[Bell's palsy in malopolska's children in 2010-2014 years]. / Porazenie obwodowe nerwu twarzowego u dzieci w Malopolsce w latach 2010-2014.

BACKGROUND: Peripheral facial nerve palsy (Bell' palsy, BP) is a not rare diseases in children, being the most common acquired mononeuropathy. AIM: The authors of this study wanted to determine whether the occurrence and course of paralysis changed in the past 5 years (2010-2014). MATERIALS AND METHODS: The study involved Lesser Poland region, where the majority of children with paralysis are hospitalized at the Pediatric Neurology Department of University Children's Hospital in Krakow. These children in subsequent years were admitted to our department without any limitations. A review of clinical documentation of 125 patients, in terms of demographics, the coexistence of other diseases, seasonality, the degree of paralysis, location of paralysis, the prevalence of the recurrence was made. Changes in the structure of the nerve VII in MRI and CT, pharmacological treatment, applied rehabilitation, the degree of improvement and time of hospitalization were analyzed. RESULTS: Similar distribution of occurrence and gender of children with BP in Lesser Poland region within 5 years were observed. The predominance of the girls resulted from demographic composition of the population. BP occurred most frequently in summer and winter. In more than half of children BP occurred in the course of acute systemic infection or craniofacial infection and in 5/125 BP followed head injury. Children with infections required antibiotic therapy. Left-sided paralysis was found in the majority of children and almost half of patients needed protection of the cornea of the eye (significant degree). In 12% of children structural changes within the facial nerve were found. In these children antiviral treatment was used and hospitalization time was more than 20 days while in the majority of children hospitalization lasted 15 days. In 8 (6.4%) children with recurrent BP kinezytherapy, electrical stimulation and laser therapy were applied. Steroid therapy was not used. Only 7/125 chil. dren had mild impairment of the eye closing at the discharge and the others received nearly complete recovery. CONCLUSIONS: Inflammatory etiology is the most common in children with BP. BP occurs more often in the summer and winter. Severity of paralysis was significant in more than half of hospitalized children. Children with structural changes within the nerve VII required longer hospitalization and comprehensive treatment.

[Apt guidelines for applying immunomodulation with interferons in the multiple sclerosis at children]. / Aktualne wytyczne do stosowania immunomodulacji interferonami w stwardnieniu rozsianym u dzieci.

Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system. The disease usually affects young people, although it may also occur in early childhood. As well as the availability of diagnostic methods rises also the possibility of disease-modifying treatment increases. With constant development of diagnostic methods also the possibility of disease-modifying treatment (DMT) increases. Since 2012 in Poland a therapeutic program of the National Health Fund gives the possibility of using first-line therapy treatment in children/adolescents with multiple sclerosis (over 12 years of age), and since June 2015 using the interferon beta in children of 7 years old and body mass below 30 kg has been approved. Interferon beta is the first choice treatment. When selecting interferon type the child's age, lifestyle and the possibility of cooperation must be taken into account. The comprehensive care to a child with MS is based on cooperation of pediatric neurologist, rehabilitation physician, physiotherapist, psychologist and nurse. Training for self-administered interferon injection takes place in the hospital department and under the direct supervision of an experienced nurse. All these elements are taking place in our Clinic. The recent studies from the literature clearly have proven the efficacy and safety of interferon beta treatment in children. Yet agains/still it is a huge challenge to search for an effective and safe drug/ medication but with much more convenient form of administration.

[Latent tetany in child, electrophysiologico-clinical study]. / Dziecko z tezyczka utajona, studium kliniczno-neurofizjologiczne.

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). In this study was presented the case of a child with electrophysiological and clinical manifestation of latent tetany. We report a case of a female patient who was admitted to the Pediatric Neurology Department in the year 2015. Some clinical, biochemical and neurophysiological results have been analyzed.

Stabbing headache in an 8-year-old girl: primary or drug induced headache?

The occurrence of stabbing headaches in children requires a thorough diagnostic approach that excludes secondary headaches. The organic background should be taken into consideration when alarming symptoms occur, such as a purely 1-sided location, a change in the character of the headache, or possibly a link to physical activity. The current study describes the case of an 8-year-old girl who suffered short-lasting stabbing headache attacks. The headaches with increasing intensity and frequency started 1 month before her hospitalization and were usually preceded by physical activity (dancing, running). The pain, which was located in the right supraorbital region, lasted 1 second and occurred several times during the day. No associated symptoms were observed. In addition, the girl suffered from allergic rhinitis and was on antiallergic treatment (levocetirizine, fluticasone nasal spray). On admission she was in good general condition, and a pediatric and neurologic examination revealed no abnormalities. Her brain MRI was normal. The initial diagnosis was that the patient was suffering from primary stabbing headaches. However, during a follow-up visit 4 months later, a relationship was observed between the cessation of the headache attacks and the discontinuation of an antihistaminic drug. Six months later, the girl remained headache free. In cases involving differential diagnoses of stabbing headaches, it is important to consider the adverse reactions of the drugs used.

Level of S100B protein, neuron specific enolase, orexin A, adiponectin and insulin-like growth factor in serum of pediatric patients suffering from sleep disorders with or without epilepsy.

BACKGROUND: Paroxysmal sleep disorders in children are important from both pathophysiological and clinical point of view. Correct diagnosis is crucial for further management. The aim of the present study was to identify peripheral markers of paroxysmal sleep disorders in children, which could improve diagnostics of these disorders. We compared serum levels of several putative biomarkers of neurological disorders, such as S100B protein, neuron specific enolase (NSE), orexin A, adiponectin, and insulin-like growth factor 1 (IGF-1) in pediatric patients suffering from sleep disturbances with those who additionally to parasomnia revealed also epilepsy. METHODS: Fifty six children from 1 month to 18 years of age hospitalized in the Pediatric Neurology Clinic, Chair of Children and Adolescent Neurology, participated in this study. Polysomnographic diagnostics was indicated due to sleep disturbances. Examination was performed with the use of polysomnography and videoelectroencephalography Grass device. Blood samples were taken before registration of sleep, after 2.5 h of sleep or 0.5 h after occurrence of clinical seizures. Concentrations of S100B protein, NSE, orexin A, adiponectin, and IGF-1 were measured by specific ELISA methods. RESULTS: The obtained data showed that serum S100B level was significantly increased in children with epilepsy and clinical seizure attacks as compared to patients with parasomnia only. Atendency to enhanced serum S100B level was also seen in epileptic children without clinical seizures during polysomnographic recording. The level of orexin A was significantly decreased in epileptic children without seizures as compared to the hormone level in parasomnic patients, but was elevated in patients who experienced seizures during polysomnographic examination. As S100B is regarded to be a marker of blood brain barrier leakage and astrocyte damage, the data suggest an increase in BBB permeability in epileptic children, especially during seizure fits. Furthermore, the enhanced S100B serum level without changes in NSE activity may be interpreted rather as an evidence of the elevated secretion of this protein during seizures than of the damage of brain tissue. In contrast to S100B and orexin A level, serum concentration of adiponectin and IGF-1 as well as NSE activity did not significantly differ between the studied groups. CONCLUSION: Out of the five putative biomarkers measured, blood concentration of S100B and orexin A may be helpful in differentiating parasomnic pediatric patients with and without epilepsy.

[Sleep paroxysmal events in children in video/polysomnography]. / Napadowe zaburzenia snu u dzieci w wideo/polisomnografii.

BACKGROUND: It is estimated that about 25% of children have sleep disorders, from short problems with falling asleep to severe including primary sleep disorders. Majority of these problems are transitory and self-limiting and usually are not recognized by first care physicians and need education. AIM OF THE STUDY: Analysis of sleep structure at the developmental age and of sleep disorders associated with different sleep phases on the basis of video/polysomnography results. MATERIAL AND METHODS: Literature review and illustration of fundamental problems associated with sleep physiology and pathology, with special attention to paroxysmal disorders. Additionally 4 cases from our own experience were presented with neurophysiological and clinical aspects. RESULTS: Discussion on REM and NREM sleep, its phases and alternating share according to child's age was conducted. Sleep disorders were in accordance with their international classification. Parasomnias, occupying most of the space, were divided in two groups: primary and secondary. Among primary parasomnias disorders associated with falling asleep (sleep myoclonus, hypnagogic hallucinations, sleep paralysis, rhythmic movement disorder, restless legs syndrome) are important. Another disorders are parasomians associated with light NREM sleep (bruxism, periodic limb movement disorder) and with deeper NREM sleep (confusional arousals, somnabulism, night terrors), with REM sleep (nightmares, REM sleep behavior disorder) and associated with NREM and REM sleep (catathrenia, sleep enuresis, sleep talking). Obstructive sleep apnea syndrome and epileptic seizures occurring during sleep also play an important role. Frontal lobe epilepsy and Panayiotopoulos syndrome should be considered in the first place in such cases. Our 4 cases document these diagnostic difficulties, requiring video/polysomnography examination 2 of them illustrate frontal lobe epilepsy and single ones myoclonic epilepsy graphy in children is a difficult technique and requires special device, local and trained personnel. It is crucial in gathering objective data about sleep disorders. SUMMARY: Correct diagnosis of paroxysmal disorders during sleep in children is possible thanks to video/polysomnography, and enables proper management and pharmacotherpy. It enables improvement or cure disorders during the sleep and moreover enables the obtainment of positive changes in child's every day life.

[The pineal cyst in children with different central nervous system diseases]. / Wystepowanie torbieli szyszynki u dzieci diagnozowanych z powodu róznych chorób osrodkowego ukladu nerwowego.

BACKGROUND: In the group of adult patients being diagnosed from different neurological complaints frequency of pineal cyst occurrence is estimated at 0,1-4,9%. Frequency of its occurrence in pediatric population is unknown, however it is regarded as a common lesion at this age, more frequent in girls and older children. This structural lesion is usually asymptomatic, without sequels and clinical complications. However, neoplastic lesions may also localize in pineal cyst. AIM OF THE STUDY: Characterization of pineal cysts found in children diagnosed from different neurological diseases. MATERIAL AND METHODS: 45 children at the age range from 14 months to 17 years were hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology in the years 2006-2010. MR examination with contrast (with the use of Siemens device 1,5 T) revealed lesions in pineal gland defined as pineal cysts. Predominance of girls was observed in the group (33/45). Additionally older children between 10 and 15 years of age were dominating (27/ 45). In 24/45 children serum tumor markers AFP and betaHCG were determined. Examined group of patients with clinical symptoms which indicated necessity of MR examination performance was very heterogenic. RESULTS: The diameter of pineal cyst was between 3-10 mm in 40/45 children, 13 mm in 2 children and 11 mm in 3 children (most often 4-5 mm in 18 children). Usually this lesion accompanied other congenital or acquired CNS disorders. In 1/3 of children included to the study, headaches were cause of brain MR examination. In 15 children brain MR examination was performed due to epileptic paroxysmal events. No positive values of tumor markers were found in any of examined children. CONCLUSIONS: 1. Pineal cysts are common structural lesions in children hospitalized from different neurological symptoms. 2. Pineal cysts were usually found in children diagnosed because of headaches and epilepsy, and these were the most often final diagnosis. 3. In the examined group children over the age of 10 years and female sex predominate. 4. It is suspected that in majority of hospitalized patients pineal cyst was an incidental finding, with no association with clinical symptoms.

[Cytokines as a marker of the different paroxysmal sleep events in children]. / Cytokiny jako obwodowy marker napadowych zaburzen snu o róznej etiologii u dzieci.

BACKGROUND: Parasomnias in children manifest by unwanted behavior and various clinical picture. These disorders are associated with different sleep phases (REM, NREM) and sometimes threaten safety of children's sleep. They require differentiation with epileptic seizures because about 30% of epileptic seizures is associated with sleep. Some cytokines serum concentration changes were observed in sleep disturbances. AIM OF THE STUDY: The search for peripheral markers of paroxysmal sleep disorders in children, which would be more simple method for differentiating between parasomnias and epileptic seizures. MATERIAL AND METHODS: The study included 21 hospitalized children (17 with epilepsy and 4 with parasomnias) at the age range from 2 months to 14 years. Their 2,5-hour sleep was recorded with videoelectroencephalography. Blood samples were taken two times (before sleep and up to 30 minutes after seizure occurrence or after 2,5-hour registration without seizure). Cytokine concentration (IL-6 and IL-8) was determined in these samples. Statistical analysis of the obtained results was performed. RESULTS: The arithmetic means of both cytokine concentrations did not differ significantly between both examined groups of children, before and after videoEEG performance. Statistically significant differences of mean cytokine concentrations were also not found in children from both groups, between samples after sleep registration and before videoEEG. Comparison of the arithmetic means of IL-6 and IL-8 concentrations after calculating all values before videoEEG and after sleep registration was also performed in children with epilepsy and parasomnias altogether. Similarly, these values did not differ significantly. Comparison of the means of all concentrations of both cytokines between groups of children with epilepsy and parasomnias was performed and also did not differ significantly. CONCLUSION: IL-6 as well as IL-8 concentrations can not have practical use in diagnostics of children's paroxysmal sleep disorders because they do not differentiate basic types of these disorders such as epilepsy and parasomnias.

[Effectiveness of plasmapheresis in adolescent myasthenia gravis treatment]. / Skutecznosc plazmaferezy w leczeniu lekoopornej miastenia gravis u mlodocianej.

BACKGROUND: Myasthenia gravis (MG) is a disease with autoimmune background. Impaired neuromuscular transmission is caused by blockage of acetylcholine receptors on postsynaptic membrane by circulating specific antibodies. Recognition of myasthenia gravis in children, especially its ocular type, may be difficult due to occurrence of similar clinical symptoms in other diseases like ecephalomyopathies. MG is characterized by variety of clinical symptoms and their alternations during excercise and rest. AIM OF THE STUDY: Case report of nearly 18-year-old girl with generalized type of MG. MATERIAL AND METHODS: Before hospitalization the girl had been treated psychiatrically for 6 months due to suspicion of conversion disorders. After performance of clinical test and electro-neurophysiological examinations mysathenia gravis was diagnosed and conservative treatment was instituted. Additionally, on the ground of low effectiveness of the treatment, sterydotherapy and immunosuppressive treatment were instituted without marked clinical improvement. Thymectomy was also low effective. Thymic inflammation was recognised histopathologically. Only after performance of 5 plasmapheresis was significant clinical improvement achieved. CONCLUSION: Plasmapheresis may be used not only in the treatment of myasthenic crisis but also in the treatment of drug-resistant mysthenia gravis.

[Visual perception deficits of cortical origin]. / Zaburzenia percepcji wzrokowej pochodzenia korowego.

This work comprises of a literature review on visual perception distortions that have their origin in structural or functional irregularities of the brain, resulting in the cortex malfunction. The main area that we pay attention to is the brain cortex, but we should not forget, that diseases destructive to the lower brain structures also inevitably lead to secondary dysfunction of the cortex, and thus they have also been included in this paper. Cerebral vision disorders are a small percentage of caseload in either neurology or ophthalmology practice, yet they certainly are interesting for the cognitive scientists, as they open a window into the complex mechanisms of the cerebral clockwork. We are presenting examples of disorders, many of which engage the creative cooperation between specialists from different fields of neuroscience. Three kinds of disorders are presented: vision loss, agnosias and hallucinations. Among others there is some information on cortical blindness, blindsight, Anton's syndrome, hysterical blindness, apperceptive and associative agnosia, prosopagnosia, pure alexia, achromatopsia, Bonnet syndrome, Alice in Wonderland syndrome, peduncular halucinosis etc.

[Children's sleep disturbances in videoelectroencephalography and polysomnography--preliminary data]. / Wstepne wyniki badan wideoelektroencefalograficznych i polisomnograficznych zaburzen snu u dzieci.

INTRODUCTION: Correlation between sleep and epilepsy is one of the most basic issues of pediatric neurology. VideoEEG nad polysomnography play an important role in diagnostic process of seizures occuring during sleep, epileptic seizures as well as parasomnic episodes. AIM: In this paper videoEEG and polysomnography assessment in diagnosis paroxysmal sleep events in children was done. MATERIAL AND METHODS: 25 sleep registrations were performed, videoEEG was performed in 23 children with the age limit of 5 years, and two 8-years-old children underwent total polysomnographic examinations. RESULTS: Examination during sleep time enabled to establish diagnosis of epilepsy in 18/25 children, in 2 children suspicion of epilepsy was made and in 5 epilepsy was excluded. Positive video results were found in 18/25 children and in 7 no clinical symptoms were recorded. Paroxysmal EEG activity was registered in 16 children, in 5 it was abnormal and in 4 it was within normal limits. Frontal epileptic seizures were observed in two children with simultaneous pathological changes in EEG, and in 1 of them in polysomnographic recording. CONCLUSIONS: VideoEEG and polysomnographic examinations in children during sleep are helpful in defining seizure types and parasomnias.

[Causes of symptomatic epilepsy in two first years of life children hospitalized in 2006-2007 years]. / Przyczyny padaczki objawowej dwóch pierwszych lat zycia u dzieci hospitalizowanych w latach 2006-2007.

INTRODUCTION: Epilepsy in two first years 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 two first years of life children from miopolskie and podkarpackie provinces hospitalized in Pediatric Neurology Clinic of Children and Adolescents Neurology Cathedra UJ in Cracow. MATERIAL AND METHODS: 102 children with epilepsy aged from 1 week to 24 months hospitalized between 1st of January 2006 and 31st of December 2007. The group included 47 girls and 55 boys. On the basis of clinical characteristics and results of additional examinations idiopathic epilepsy was diagnosed in 16/102 (13.3%) children and in remaining 86 (87.7%) symtopmatic epilepsy was established. Perinatal burdening was cause of epilepsy in 31/86 (33.72%) children. Other causes were identified in 32/54 children (59.3%) and in remaining 231 54 (40.7%) children the cause was not established. In 3/32 epilepsy occured in the course of hydrocephalus and in 3/32 children as one of CNS inflammation complications. Epilepsy as a result of vascular lesions and bleeding to CNS occured in 4 children. Multiple developmental deffects syndrome was diagnosed in 4 children and in 11 specific neurodevelopmental disorders were the cause of epilepsy. In 6 children epilepsy occured in the course of neurometabolic diseases, neurocutaneous syndromes and neoplasms. In children in two first years of life polimorphic seizures were diagnosed the most often (32/86 that is 37.2%) and tonic, tonic-clonic seizures were less often (21/86 that is 24.43%). Focal seizures occured in 20/86 (23.26%) patients, in 4/86 (4.65%) mioclonic jerks were observed and infantile spasms in 9/86 (10.46%). CONCLUSIONS: (1) In most hospitalized children in two first years of life symptomatic epilepsy was diagnosed. (2) Epilepsy in two first years of life was more often in boys. (3) The most often cause of symptomatic epilepsy was pathology of perinatal period. (4) Polymorphic seizures were the most often manifestation of early childhood epilepsy.

[Neuroboreliosis with motoric disturbations in the developmental age]. / Neuroborelioza z zaburzeniami ruchowymi w wieku rozwojowym.

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. Of these 13 patients, there were 9 males and 4 females, ranging in age between 3-17 years. Neurological diagnostic was performed using ELISA Biomedica kit and western blot bands. A 2-6 week sequential treatment with either iv ceftazidime or amoxicillin and oral doxycycline or amoxicillin was provided. Children were monitored regularly during the next 4-36 months. RESULTS: The 13 children with neuroborreliosis constitute 0.5% of the pediatric neurology department's patients. The clinical manifestation of LD were usual and unusual from patient to patient. They included four cases of facial nerve paralysis (with bilateral paralysis in one case), in three cases transverse myelitis and in a single case, hemiparesis, and oculomotor nerve paresis. In 9/13 children motoric disturbances of neuroboreliosis was diagnosed indeed. The antibiotic treatment was successful in 6 patients and only partially effective in 3 children with facial nerve paralysis. CONCLUSION: The most common symptoms of neuroborreliosis in children was motoric dysfunction.

Caudal regression syndrome associated with the white matter lesions and chromosome 18p11.2 deletion.

Caudal regression syndrome (CRS) is a rare combination of congenital abnormalities characterized by caudal vertebral agenesis/dysgenesis that is usually associated with congenital anomalies of spinal cord, gastrointestinal and genitourinary organs. Although the exact teratogenic mechanism is not known, same environmental, e.g., hyperglycemia and genetic factors appears to play a crucial role in this fetopathy. Herein, we report an unusual case of CRS associated with unspecific white matter lesions and 18p-syndrome manifested by congenital ptosis, hypothyroidism, facial dysmorphy and chromosome 18p11.2 deletion.

CNS Lyme disease manifestation in children.

BACKGROUND: Neurological symptoms develop in 10-20% of children with borreliosis. AIM OF THE STUDY: It was a presentation of clinical manifestation of neuroborreliosis in children. MATERIAL AND METHODS: Children with neuroborreliosis and other neurological diseases were admitted to the University Hospital during 2005-2006 without any selection. Of these 9 patients, there were seven males and two females, ranging in age between 3-17 years. Neurological diagnostic was performed using ELISA Biomedica kit and western blot bands. A 2-6 week sequential treatment with either i.v. ceftazidime or amoxicillin and oral doxycycline or amoxicillin was provided. Children were monitored regularly during the next 4-24 months. RESULTS: The 9 children with borreliosis constitute 0.53% of the pediatric neurology department's patients. The clinical manifestation of LD were usual and unusual from patient to patient. They included three cases of facial nerve paralysis (with bilateral paralysis in one case). In two cases, they included transverse myelitis and in a single case, hemiparesis, meningitis and acute ataxia. Typically, other patients with early stage borreliosis first manifest focal seizures, raising the suspicion that borreliosis could be responsible for triggering seizures. The antibiotic treatment was successful in 7 patients and only partially effective in 2 children with facial nerve paralysis. CONCLUSIONS: The most common symptom of neuroborreliosis in children is motor dysfunction. Acute ataxia may be a clinical presentation of neuroborreliosis. It is probable that borreliosis_triggers seizures in children with EEG abnormalities.

[MRI and MRA data in children with migraine with aura]. / Wyniki badan MR i MRA u dzieci z migrenq z aura.

INTRODUCTION: One of indications to perform structural MRI and MRA is migraine with aura. Results may influence migraine management. THE AIM: THE AIM of this study was an assessment of MRI and MRA results in children with symptoms of migraine with aura and also the analysis if MR results influence migraine management. MATERIALS AND METHODS: During the year 2006, 20 children with migraine were hospitalized in the Department of Pediatric Neurology Jagiellonian University Krakow. There were 11 girls and 9 boys, aged 6-18 years (mean 13,7 years) with symptoms of migraine with aura. In 12 of them visual, in 9 sensory, in 6 dysphasic and in 3 motor aura was present. In 7 children two or more aura types coexisted. In 9 cases family history of migraine was positive. MRI was performed in all children, while additional MRA in 18 children and digital cerebral angiography in 1 of them. MRI was performed in SE T1, FSE T2, PD, FLAIR and IR T1 sequences with 3 or 5 millimetre thickness of slices, in the sagital, coronal and transversal plane, using a 1,5 T system (HiSpeed GE). 3D TOF techniques were used to perform MRA. RESULTS: Results of MRI were normal in 11/20. In other children brain pathology was detected, within lateral ventricular asymmetry (2), demyelination (2), dilatation of CSF spaces (2) and vascular malformation (2). Isolated pathology, such as a calcification of the pineal gland and focal demyelination of vascular origin were detected in individual children. MRA in 16 children did not revealed pathology, however in 2 other children slight asymmetry of distal insular branches of middle cerebral arteries was detected. Aneurysm of the right anterior communicans artery was suspected in one child and it was confirmed by MRA. In one case digital angiography confirmed described on MRI result left vertebral and cerebral posterior arteries hypoplasia and also revealed collaterals in the posterior skull cavity. CONCLUSIONS: Detection of vascular pathology in 20 % of patients with migraine with aura using MRI and MRA is an argument to perform both examinations in such patients.

[Trigeminalgia caused by neurovascular compression in 12 years old girl]. / Trigeminalgia z konfliktu naczyniowo- nerwowego u 12-letniej dziewczynki.

Trigeminalgia is one of the most frequent clinical problems, common in adults but also found in children. In this paper we described a case of 12 years old girl with symptomatic trigeminalgia caused by neurovascular compression, hospitalized in the Department of Pediatric Neurology Jagiellonian University in Kraków. It creates a very difficult diagnostic problem. The girl was first unsuccessfully treated with carbamazepine and afterwards the surgery of neurovascular decompression was performed. We emphasis the crucial role of MR and MRA in cases refractory to classic pharmacotherapy.

[Videoelectroencephalography in children with paroxysmal events]. / Wideoelektroencefalografia w stanach napadowych u dzieci.

The paper presents the crucial role of video EEG, modern diagnostic method, which allowed synchronized recording of clinical status and EEG pattern of the patient. This method gives the possibility to compare these two parameters in term of paroxysmal events. Video EEG allows to diagnosed clinical events associated with bioelectrical discharges (epilepsy), recording of bioelectrical events without clinical seizures, diagnosing clinical attacks without bioelectrical discharges (pseudoseizures) and nonepileptic events (without epileptic character in video and EEG). This method is very useful especially in children and adolescents because of huge polymorphism of clinical signs, more common ambiguous diagnosis in this age and due to heterogeneity of bioelectrical brain function in children. Video EEG monitoring gives the possibility for clinical and electro-physiological interpretation of paroxysmal events and plays a crucial rule in localizing of epileptogenic focus, classification of the seizure, epilepsy type or syndrome. The role of suggestion and placebo is important in diagnosing psychogenic pseudoseizures. The duration of video EEG recording is differentiated and much more shorter in diagnosing the type of the event. Prolonged monitoring is needed in children with drag resistant epilepsy and in pre-operation evaluation.

[The role of videoelectroencephalography in diagnostics of seizures in neonates and infants]. / Znaczenie wideoelektroencefalografii w diagnostyce stanów napadowych okresu noworodkowego i wczesnoniemowlecego.

OBJECTIVES: The clinical characteristic of attack is frequently difficult to unequivocal establishment by observation. It refers especially to seizures in youngest children, due to complicated attack morphology. VideoEEG as a diagnostic tool makes possible more precise establishment of seizures type and derivation. THE AIM: The aim of this analysis was to establish an importance of videoEEG for the differentiation of epileptic and non-epileptic attacks in neonates and youngest infants. MATERIALS: 20 children, 10 girls and 10 boys from the Neurological Division, the Neonatal Intensive Care Unit, the Intensive Care Unit, and the Division of Cardiology, admitted for examination between the 2000 and 2004 years in the Lab of Clinical Electrophysiology of the Department of Pediatric Neurology were included. The age of children, including 7 neonates, was at the moment of examination between 3 and 10 weeks. The first attack occurred between the first day of life and 7th week. In 15/20 of children features of encephalopathy were present. METHODS: The videoEEG recordings were provided in identical conditions, including place and personnel as well, using apparatus PL. 270 video option MedtronicDantec and camera Samsung with movable focus. The visual analysis was provided by two licensed in electrophysiology physicians and results were averaged. RESULTS: In 15/20 children clinical attacks were registered, including stereotypical seizures in 7 children and polimorphic seizures in 8 children. The most frequent attacks were apneic (11/ 20) and the registered seizures were generalised tonic (6/20) and focal (5/ 20). In 5 children attacks were not registered during examinations. In 2 children of this group the bioelectrical activity was also unchanged, whereas in 3 children the significant pathology was detected. Among 15 children with registered in videoEEG attacks, in 9 children ictal discharges were recorded, in 2 children burst-suppression discharges and in the remaining 4 children ictal changes of bioelectrical activity were not recorded. Interictal activity similar to ictal was detected in 2 children with burst-suppression discharges only, and in other cases it was differentiated. 2 of 4 children without discharges during attacks had also normal interictal activity, whereas in the remaining 2 children the immature low voltage flat activity was recorded. Among 9 children with ictal discharges, in 5 children interictal discharges were detected as well, whereas in 2 children only low-voltage activity with pathological features of sleep was detected, but in 2 children interictal activity was normal. The results of videoEEG and other tests allowed to recognized nn epileptic attacks in 5 children, probably epileptic in 1, epileptic seizures in 8 and epilepsy with genetic and hypoxic-ischaemic disorders in 6 children. CONCLUSION: The videoEEG allowed to register clinical seizures in the majority of youngest children with paroxysmal events, contributing to the diagnosis of tonic and polimorphic seizures and to the correct classification of apnoea. In 25% of children with and without registered clinical attacks, this examination allowed to diagnose non-epileptic attacks. To establish epilepsy, the ictal changes of bioelectrical activity were important and interictal as well.

[Epilepsy in three children with Wolf-Hirschhorn syndrome]. / Padaczka u trojga dzieci z zespolem Wolfa-Hirschhorna.

OBJECTIVES: Wolf-Hirschhorn syndrome (4p detetion) belongs to the group of disorders caused by chromosomal aberrations, associated with frequent occurrence of epilepsy. To illustrate phenotype - genotype association, the study presents 3 children with this syndrome and epilepsy. MATERIALS AND METHODS: The diagnosis of Wolf-Hirschhorn syndrome was established in 2 patients during neonatal period and in the third child, the 5 month of life. In the majority of patients characteristic phenotype and associated malformations were detected, and the low birth weight and fronto-temporal diameter of the head as well. The structural neuroimaging revealed the diffuse decrease of brain volume without neurodevelopmental malformations. The earliest manifestation of epilepsy was observed in the 4 month old child with 4p. microdeletion as a status epilepticus. In remaining two children with 4p deletion, generalised tonic-clonic seizures were observed for the first time in the 7th month and 7th year, respectively. The standard EEG was performed in infants, while in the 7 year old child a-one-hour videoEEG was recorded. RESULTS: In older children diazepam and clona-zepam were effective to abort seizures, patients became seizure free on carbamazepin and phenobarbital. In the youngest child, status epilepticus being resistant to benzodiazepins, was interrupted with difficulties. Despite generalised type of seizures, EEG revealed focal changes of bioelectrical activity of the brain in two children. In the 7 month old child there were high voltage slow waves in the parieto-temporo-occipital region, while in the 7 year old child videoEEG demonstrated inter-hemispheric asymmetry and asynchrony, and the presence of epileptic grapho-elements, such as spikes and the spike-slow wave complexes as well. CONCLUSION: Epilepsy appeared in children with Wolf-Hirschhorn syndrome with deletion and microdeletion. Status epilepticus in this syndrome may be resistant to benzodiazepins.