Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication.

BACKGROUND AND PURPOSE: Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes. METHODS: This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.1-23.0 years. The following clinical characteristics were evaluated: gender, patient age at seizure onset, history of febrile seizures and migraine, family history of epilepsy, duration and seizure manifestations, circadian distribution and frequency of seizures, history of medications including the number of drugs, therapeutic response and final outcome. RESULTS: Visual hallucinations were the first symptom in 62% and the only manifestation in 38.8% of patients. Patients were subdivided into two groups: group A with isolated COE-G; group B with other epileptic syndromes associated with COE-G. The most significant (P < 0.05) difference concerned antiepileptic therapy: in group A, 45 children responded to monotherapy; in group B only 15 children responded to monotherapy. At the end of follow-up, the percentage of seizure-free patients was significantly higher in group A than in group B. CONCLUSIONS: Childhood occipital epilepsy of Gastaut has an overall favourable prognosis and a good response to antiepileptic therapy with resolution of seizures and of electroencephalogram abnormalities. The association of typical COE-G symptoms with other types of seizure could be related to a poor epilepsy outcome.

Genital ulcer as a new clinical clue to PFAPA syndrome.

Vaginal ulcers can be associated with a number of different diseases. We describe two girls who presented genital ulcers as a persistent symptom of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. The possibility of considering this clinical manifestation as a clue for the diagnosis of PFAPA is discussed.

COVID-19 and abdominal pain: a pediatric case report and a point of view in pediatric emergency medicine.

COVID-19 is to date a global pandemic that can affect all age groups; gastrointestinal symptoms are quite common in patients with COVID-19 and a new clinical entity defined as Multisystem Inflammatory Syndrome in Children (MIS-C) has been described in children and adolescents previously affected by COVID-19. Presenting symptoms of this new disease include high fever and severe abdominal pain that can mimic more common causes of abdominal pain; patients can rapidly deteriorate presenting severe cardiac dysfunction and multiorgan failure. Some fatalities due to this serious illness have been reported. We describe the case of a ten-year-old patient presenting with persistent high fever associated with continuous and worsening abdominal pain. Various hypotheses were performed during his diagnostic workup and an initial appendectomy was performed in the suspect of acute appendicitis. As his clinical picture deteriorated, the child was subsequently diagnosed and successfully treated as a case of MIS-C. The objective of this case report and brief review of abdominal pain in children throughout the age groups is to provide the emergency pediatrician with updated suggestions in diagnosing abdominal pain in children during the COVID-19 pandemic.

Neuropsychological impairment in childhood absence epilepsy: Review of the literature.

AIM: Childhood absence epilepsy (CAE) is a paediatric epilepsy syndrome characterized by typical absence seizures in school age children. Although it is commonly considered to have a good prognosis, with a good response to antiepileptic drugs, recent studies questioned this traditional view of a "benign" disorder, in particular regarding neuropsychological functioning. The aim of this study is to review the neuropsychological involvement in patients affected by CAE. METHODS: A literature search was carried out in PubMed's and Medline's databases for all relevant studies published between 1924 and 2014. The keywords used were neuropsychology, absence seizures, and CAE. Specific review articles, systematic reviews, textbooks and case reports were examined for any further publications. RESULTS: In intellectual functioning, CAE patients seem to perform worse than healthy children, even if their IQ scores fall within the normal range. Similarly, CAE seems to affect verbal skills and learning. Executive functions have been reported to be mildly impaired. Data regarding memory are still conflicting. DISCUSSION: Given the neuropsychological deficits in many CAE patients which significantly affect their quality of life, CAE should not be considered entirely "benign". An early identification of neuropsychological dysfunction in CAE children is essential for appropriate treatment.

Melatonin reduces excitotoxic blood-brain barrier breakdown in neonatal rats.

The blood-brain barrier (BBB) is a complex structure that protects the central nervous system from peripheral insults. Understanding the molecular basis of BBB function and dysfunction holds significant potential for future strategies to prevent and treat neurological damage. The aim of our study was (1) to investigate BBB alterations following excitotoxicity and (2) to test the protective properties of melatonin. Ibotenate, a glutamate analog, was injected intracerebrally in postnatal day 5 (P5) rat pups to mimic excitotoxic injury. Animals were than randomly divided into two groups, one receiving intraperitoneal (i.p.) melatonin injections (5mg/kg), and the other phosphate buffer saline (PBS) injections. Pups were sacrificed 2, 4 and 18 h after ibotenate injection. We determined lesion size at 5 days by histology, the location and organization of tight junction (TJ) proteins by immunohistochemical studies, and BBB leakage by dextran extravasation. Expression levels of BBB genes (TJs, efflux transporters and detoxification enzymes) were determined in the cortex and choroid plexus by quantitative PCR. Dextran extravasation was seen 2h after the insult, suggesting a rapid BBB breakdown that was resolved by 4h. Extravasation was significantly reduced in melatonin-treated pups. Gene expression and immunohistochemical assays showed dynamic BBB modifications during the first 4h, partially prevented by melatonin. Lesion-size measurements confirmed white matter neuroprotection by melatonin. Our study is the first to evaluate BBB structure and function at a very early time point following excitotoxicity in neonates. Melatonin neuroprotects by preventing TJ modifications and BBB disruption at this early phase, before its previously demonstrated anti-inflammatory, antioxidant and axonal regrowth-promoting effects.

Utilization of highly deoxynivalenol-contaminated wheat via extrusion processing.

Deoxynivalenol (DON) is a toxic natural metabolite produced by Fusarium graminearum. In this study we investigated the effects of sodium bisulfite and extrusion cooking under high temperature and pressure on DON levels in wheat grain and mill fractions. Samples of highly naturally DON-contaminated soft winter wheat were soaked for 1 h in water or aqueous sodium bisulfite (SB) solutions (0.5, 1.5, 2.5, or 5% SO2 equivalent) and extruded. The soaking treatment with SB solution (5% SO2 equivalent) lowered DON from 7.3 microg/g to 0.8 microg/g without extrusion and to 0.3 microg/g with an extrusion process. When the contaminated kernels were tempered with water or SB solutions (5 or 10% SO2 equivalent) and milled, the flour samples, as expected, showed lower levels of DON (from 7.3 to 3.1 microg/g). Extrusion of milled flour and whole meal samples, both obtained from SB-tempered wheat, did not change DON levels significantly under the studied extrusion conditions as compared to the nonextruded milled flour and whole meal samples. However, the extrusion process can be utilized to remove moisture and/or odor of chemicals used in the soaking solutions and to produce potentially useful extruded products.