Safety and efficacy of therapeutic plasma exchange in pediatric neuro-immunological diseases.

Purpose: Therapeutic plasma exchange (TPE) is the treatment of choice in various neuro-immunological disorders in pediatric populations. This study assesses the safety and efficacy of TPE in this demographic.

Materials And Methods: A single-center, retrospective cohort study involving pediatric patients who experienced neuro-immunological events and underwent TPE procedures at a tertiary referral center was conducted.

Screening of subclinical P300 event-related potentials changes in childhood acute lymphoblastic leukemia survivors.

Modern treatment of childhood acute lymphoblastic leukemia (ALL) has resulted in a high cure rate; however, it can cause central nervous system toxicity. In the present study, a group of 136 ALL survivors were screened for changes in P300. Therapy was conducted according to a modified New York (NY) protocol (30 patients) and two subsequent revisions of a modified Berlin-Frankfurt-Münster (BFM) protocol (32 and 74 patients).

[Latent tetany in child, electrophysiologico-clinical study].

Tetany is the abnormal state of increased neuromuscular excitability. It is manifested with muscle cramps and spasms, usually associated with abnormal calcium metabolism. This state can be devided into two main types: tetany with clinical manifestaton (hypocalcemic) and occurred more frequently latent tetany (normocalcemic).

[The function of autonomic nervous system during paroxysmal events in children and adolescents, and available diagnostic methods].

Unlabelled: The aims of the study was to establish current knowledge regarding the involvement of autonomic nervous system (ANS) in paroxysmal disorders in children and available diagnostic tools.

Material And Methods: Using key words ANS, epilepsy, syncope and breath-holding spells the search was performed in collections of Elsevier and Springer Journals, as well as MEDLINE/PubMed.

Results: Manifestation of ANS dysfunction may constitute the group of leading symptoms and signs of many diseases, including epilepsy with autonomic attacks and familial dysautonomia.

[Results of autonomic testing in children with migraine with aura, including visual and sensory].

Background: Despite the signs of involvement of autonomic nervous system (ANS) in the pathomechanism of migraine, the significance of its dysfunction was not fully explained. One of parameters serving to establish a balance of both ANS parts is relation of low frequency (LF) to high frequency (HF) heart rate variability (HRV), LF/ HF ratio. HF reflects parasympathetic activity, whereas LF both sympathetic and parasympathetic as well as respiratory rhythm.

[Cerebral blood flow during neurocardiogenic syncopes in children].

Background: Neurocardiogenic syncope, the most common episodic event in children results from global cerebral hypoperfusion. Monitoring of the blood flow during head-up tilt-induced syncope resulted in registration of preferential reduction of end-diastolic velocities in middle cerebral arteries (MCA). The significance of those changes was however not explained.

Seizure-Precipitating Factors in Relation to medical Recommendations: Especially Those Limiting Physical Activity.

Identification of factors precipitating epileptic seizures should always have practical implications and should always result in special recommendations given to patients. The purpose of our study is to analyze the relation between seizure-triggering factors and restrictive recommendations involving limitation of physical activity in particular. The research group consisted of 407 children hospitalized due to seizures.

Schizencephaly-diagnostics and clinical dilemmas.

Background: Schizencephaly is an uncommon congenital disorder of cerebral cortical development. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotropic, dysplastic gray matter.

[Familial case of oral-facial-digital syndrome type 1 (OFD 1)].

Unlabelled: Ciliopathies are phenotypically and genetically heterogeneous disorders that share ciliary dysfunction as a common pathological mechanism. Ciliary dysfunction results in a broad range of malformations including renal, hepatic and pancreatic cysts, visceral abnormalities, retinal degeneration, anosmia, cerebellar or other brain anomalies, polydactyly, bronchiectasis and infertility. The paper presents a familial case of oral-facial-digital syndrome type 1 in 14 year old girl suspected to polycystic kidney disease.

Alternating hemiplegia of childhood: new diagnostic options.

A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides of the body. Age of onset is before 18 months and the common earliest manifestations are dystonic or tonic attacks and nystagmus.

[The influence of child's behaviour on the course of neurological diagnosis].

Introduction: Among the huge amount of neurological diseases that cause diagnostic difficulties, there happens, from time to time an additional difficulty--disorders of psychogenic origin, and among those- conversion disorders. Until now there still is no satisfactionary explanation for the neurobiological basis of conversion. The explanations offered by psychology are also rather general, based on the tradition of psychoanalysis.

[Course of gelastic epilepsy in a boy with non operated hypothalamic hamartoma].

Background: Gelastic epilepsy is usually symptomatic and most often associated with hypothalamic hamartoma. Usually, in the course of this epilepsy different seizure types develop, partial and generalized as well. Moreover, progressive behavioral disorders are observed.

[Familial epilepsy].

Background: Epilepsy is a chronic disease, with heterogeneous etiology, clinical spectrum and prognosis. Among many causes of epilepsy genetic factors and hereditary diseases with different inheritance mechanisms manifesting with epileptic seizures play an important role. Analysis of genealogy of families burdened with epilepsy, development of molecular and genetic tests may contribute to better understanding of mechanisms of epileptogenesis and more effective treatment of epilepsy.

[Arachnoid cysts and epilepsy in children].

Background: At neurodevelopment age arachnoid cysts (ac) are commonly found by accident in neuroimaging examinations performed in the process of differential diagnostics of different diseases, including epilepsy. They are neurodevelopmental disorders which usually develop in the fetal life. Ac are usually supratentorial, less often subtentorial and sporadically interstellar.

[Structural MRI changes in children with epilepsy aged 2-18 years].

Background: Epilepsy can be one of symptoms of the damage to CNS in children, therefore neuroradiological examinations are necessary to complete diagnostics. The guidelines for imaging children with recent-onset epilepsy were published by experts of International League Against Epilepsy.

Aim: The aim of this retrospective research was to analyze results of MRI in children aged 2 to 18 years, hospitalized between years 2008 and 2010 in the Department of Pediatric Neurology of the Chair of Neurology of Children and Adolescents of Jagiellonian University after the first epileptic attack, with recent-onset epilepsy or chronic epilepsy.

[Structural MR of the brain changes in infants with epilepsy].

Background: Epilepsy in first year of life needs constant attention due to diagnostic and therapeutic difficulties.

Aim: The aim of the study was to identify cause of symptomatic epilepsy in infants from młopolskie and podkarpackie provinces hospitalized in Pediatric Neurology Clinic of Children and Adolescents Neurology Cathedra UJ in Cracow.

Material And Methods: 110 children with epilepsy aged from 1 week to 24 months hospitalized between 1st of January 2006 and 31st of December 2009.

[The relationship between bioelectrical activity and cerebral blood flow].

Background: It is widely accepted that neuronal activation leads to a fast localized increase in cerebral blood flow. In the analyzed studies researchers demonstrated findings, aimed to better understanding of neurovascular coupling. Practical consequences are of great importance.

Prenatal stress decreases glycogen synthase kinase-3 phosphorylation in the rat frontal cortex.

It has been postulated that hyperactive glycogen synthase kinase-3 (GSK-3) is an important factor in the pathogenesis of depression, and that this enzyme also contributes to the mechanism of antidepressant drug action. In the present study, we investigated the effect of prenatal stress (an animal model of depression) and long-term treatment with antidepressant drugs on the concentration of GSK-3beta and its main regulating protein kinase B (PKB, Akt). The concentration of GSK-3beta, its inactive form (phospho-Ser9-GSK-3beta), and the amounts of active (phospho-Akt) and total Akt were determined in the hippocampus and frontal cortex in rats.

Pharmacodynamic interactions between antiepileptic drugs: preclinical data based on isobolography.

Background: At least 20 - 30% of epileptic patients do not sufficiently respond to monotherapy. Some of them can benefit from drug combinations; hence, animal data may provide some useful novel clues for rational polytherapy.

Objective: To review combinations of antiepileptic drugs, evaluated with the help of isobolographic analysis, in terms of their efficacy and adverse effects.

[Clinical features of GM1 and GM2 gangliosidosis in own observation].

Background: Lysosomal enzyme defects leeds to intracellular storage and cause damage in many organs, almost always affects central nervous system. AIM. The aim of the study was to reveal the location and clinical characteristics of gangliosidosis in pediatric neurology.

[Clinical manifestation of neoplasm cerebri in children hospitalized in pediatric neurology department].

Background: Brain tumors are the second most common malignancy of childhood, following leukemia. Cause factor of some brain tumors is suggested by genetic predisposition.

Aim Of The Study: It was a correlation between early symptoms of the brain tumor and its histology, localization and MR imaging.

[Neuroboreliosis with motoric disturbations in the developmental age].

Background: Neurological symptoms develop in 10-20% of children suffered borreliosis (LD).

Aim Of The Study: It was a presentation of motoric disturbances of neuroboreliosis in children.

Material And Methods: Children with neuroborreliosis and other neurological diseases were admitted to the University hospital during 2005-2007.

[The type of involvement of the nervous system in multiple sclerosis in children and adolescents].

Introduction: The multiple sclerosis (MS) is characterized by variable clinical symptomatology, neuroradiological changes and neuropathological features, what influence the variable course of disease.

The Aim: It was to describe the type of nervous system involvement in children with MS, considering the clinical manifestation and neuroradiological changes.

Material And Methods: 9 children with multiple sclerosis hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology, in 2006 and 2007 year were included.