in eastern Australia.

MRI and clinical studies of facial and bulbar muscle involvement in MuSK antibody-associated myasthenia gravis.

A proportion of patients with myasthenia gravis (MG) without acetylcholine receptor (AChR) antibodies have antibodies to muscle-specific kinase (MuSK). MG with MuSK antibodies (MuSK-MG) is often associated with persistent bulbar involvement, including marked facial weakness and tongue muscle wasting. The extent of muscle wasting in MuSK-MG, and whether it is also found in the few acetylcholine receptor (AChR-MG) patients who have persistent bulbar involvement, is not clear.

Single-fiber electromyography in limb and facial muscles in muscle-specific kinase antibody and acetylcholine receptor antibody myasthenia gravis.

We examined the findings from single-fiber electromyography in extensor digitorum communis (EDC) and orbicularis oculi (OOc) in 13 myasthenia gravis (MG) patients with muscle-specific kinase antibodies (MuSK-MG) and 12 MG patients with acetylcholine receptor antibodies (AChR-MG) with similar clinical scores. More than 70% of AChR-MG patients had abnormal jitter in both EDC and OOc, but the majority of MuSK-MG patients had normal jitter in EDC despite abnormal jitter in OOc. These findings demonstrate clear differences between the neurophysiology of MuSK-MG and AChR-MG.