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Clinical Neuroscience

JULY 30, 2018

[Online supplementay figure to the article titled Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences]


[Online supplementay figure to the article titled Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences]

Clinical Neuroscience

SEPTEMBER 30, 2018

[The evaluation of paroxysmal events in neonates and infants]

NAGY Eszter, FARKAS Nelli, HOLLÓDY Katalin

[Introduction - Differential diagnosis of neonatal and infantile seizures based only on inspection poses a challenge even for specialists. Aims - To investigate the evaluations of neonatal and infantile paroxysmal events based only on inspection. Research question - Is there any difference in the opinion of neonatologists, paediatric neurologists and neurologists about the assessment of common paroxysmal events in infancy? Patients and methods - Video recordings about paroxysmal movements of 15 neonates or infants (aged 2 days- 5 months) were displayed for 47 paediatric neurologists, 35 neonatologists and nurses working in Neonatal or Perinatal Intensive Care Units and 43 neurologists. They had to decide without knowing the past medical history or EEG results whether events presented were epileptic or nonepileptic in nature. Results - Answers of neonatologists and paediatric neurologists were correct in 67% of cases (824/1230), no significant difference was found between their results. The largest uncertainty was in the judgement of discrete hand movements and very rapid clonus with epileptic origin, they were judged correctly by only one third of participants. The result of neurologists was only slightly, but not significantly different from that of paediatric neurologists. Conclusion - In most cases, the correct diagnosis of neonatal and infantile paroxysmal events requires video-EEG recording. No significant difference was revealed between the evaluation of neonatologists and paediatric neurologists about the differential diagnosis of movements. The ongoing cooperation of paediatric neurologists and neurologists going back to several decades facilitates the shaping of a common perspective.]

Clinical Neuroscience

JULY 30, 2018

Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences


Purpose - To develop an evidence-based, standardized structured reporting (SR) method for brain MRI examinations in neonatal hypoxic-ischemic encephalopathy (HIE) suitable both for clinical and research use. Materials and methods - SR template development was based on comprehensive review of the pertinent literature with the basic sections and subdivisions of the template defined according to MRI sequences (both conventional and diffusion-weighted, MR-spectroscopy (MRS), and T2*-weighted imaging), and the items targeted on age-related imaging patterns of HIE. In order to evaluate the usability of the proposed SR template we compared data obtained from the brain MR image analysis of 87 term and 19 preterm neonates with the literature. The enrolled 106 infants were born between 2013 and 2015, went through therapeutic hypothermia according to the TOBY criteria due to moderate to severe asphyxia and had at least one brain MRI examination within the first two weeks of life. Ethical approval was obtained for this retrospective study. Descriptive statistical analysis was also performed on data exported from the structured reporting system as feasibility test. Results - The mean gestational age of the study population was 38.3±2.2 weeks; brain MRI was performed on 5.8±2.9 day of life, hence in 78% of our patients after the conclusion of therapeutic hypothermia. Our main imaging findings were concordant to the pertinent literature. Moreover, we identified a characteristic temporal evolution of diffusion changes. Interestingly 18% (n=19/106) of the clinically asphyxiated infants had isolated axial-extraaxial haemorrhage without any imaging sign of HIE. Conclusion - In this article our approach of reporting HIE cases with our novel SR template is described. The SR template was found suitable for reporting HIE cases, moreover it uncovered time and location dependent evolution of diffusion abnormalities (and pseudonormalization, as well), suggesting its usefulness in clinical research applications. The high number of isolated intracranial haemorrhages, and the changing diffusion pattern emphasizes the importance of early imaging in HIE.

Clinical Neuroscience

MARCH 30, 2018

[Perinatal stroke - from symptoms to follow-up]

VOJCEK Eszter, CSÉCSEI Márta, FLACH Edina, RUDAS Gábor, GRÁF Rózsa, PRINCZKEL Erzsébet

[Background and purpose - We aimed to analyze patient characteristics of term neonates with the diagnosis of stroke between 2006 and 2017 at the 3rd level Neonatal Intensive Care Unit of the Szent János Hospital. Method - We conducted a retrospective and prospective analysis including 18 newborns with stroke. Presentation, imaging methods, etiology and clinical context were discussed. All patients had a follow-up at 2 years of age or later. Subject of the study - In the past 10 years 17 term born and one premature neonate born at 36 weeks of age were diagnosed with stroke in our unit. All patients were born at good condition generally with high Apgar scores (9±1). Cesarean section was performed in 4 cases. Results - With an estimated incidence of one in 1600-4000 births, the incidence of perinatal stroke in our unit was found to be the same as mentioned in the international databeses. Regarding imaging method, cranial ultrasound scan do not visualise arterial ischaemic stroke therefore head MRI is recommended. Neurological symptoms of the patients presented in the first two days of life. Etiology included thrombophilia (4/18), infection (4/18), vascular malformation (2/18), moderate asphyxia (2/18) and pre-eclampsia (2/18). Middle cerebral artery was involved in 50% while the anterior cerebral artery was affected in 33%. The stroke occured in the left hemisphaerium in 44%, in the right side in 39% and was bilateral in 17%. In two cases the stroke was diagnosed in utero. Early childhood developmental support resulted in average or above average gross and fine motor development and cognitive outcome. Conclusion - Presenting neurological symptoms tipically occur in the first few days after birth when perinatal stroke need to be considered among the broad spectrum of neonatal illnesses. Normal developmental outcome can be achieved even in cases of extensive brain damage with early childhood developmental support. Severely impaired development was observed in the cases of in utero stroke. Inherited prothrombotic disorders may have implications for subsequent pregnancies of the mother. ]

Clinical Neuroscience

JULY 30, 2014

[Maybe it hurts more than you think! - Neonatal pain]

MIKOS Borbála

[Neonatal pain is often undertreated. This is based on the assumption that because of the immature nervous system and the lack of the myelinisation preterm and newborn does not feel pain. It is confirmed by a number of articles that the fetus and neonate can experience and respond to painful events. This publication gives a brief overview of the ontogeny of the pain, short-and long-term postnatal consequences, as well as the perception of the possibility of a particularly frail child population: premature infants and neonates, based on animal and human studies.]

Clinical Neuroscience

MAY 30, 2013

[Congenital myasthenic syndromes and transient myasthenia gravis]

GAJDA Anna, SZABÓ Hajnalka, GERGEV Gyurgyinka, KARCAGI Veronika, SZABÓ Nóra, ENDREFFY Emőke, TÚRI Sándor, SZTRIHA László

[Hypotonia in the neonatal period and early infancy is a common clinical finding. It can be caused by various heterogeneous disorders of different origin which might lead to diagnostic difficulties. Disorders of the neuromuscular junction, such as congenital myasthenic syndromes and neonatal transient myasthenia gravis are among the aetiologies. We report on a case of congenital myasthenia caused by mutation in the long cytoplasmic loop of the epsilon subunit of the acetylcholine receptor and a neonate of a myasthenic mother diagnosed with transient myasthenia gravis.]

Lege Artis Medicinae

APRIL 20, 2013

[Health-economic analysis of diseases related to disturbed neonatal adaptation: a cost of illness study]

BONCZ Imre, KOVÁCS L. Gábor, ERTL Tibor, ÁGOSTON István, MOLICS Bálint, BÓDIS József

[OBJECTIVES - The aim of our study is to perform a health-economic analysis of diseases related to disturbed neonatal adaptation by assessing the burden of health care costs in Hungary. DATA AND METHODS - Data were derived from the financial dataset of the National Health Insurance Fund Administration (OEP) covering year 2009. Four diseases were included in the analysis: polycystic ovary syndrome (E2820), retinopathy of prematurity (H3510), hyperstimulation of ovaries (N9810) and respiratory distress syndrome of the newborn (P2200). RESULTS - The annual health insurance costs of the assessed diseases were the following: polycystic ovary syndrome: 78.9 million Hungarian Forints (HUF) or 281 160 Euro (EUR), retinopathy of prematurity: 41.3 million HUF (147 090 EUR), hyperstimulation of ovaries: 8.7 million HUF (30 839 EUR) and respiratory distress syndrome of the newborn: 1.562 million HUF (5 567 336 EUR). We found by far the highest annual per capita health insurance expenditure for respiratory distress syndrome of the newborn (1,43 million HUF or 5098 EUR/patient/year). Hyperstimulation of ovaries (58 073 HUF or 207 EUR/patient/year), retinopathy of prematurity (19 513 HUF or 70 EUR/patient/year) and polycystic ovary syndrome (7679 HUF or 27 EUR/patient/year) was associated with significantly lower annual per capita health insurance expenditure. CONCLUSIONS - The diseases related to disturbed neonatal adaptation have substantial annual health insurance expenditures (1 689 million HUF or 6.02 million EUR) and of these diseases respiratory distress syndrome of the newborn has the highest burden of disease.]

Clinical Neuroscience

SEPTEMBER 23, 2011

[The clinical utility of genetic testing in epilepsy]


[We summarize those epilepsies, in which genetic testing has clinical significance. Different types of genetic tests are presented. Na-channel epilepsies include different clinical entities, the exact genetic diagnosis is relevant in the prognosis, genetic counseling, as well in the therapeutic decision - as Na-channel blockers may worsen them. Molecular genetic tests are available for most of the malformations of cortical development, important for genetic counseling and prenatal diagnosis. Molecular genetic testing of progressive myoclonic epilepsies, which may be difficult to differentiate clinically is almost complete. For some neonatal/infantile epileptic encephalopathies, for most of the neurometabolic disorders, molecular genetic tests are available, so are cytogenetic tests for chromosomal abnormalities accompanied with epilepsy. The clinical significance of the genetic diagnostic of rare, focal inherited epilepsies is limited, their importance is mostly in epilepsy pathophysiology research. The genetic background of the common idiopathic generalized epilepsies is unrevealed so far.]


NOVEMBER 20, 2004

[The pathophysiological role of the cell surface calcium-sensing receptor New clinical entities and drugs, potential therapeutic targets]

TÓTH Miklós

[The extracellular calcium-sensing receptor (CaSR) was recognized and cloned a decade ago. It is a G-proteincoupled receptor that plays an essential role in the regulation of extracellular calcium homeostasis. Diseases known as familial hypocalciuric hypercalcemia, neonatal severe primary hyperparathyroidism and autosomal dominant hypocalcemia are the consequences of naturally occurring mutations of the CaSR. However, the spectrum of the CaSR diseases became more complex with the recognition of both hypo- and hypercalcemic states caused by anti-CaSR autoantibodies. Activating anti-CaSR autoantibodies have been implicated in the pathogenesis of isolated idiopathic hypoparathyroidism and of hypoparathyroidism associated with autoimmune polyglandular syndromes. Inactivating CaSR autoantibodies may cause fluctuating hypercalcemic disorder that resembles primary hyperparathyroidism. The CaSR recently became one of the most intensively investigated target of potential new drugs. Cinacalcet has been approved for the treatment of secondary hyperparathyroidism associated with chronic renal insufficiency and for the management of inoperable or metastatic parathyroid carcinoma.The CaSR may be one of the main molecular target of strontium ranelate, wich is a new antiosteoporotic compound.]

Hungarian Radiology

APRIL 10, 2005

[Dudoenum obstruction caused by duodenal diaphragm]

RUDAS Gábor, SEKYRA Pavel, PUMBERGER Wolfgang, POVYSIL Brigitta

[INTRODUCTION - Duodenum obstruction is a rare gastrointestinal developmental anomaly. It may be complete or incomplete. The incomplete or intrinsic form is often caused by an intraluminal membrane or web duodenal stenosis. The passage is existed with the help of a small perforated lake. Clinically the leading sign is the vomiting. CASE REPORT - A three days old baby admitted because of vomiting. Abdominal X-Ray and US could not found any reason of vomiting but the gastrointestinal contrast series had diagnostic value. Surgery proved the radiological diagnosis. CONCLUSION - For the diagnosis the conventional XRay and ultrasound is not sufficient in every case, the gold standard is the gastrointestinal contrast examination.]