MRI in CLN2 disease patients: Subtle features that support an early diagnosis

Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a rare, paediatric-onset, neurodegenerative disorder characterised in its early stages by language delay, seizures and loss of motor function. It is rapidly progressive and ultimately results in the premature death of patients. We aim to highlight common magnetic resonance imaging (MRI) features seen in early CLN2 disease and increase disease awareness among clinicians in order to facilitate early diagnosis and treatment of patients with disease-modifying enzyme replacement therapy.We obtained MRI scans from 12 Turkish children with CLN2 disease, at symptom onset or time of diagnosis, and at various times during disease progression. Patient details including age at onset of symptoms, age at diagnosis and clinical presentation were collected. MRIs were analysed to identify common features present in patients with CLN2 disease.The median diagnostic delay in this cohort was 2 years, highlighting the need for increased disease awareness among clinicians. Key MRI features suggestive of CLN2 disease that were identified included cerebellar atrophy in 11 patients, linear hyperintensity of central white matter in 10 patients, cerebral atrophy in 8 patients and thinning of the corpus callosum in 6 patients. Thalamic hypointensity was seen in 1 patient and may also indicate CLN2 disease.It is important to consider the presenting symptoms alongside clinical test results in order to support early diagnosis of CLN2 disease. Clinical suspicion of CLN2 disease accompanied by the detection of any of the above-mentioned features on MRI should encourage healthcare professionals to test for CLN2 disease.BioMarin Pharmaceutical Inc

An Overview of Food Emulsions: Description, Classification and Recent Potential Applications

Emulsions take place partially or completely in the structures of many natural and processed foods or some foods are already emulsified in certain stages of production. In general “emulsion” is described as a structure created through the dispersion of one of two immiscible liquids within the other one in form of little droplets. Many terms are available to describe different emulsion types and it is very important to define and clarify these terms like “macro emulsion”, “nanoemulsion” and “multiple emulsion”. Nanoemulsions become increasingly important in food industry as an innovative approach in carrying functional agents. Application potential of multiple emulsions (W/O/W) is also stated to be very high in food industry. The two main strategic purposes of utilization of multiple emulsions in food applications are to encapsulate various aromas, bioactive compounds or sensitive food compounds and to allow the production of the low-fat products. This review provides an overview to the general terms of emulsion types, the role of various emulsifying agents, and the application potential of emulsions in food industry.Emulsions take place partially or completely in the structures of many natural and processed foods or some foods are already emulsified in certain stages of production. In general “emulsion” is described as a structure created through the dispersion of one of two immiscible liquids within the other one in form of little droplets. Many terms are available to describe different emulsion types and it is very important to define and clarify these terms like “macro emulsion”, “nanoemulsion” and “multiple emulsion”. Nanoemulsions become increasingly important in food industry as an innovative approach in carrying functional agents. Application potential of multiple emulsions (W/O/W) is also stated to be very high in food industry. The two main strategic purposes of utilization of multiple emulsions in food applications are to encapsulate various aromas, bioactive compounds or sensitive food compounds and to allow the production of the low-fat products. This review provides an overview to the general terms of emulsion types, the role of various emulsifying agents, and the application potential of emulsions in food industry

Surgery for intractable temporal lobe epilepsy: experience of a single institution

WOS: 000343068800025PubMed ID: 25539559Background/aim: In the treatment of epilepsy, encouraging results have been achieved with surgical treatment, especially for temporal lobe epilepsy (TLE). Drug resistance must be diagnosed as early as possible, because the ensuing seizures can be eliminated surgically via temporal lobe surgery in a high percentage (70%-90%) of patients. In this study we share our experience, in a single institution, of surgical treatment of intractable TLE. Materials and methods: Between March 2006 and November 2010 we performed 127 corticoamygdalohippocampectomy (CAH) procedures. All CAN surgical procedures were done as described by Niemeyer's technique. Resection lengths were 4-4.5 cm from the temporal pole. Results: At the end of 24 months, 79.7% (n = 94) patients were still on antiepileptic medications, with 55 of them on a decreased number or dose of drugs, and 20.3 (n = 24) patients were antiepileptic drug-free. Postoperative Engel's classes were 1, 2, and 3 in 87.2%, 5.08%, and 7.6%, respectively. There was no mortality in follow-up, and dysphasia in 1 patient (0.84%) was the only morbidity. Conclusion: In our series we found that the outcome of surgery is associated with careful patient selection, which requires a detailed investigation of the patients. Our final conclusion is that outcome scores are independent of age, pathology, or sex but are dependent on correct patient selection

Subacute sclerosing panencephalitis in a child with recurrent febrile seizures.

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis

Reply to "Impact of drug-resistant epilepsy on sleep in children: How they behave?"

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How do children with drug-resistant epilepsy sleep? A clinical and video-PSG study

Aim: The aim of this study was to assess sleep architecture and sleep problems among three homogenous groups of children including children with drug-resistant focal epilepsy, children with newly diagnosed, drug-naive focal epilepsy, and healthy children using overnight video-polysomnography (V-PSG) and a sleep questionnaire.Methods: We compared sleep architecture among 44 children with drug-resistant focal epilepsy, 41 children with newly diagnosed, drug naive focal epilepsy, and 36 healthy children. All children underwent an overnight V-PSG recording, and their parents completed the Children's Sleep Habits Questionnaire (CSHQ). Sleep recordings were scored according to the American Academy of Sleep Medicine criteria.Results: Compared with children with newly diagnosed epilepsy and healthy controls, children with drug-resistant epilepsy receiving antiepileptic treatment showed disturbed sleep architecture, a significant reduction in time in bed, total sleep time, sleep efficiency, NREM3%, REM%, and a significant increase in awakenings, wake after sleep onset, and periodic leg movement. Children with drug-naive, newly diagnosed focal epilepsy showed a statistically significant increase in sleep onset latency, rapid eye movement (REM) latency, N1%, awakenings, and a significant decrease in time in bed when compared with the controls. Children with drug-resistant epilepsy had the highest CSHQ total scores, while children with drug-naive, newly diagnosed focal epilepsy had higher scores than healthy children.Conclusion: This is one of the few polysomnographic studies adding to the limited research on the sleep macrostructure of children with drug-resistant epilepsy compared with children with drug-naive, newly diagnosed focal epilepsy and healthy children by obtaining objective measurements of sleep concurrently with a validated questionnaire. Children with drug-resistant epilepsy had a greater incidence of sleep disturbance on the basis of qualitative aspects and architecture of sleep than children with newly diagnosed epilepsy, suggesting the need for referral of children with drug-resistant epilepsy for overnight sleep evaluation in order to improve the clinical management and optimize therapeutic strategies

Synthesis, spectroscopic characterization, DFT calculations, and molecular docking studies of new unsymmetric bishydrazone derivatives

Three new unsymmetric isatin bishydrazone compounds; Comp. I, II, III, were synthesized by the condensation of 3,5-dichloro-salicylaldehyde, 3-bromo-5-chloro-salicylaldehyde, and 3,5-dibromo-salicylaldehyde with isatin monohydrazone, respectively. The synthesized compounds were characterized by elemental analysis, H-1-NMR, FT-IR, UV-Vis spectroscopy, and mass spectrometry technique. For studied molecules, chemical parameters like frontier orbital energies, energy gap, electronegativity, chemical potential, chemical hardness, softness, electrophilicity, nucleophilicity, electrodonating power, electroaccepting power, polarizability, and dipole moment were calculated and discussed. Investigating the validity of well-known electronic structure principles like Maximum Hardness, Minimum Polarizability, and Minimum Electrophilicity Principles in the study, it was determined which compound is more stable compared to others. In recent days, a new software having PRIMorDIA name was developed to explore reactivity and electronic structure in large biomolecules by some of the authors of this paper. Molecular docking studies for these newly synthesized molecules were performed using PRIMorDIA software. Considering the intramolecular interactions, NBO analyzes of three bishydrazone derivatives were conducted to evaluate the chemical behavior. (C) 2021 Elsevier B.V. All rights reserved