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Background The understanding of fatal familial insomnia (FFI), a rare neurodegenerative autosomal dominant prion disease, has improved in recent years as more cases were reported. This work aimed to propose new diagnostic criteria for FFI with optimal sensitivity, specificity, and likelihood ratio. Methods An international group of experts was established and 128 genetically confirmed FFI cases and 281 non-FFI prion disease controls are enrolled in the validation process. The new criteria were proposed based on the following steps with two-round expert consultation: (1) Validation of the 2018 FFI criteria. (2) Diagnostic item selection according to statistical analysis and expert consensus. (3) Validation of the new criteria. Results The 2018 criteria for possible FFI had a sensitivity of 90.6%, a specificity of 83.3%, with a positive likelihood ratio (PLR) of 5.43, and a negative likelihood ratio (NLR) of 0.11; and the probable FFI criteria had a sensitivity of 83.6%, specificity of 92.9%, with a PLR of 11.77, and a NLR of 0.18. The new criteria included more specific and/or common clinical features, two exclusion items, and summarized a precise and flexible diagnostic hierarchy. The new criteria for possible FFI had therefore reached a better sensitivity and specificity (92.2% and 96.1%, respectively), a PLR of 23.64 and a NLR of 0.08, whereas the probable FFI criteria showed a sensitivity of 90.6%, a specificity of 98.2%, with a PLR of 50.33 and a NLR of 0.095. Conclusions We propose new clinical diagnostic criteria for FFI, for a better refining of the clinical hallmarks of the disease that ultimately would help an early recognition of FFI and a better differentiation from other prion diseases.

Objectives At the end of 2019 the SARS-CoV-2 outbreak spread around the globe with a late arrival to South America. The objective of this study was to evaluate the impact of the long period of mandatory social isolation that took place in Argentina on the general psychological well-being of healthcare workers due to the COVID-19 pandemic. Methods A survey was conducted during June 2020, in healthcare workers. Pittsburgh Sleep Quality Index, Insomnia Severity Index, Sleepiness-Wakefulness Inability and Fatigue Test, and Goldberg depression and anxiety scale, were used to analyze the effects of the SARS-Cov 2 outbreak after three months of mandatory social isolation. Analyses were performed by logistic regression and a clustering algorithm in order to classify subjects in the function of their outcome's severity. Results From 1059 surveys, the majority reported symptoms of depression (81.0%), anxiety (76.5%), poor sleep quality (84.7%), and insomnia (73.7%) with 68.9% suffering from nightmares. Logistic regression showed that being in contact with COVID-19 patients, age, gender and the consumption of sleep medication during the mandatory social isolation were relevant predictors for insomnia, anxiety, and depression. Clustering analysis classified healthcare workers in three groups with healthy/mild, moderate, and severe outcomes. The most vulnerable group was composed mainly of younger people, female, non-medical staff, or physicians in training. Conclusion An extremely high proportion of Argentinian healthcare workers suffered from sleep problems, anxiety, and depression symptoms. The clustering algorithm successfully separates vulnerable from non-vulnerable populations suggesting the need to carry out future studies involving resilience and vulnerability factors., Highlights • COVID-19 social isolation is related to insomnia, anxiety, and depression symptoms. • Nightmares were correlated with high levels of anxiety during COVID-19 outbreak. • Younger people, women, and non-medical health workers were the most affected population. • K-means algorithm successfully separates vulnerable from non-vulnerable populations.

Kleine-Levin syndrome is an uncommon disorder with recurrent episodes of hypersomnia, and behavioral abnormalities such as binge-eating and hypersexuality. Our aims were to report cases of the Kleine-Levin syndrome diagnosed in Buenos Aires, Argentina and to characterize the clinical presentation of these patients. We evaluated patients with Kleine-Levin syndrome according to the International Classification of Sleep Disorders. Psychiatric, physical and neurological symptoms were present. Some patients were investigated with brain Magnetic Resonance Imaging, Single Photon Emission Computed Tomography, electroencephalogram and some with polysomnography. Seven patients (2 female, 5 male), ages from 8 to 47 years (median 20.7 years) were included in the study. The duration of symptoms was 1.5-20 days with a mean of 8. The range of interval between episodes: 2.5-24 months, median=13. All seven patients had a history of hypersomnia (one of them post head injury); 5 reported hyperphagia and 2 reduced appetite. Brain MRI was performed in 6 patients: 1 showed non-specific abnormalities and another presented diencephalic hematoma; the rest were normal. Our paper is the first one in Buenos Aires reporting Kleine-Levin syndrome of different ethiologies. The prevalence is difficult to estimate in our country.