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Lafora disease is a rare genetic disorder characterized by a disruption in glycogen metabolism. It manifests as progressive myoclonus epilepsy and cognitive decline during adolescence. Pathognomonic is the presence of abnormal glycogen aggregates that, over time, produce large inclusions (Lafora bodies) in various tissues. This study aims to describe the clinical and histopathological aspects of a novel Lafora disease patient, and to provide an update on the therapeutical advancements for this disorder. A 20-year-old Libyan boy presented with generalized tonic-clonic seizures, sporadic muscular jerks, eyelid spasms, and mental impairment. Electroencephalography showed multiple discharges across both brain hemispheres. Brain magnetic resonance imaging was unremarkable. Muscle biopsy showed increased lipid content and a very mild increase of intermyofibrillar glycogen, without the polyglucosan accumulation typically observed in Lafora bodies. Despite undergoing three lines of antiepileptic treatment, the patient's condition showed minimal to no improvement. We identified the homozygous variant c.137G>A, p.(Cys46Tyr), in the EPM2B/NHLRC1 gene, confirming the diagnosis of Lafora disease. To our knowledge, the presence of lipid aggregates without Lafora bodies is atypical. Lafora disease should be considered during the differential diagnosis of progressive, myoclonic, and refractory epilepsies in both children and young adults, especially when accompanied by cognitive decline. Although there are no effective therapies yet, the development of promising new strategies prompts the need for an early and accurate diagnosis.

Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient's neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

The health care industry is complex, dynamic, and large. In such uncertain environments where a great deal of revenue is at stake, competition and comparative claims flourish. One such manifestation is hospital ratings systems. This research examines two influential hospital ratings to explore whether the hospital ratings of each system was straightforward and reproducible. Regressions and structural equations models were fit to examine the relationships among the hospital ratings constructs. Both hospital ratings systems were excellent in their transparency and reproducibility. The Consumer Reports and Leapfrog ratings systems can confidently tout that their hospital scores reflect what they claim to measure. The unique aspects of each system are also noted.

Objective: Sleep plays a role in some oligodendrocyte processes, including myelination. This study aimed to analyze the possible correlations between sleep quality and Multiple Sclerosis (MS) course., Methods: Forty patients with Relapsing-Remitting MS were admitted. Based on the score obtained by the Pittsburgh Sleep Quality Index (PSQI), they were divided into good sleepers (<5) and bad sleepers (≥5). A set of data was collected retrospectively for each patient to investigate whether PSQI scores correlated with EDSS score changes, the number and the duration of each relapse and the cumulative day-number of MS reactivations over a three-year period., Results: In a multivariate model, a PSQI score ≥5 independently and significantly correlated with an increase in number and duration of relapses (p = 0.000) and number of days of MS activity (p = 0.000) during the three-year retrospective observation period., Conclusions: The results of this study show that the course of MS may be influenced by sleep quality. Assessment of sleep quality could be used to obtain reliable prognostic information in patients with relapsing-remitting MS. Further investigations are necessary to evaluate whether the correction of sleep disorders may be effective in improving the prognosis of MS patients., (Copyright © 2019 Elsevier B.V. All rights reserved.)

In this article, we attempt to clarify our statements regarding the effects of mean centering. In a multiple regression with predictors A, B, and A × B (where A × B serves as an interaction term), mean centering A and B prior to computing the product term can clarify the regression coefficients (which is good) and the overall model fit R 2 will remain undisturbed (which is also good).

There seems to be confusion among researchers regarding whether it is good practice to center variables at their means prior to calculating a product term to estimate an interaction in a multiple regression model. Many researchers use mean centered variables because they believe it's the thing to do or because reviewers ask them to, without quite understanding why. Adding to the confusion is the fact that there is also a perspective in the literature that mean centering does not reduce multicollinearity. In this article, we clarify the issues and reconcile the discrepancy. We distinguish between "micro" and "macro" definitions of multicollinearity and show how both sides of such a debate can be correct. To do so, we use proofs, an illustrative dataset, and a Monte Carlo simulation to show the precise effects of mean centering on both individual correlation coefficients as well as overall model indices. We hope to contribute to the literature by clarifying the issues, reconciling the two perspectives, and quelling the current confusion regarding whether and how mean centering can be a useful practice.

Is investing in new technology always the right choice for a company and its customers? Allan Moulter, the CEO of Quality Care, isn't sure he wants to invest in the computerized reception system that consultant Jack Zadow has outlined for him. But in this HBR case study, the argument Zadow makes is impossible to ignore. Quality Care's rivals have invested in similar systems or are planning to do so. The new system promises to take care of routine busywork, freeing staff up for other interactions with patients. It seems as if the competition hasn't even cut staff and is counting on increased customer retention to pay for the investment. And yet, Quality Care's surveys of its own customers show that they prefer the human touch when checking in. How would customers feel if the first ¿person¿ they met when they came in the door turned out to be a machine? Moulter prides himself on his responsiveness to customers. And with 86% of Quality Care's customers either satisfied or completely satisfied, aren't things fine as they are? Has Moulter considered all the facets of his predicament? How will Quality Care's staff be affected by a decision one way or another? What about the costs of upgrading the system? Can Quality Care maintain its standing without going high-tech? Would customers rebel when confronted with the proposed reception area or would they appreciate the increased efficiency? Six experts weigh the costs and benefits of technology in a service industry.

The roles of affective and cognitive processes in judgment have been the focus of much recent research and theoretical debate. This study was designed to investigate the structure of voters' affective reactions and trait attributions to national political figures. Three-mode factor analysis was used to determine the structure of the affect and trait scales. Politician factors and subject types were also derived, as were the interrelationships among these modes. Positive and negative affect factors, and affect and trait factors, were distinct but correlated; Democratic and Republican politician factors were uncorrelated. Information on the subject types moderated these relationships.