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Clinical Neuroscience

MARCH 30, 2021


[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

Clinical Neuroscience

NOVEMBER 20, 2015

[Attachment as a predictor of risk for eating disorders on a representative hungarian adult sample]

SZALAI Dömötör Tamás, CZEGLÉDI Edit

[Background and purpose – Many studies confirm the relationship between attachment disturbances and (the severity of) eating disorders, however among them only one Hungarian study can be found. The exact predisposing traits of attachment and the strength of relationship is still uncleared. Our aim was to explore these aspects. Methods – Study was based on a cross-sectional nationally representative survey, called „Hungarostudy 2013” (N=2000, 46.9% males, mean age 46.9 years, SD=18.24 years). Measures: Sociodemographic and self-reported anthropometric data (weight and height), short Hungarian version of Relationship Scale Questionnaire, SCOFF questionnaire and short Hungarian version of Beck Depression Inventory. Results – The frequency of risk for eating disorders (anorexia or bulimia nervosa) was 3.9% (N=76) among the respondents (N=1860). Attachment anxiety was significantly higher in the risk for eating disorders group (t(1888)=-3.939, p<0.001), and significantly predicted the risk for eating disorders after adjusting for the potential background variables (OR=1.09, p=0.040). Detachment was not a significant predictor of risk for eating disorders (OR=0.98, p=0.515). Younger age (OR=0.97, p<0.001), higher level of depression (OR=1.09, p<0.001) and higher body mass index (OR=1.08, p<0.001) were also significant cross-sectional predictors of risk for eating disorders. The explained variance of the model was 10.7%. Conclusion – The study supported, that higher attachment anxiety is associated with the increased risk of eating disorders, with a possible therapeutic relevance. Assessment of attachment’s further aspects and creating multivariable models are required for more thorough understanding and optimising of intervention points.]

Clinical Neuroscience

MARCH 30, 2016

[Hungarian adaptation of a short eating disorder questionnaire (SCOFF)]

DUKAY-SZABÓ Szilvia, SIMON Dávid, VARGA Márta, SZABÓ Pál, TÚRY Ferenc, RATHNER Günther

[Aim - Eating disorders are becoming an increasingly relevant health issue, therefore the fast and accurate screening of people at risk is of great practical importance. The aim of SCOFF questionnaire is to assess this risk and the extent to which a person is affected, by using five simple yes or no questions. The objective of our study was to assess the validity of the Hungarian version of the test Methods - 777 medical students (210 men, 567 women, mean age 22.3±2.33 years) participated in the survey. The online questionnaire contained anthropometric data, the Eating Behaviour Severity Scale and, beside the SCOFF, the Eating Disorder Inventory (EDI). Results - The SCOFF is excellent at screening clinical eating disorders. Its sensitivity was 100% and specificity 85.1%. It is less efficient at detecting subclinical cases, but it does not show worse results than EDI. According to the data SCOFF is better at identifying more serious cases. Applying on the same sample SCOFF had higher sensitivity and lower specificity than EDI. Discussion - SCOFF is suitable for primary screening of eating disorders with a non-diagnostic purpose, taken two “yes” answers out of five as the critical margin, specified by the authors.]

Lege Artis Medicinae

APRIL 18, 2020

[The relationship of traumatic experiences and eating disorders – therapeutical options]


[In the etiology of eating disorders (espe­cially bulimia nervosa and binge eating) traumatic experiences (sexual, physical, emo­tional abuse, neglect) play an important role. Traumatization can have a serious impact, which is influenced by the parameters of the traumatization, risk and protective factors, and the resiliency of the traumatized patient. The consequences may lead to the development of specific psychiatric and somatic disorders, and may cause lifetime revictimization. The exploration of data related to the traumatization is essential in eating disorders as well. If traumatic expericences can be detected in the back­ground of eating disorders, the targeted therapy of eating disorders while applying its specific elements should also follow the guide­lines of the general trauma-therapy. Providing safety in therapeutical relation­ship is fundamental. The therapeutic options are extensive. Along with psychodynamically oriented therapies, the newer methods based on cognitive-behavioral therapy (e.g., dialectic behavior therapy, integrative cognitive analytic therapy) are also proposed. Hyp­no­therapy can also be useful. ]

Lege Artis Medicinae

JULY 20, 2019

[Gynecological correlations of eating disorders]


[In recent decades, the incidence of eating disorders (its two major forms are anorexia nervosa and bulimia nervosa) has increased, their course has been delayed, and their complications are becoming more frequent and severe. Eating disorders cannot only damage physical and mental health but also affect reproductive health, future mothers and their children. Understanding the biological and psychosocial factors that explain the gynecological conditions related to eating disorders is essential regarding fertility and pregnancy. The present review summarizes data published from 2000 about the gynecological effects of eating disorders. In daily practice, the multidisciplinary collaboration is particularly important to pro­vide women with eating disorders with comprehensive care. The appropriate information of women who plan to have children is essential in order to increase the likelihood of conception and reduce the risk of miscarriage. ]

Clinical Neuroscience

SEPTEMBER 30, 2020

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEM Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Clinical Neuroscience

JANUARY 30, 2021

Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

Journal of Nursing Theory and Practice

DECEMBER 30, 2019

[Health awareness among nurses]


[The purpose of this study is to demonstrate the dietary habits and health behaviors of nurses studying in SE-ETK. The study describes in a descriptive/informative manner the main characteristics of the target group mentioned above. In our research, and in our conclusions drawn and deduced from it, we sought answers to questions that have been addressed in the literature from other approaches so far. Our own research focused on nurses’ dietary habits and their health awareness. To explore whether occupational harm can only be considered, or whether the links here are much deeper and more complex. Based on the data, we can say that their theoretical knowledge of healthy eating is more complete, but in practice, their health awareness/behavior is not. Their answers to our eating and sports questions have an inverse relationship with their BMI data. This is probably because their workplace conditions (shifts) do not allow their health awareness to develop. ]

Lege Artis Medicinae

SEPTEMBER 20, 2018

[Feeding and eating in infancy and early childhood part III. - Development of self-feeding skills in the large-sample of the “For Healthy Offspring” project ]

NÉMETH Tünde, VÁRADY Erzsébet, DANIS Ildikó, SCHEURING Noémi, SZABÓ László

[Feeding and eating in infancy and early childhood part III. - Development of self-feeding skills in the large-sample of the “For Healthy Offspring” project INTRODUCTION - After introducing adequate complementary food to the diet of breastfed/formula-fed babies, the frequency and amount of semisolid/solid food is increasing, the breastmilk/formula intake is decreasing and finally the weaning process is completed. During this process the developing feeding skills of the infant enables them to self-feed. The self-feeding infant and toddler should participate in family meals. SUBJECTS AND METHODS - In the Healthy Offspring project self reported questionnaires were received from 1133 parents of 0-3 year old children. Issues concerning the development of self-feeding skills were analyzed. RESULTS - With advancing age the proportion of infants/toddlers, reported to be able to (partially) self-feed, has increased. The age, at which the majority of toddlers (83.1%) were reported to self-feed, was at 13-15 months. By the age over 2 years 57.2% of the toddlers were fully self-feeding, 39.3% were self-feeding with some assistance, and 3.5% were still completely fed by their mother/caregiver. While self-feeding became more prevalent, the proportion of toddlers with feeding problems and insufficient weight gain has increased. With more prevalent complementary feeding more parents assessed their feeding style rather scheduled than on demand. In the whole sample the proportion of infants/toddlers, who ate with the family, was 43.8%. CONCLUSIONS - In our sample, as previously described in the scientific literature, the developmental readiness to self-feed has developed in the majority of infants by the age of 13-15 months. During progres­sion of weaning an increasing proportion of parents thought, that feeding was rather scheduled than on demand. This finding points at the importance of educating parents about the importance of responsive feeding during and after weaning. For self-feeding toddlers, responsive feeding means, that the mother/caregiver offers a choice of healthy and adequate amount of food, at a proper place, at proper times, responds to the hunger and satiety cues of the child and the toddler decides, whether to eat, what and how much to eat.]

Clinical Neuroscience

JULY 30, 2020

[Advanced Parkinson’s disease characteristics in clinical practice: Results from the OBSERVE-PD study and sub-analysis of the Hungarian data]

TAKÁTS Annamária, ASCHERMANN Zsuzsanna, VÉCSEI László, KLIVÉNYI Péter, DÉZSI Lívia, ZÁDORI Dénes, VALIKOVICS Attila, VARANNAI Lajos, ONUK Koray, KINCZEL Beatrix, KOVÁCS Norbert

[The majority of patients with advanced Parkinson’s disease are treated at specialized movement disorder centers. Currently, there is no clear consensus on how to define the stages of Parkinson’s disease; the proportion of Parkinson’s patients with advanced Parkinson’s disease, the referral process, and the clinical features used to characterize advanced Parkinson’s disease are not well delineated. The primary objective of this observational study was to evaluate the proportion of Parkinson’s patients identified as advanced patients according to physician’s judgment in all participating movement disorder centers across the study. Here we evaluate the Hungarian subset of the participating patients. The study was conducted in a cross-sectional, non-interventional, multi-country, multi-center format in 18 countries. Data were collected during a single patient visit. Current Parkinson’s disease status was assessed with Unified Parkinson’s Disease Rating Scale (UPDRS) parts II, III, IV, and V (modified Hoehn and Yahr staging). Non-motor symptoms were assessed using the PD Non-motor Symptoms Scale (NMSS); quality of life was assessed with the PD 8-item Quality-of-Life Questionnaire (PDQ-8). Parkinson’s disease was classified as advanced versus non-advanced based on physician assessment and on questions developed by the Delphi method. Overall, 2627 patients with Parkinson’s disease from 126 sites were documented. In Hungary, 100 patients with Parkinson’s disease were documented in four movement disorder centers, and, according to the physician assessment, 50% of these patients had advanced Parkinson’s disease. Their mean scores showed significantly higher impairment in those with, versus without advanced Parkinson’s disease: UPDRS II (14.1 vs. 9.2), UPDRS IV Q32 (1.1 vs. 0.0) and Q39 (1.1 vs. 0.5), UPDRS V (2.8 vs. 2.0) and PDQ-8 (29.1 vs. 18.9). Physicians in Hungarian movement disorder centers assessed that half of the Parkinson’s patients had advanced disease, with worse motor and non-motor symptom severity and worse QoL than those without advanced Parkinson’s disease. Despite being classified as eligible for invasive/device-aided treatment, that treatment had not been initiated in 25% of these patients.]