Late infantile epileptic encephalopathy: A distinct developmental and epileptic encephalopathy syndrome

Objective: Within the spectrum of developmental and epileptic encephalopathy (DEE), there are a group of infants with features that are distinct from the well-recognized syndromes of early infantile developmental and epileptic encephalopathy (EIDEE), infantile epileptic spasm syndrome (IESS), and Lennox-Gastaut syndrome (LGS). We refer to this condition as late infantile epileptic encephalopathy (LIEE). Our objective is to highlight the characteristics of this group by analyzing patients who exhibit prototypical features. Methods: From July 2022 to May 2023, we searched for LIEE features in pediatric patients who underwent epilepsy follow-up at the University of Chicago Comer Children's Hospital. Results: Out of 850 patients evaluated, thirty patients (3.5%) were identified with LIEE based on electroclinical characteristics. These patients had an average onset of epilepsy at 6.8 months and an average onset of LIEE features at 18.1 months. The epilepsy etiology was most commonly genetic and metabolic (50%), followed by congenital cortical malformations (23%), acquired structural abnormalities (20%), and unknown (7%). The predominant seizure types were myoclonic-tonic (70%), spasm-tonic (50%), epileptic spasms (47%), tonic (43%), and myoclonic (43%) seizures. All patients reported a history of either spasm-tonic or myoclonic-tonic seizures in addition to other types. All patients had EEGs showing discontinuity, electro-decrements, or both along with diffuse slowing, background voltages between 100-300 μV, and superimposed multifocal, diffuse epileptiform discharges. Every patient, except one, fulfilled the definition of drug-resistant epilepsy and all reported either moderate to severe developmental delay. Significance: Late infantile epileptic encephalopathy (LIEE) is characterized by several unique clinical and electrographic features. Typically, LIEE manifests in patients during the second year of life and occurs before two years of age, hence late-infantile onset. The condition is commonly observed in infants with symptomatic epilepsy. Myoclonic-tonic and spasm-tonic seizures are the quintessential seizure types. The inter-ictal EEG exhibits more organization and lower voltages than seen with hypsarhythmia and lacks the defining EEG characteristics of EIDEE, IESS, or LGS. We propose that LIEE is a distinct electroclinical syndrome within the spectrum of developmental and epileptic encephalopathies

A web-based surveillance model of eosinophilic meningitis: future prediction and distribution patterns

Background: web-based surveillance is a useful tool for predicting future cases of various emerging infectious diseases. There are limited data available on web-based surveillance and patterns of distribution of eosinophilic meningitis (EOM), which is an emerging infectious disease in various countries around the world.  Methods: this study applied web-based surveillance to the prediction of EOM incidence and the analysis of its distribution pattern by using a national database, which may be used for future prevention and control. The number cases of EOM in each month over a period of 12 years (between 2006 to 2017) from Loei province were retrieved from the National Disease Surveillance (Report 506) website, operated by Thailand's Public Health Center.  Results: we developed autoregressive integrated moving average (ARIMA) models and seasonal ARIMA (SARIMA) models. The best model was used for predicting numbers of future cases. The forecast values from the SARIMA (1, 1, 2)(0,1,1)6 model were close to actual values and were the most valid, as they had the lowest RMSE and AIC. The predictive model for future cases of EOM was related to previous numbers of EOM cases over the past eight months. The disease exhibited a seasonal pattern during the study period.  Conclusions: web-based surveillance can be used for future prediction of EOM, that the predictive model applied here was valid, and that EOM exhibits a seasonal pattern

Additional risk factors associated with symptomatic hydrochlorothiazide-induced hyponatremia in hypertensive patients

Background. Hydrochlorothiazide is a cheap and effective antihypertensive agent but may cause hyponatremia. Even though several risk factors for hydrochlorothiazide-induced hyponatremia have been reported, this study aimed to evaluate additional risk factors for hydrochlorothiazide-induced hyponatremia in hypertensive patients. Material and methods. The inclusion criteria were: adult patients, diagnosed with hypertension and receiving hydrochlorothiazide treatment. Eligible patients were divided into two groups: with and without hyponatremia. Those with hyponatremia were identified by using the ICD-10 code E871, while those without hyponatremia were patients who did not have any reported hyponatremia until the last visit. The ratio between hyponatremia and non-hyponatremia group was 1:2. Predictors for hyponatremia were analyzed by using logistic regression analysis. Results. During the study period, there were 68 patients admitted due to symptomatic hyponatremia from hydrochlorothiazide. There were four independent factors in the model predictive of occurrence of symptomatic hydrochlorothiazide-induced hyponatremia in hypertensive patients: sex, body mass index, plasma glucose, and serum albumin. Male sex, body mass index, and serum albumin were negatively associated with occurrence of symptomatic hydrochlorothiazide-induced hyponatremia in hypertensive patients with adjusted OR of 0.099, 0.683, and 0.122, respectively. The plasma glucose had adjusted OR of 1.030 [95% CI of (1.009, 1.051)]. Conclusions. Factors associated with hydrochlorothiazide-induced symptomatic hyponatremia in hypertensive patients were sex, body mass index, plasma glucose level, and serum albumin level. The latter two risk factors have never been reported as risk factors for hydrochlorothiazide-induced symptomatic hyponatremia in hypertensive patients

Electroclinical features of myoclonic-tonic and spasm-tonic seizures in childhood

Myoclonic-tonic (MT) and spasm-tonic (ST) seizures represent distinctive features in late infantile epileptic encephalopathy (LIEE). This commentary aims to delineate the electroclinical characteristics of MT and ST seizures, setting them apart from other seizure types. Our analysis encompasses 211 ST and MT seizures observed in 31 patients diagnosed with LIEE, providing a comprehensive overview of video-EEG features and polygraphic signatures. In MT seizures, EEG findings reveal a high-voltage diffuse spike/polyspike and wave discharge, often succeeded by diffuse electrodecrements. The amplitude-integrated EEG (aEEG) signature is described as a “reversed checkmark.” Conversely, ST seizures exhibit EEG findings such as a vertex positive deflection after a slow-wave and relative electrodecrement, with intermixed epileptiform discharges. In comparison to MT seizures, polygraphic characteristics in ST seizures appear more distinct, featuring a brief rhomboid shape corresponding to the spasm, followed by a lengthier rectangular shape indicative of the tonic phase of the ST seizure. While the pathophysiology of ST and MT seizures remains inadequately understood, their concurrent occurrence and association with other seizure types (tonic, epileptic spasm, myoclonic) within the temporal context of LIEE and other epileptic encephalopathies prompt us to anticipate advancements in our understanding through future research. We hope that this study serves as a foundation for unraveling these complexities in the times to come

Residency Training: A Practical Guide for Medical Students who are Planning a Future in Child Neurology

At the time of graduation from medical school, medical students have been exposed primarily to adult neurology and have limited exposure to child neurology. Child neurology is a unique field that encompasses caring for children with neurological conditions ranging from routine to rare. There are many opportunities for a variety of unique careers in child neurology including both in the inpatient and outpatient setting. This article aims to provide practical advice for the medical student interested in child neurology to best prepare for a successful match and rewarding career