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Introduction The co-occurrence of headache and epilepsy is well-documented in the adult population. The aim of the prospective study was to analyse in the paediatric population the correlations between the types of peri-ictal headaches and types of seizures. Furthermore, an attempt was made to find trends in characteristic features of peri-ictal headaches. Material: A total of 57 children with peri-ictal headache were enrolled in the study. The participants’ guardians were asked to keep a diary of the seizure and peri-ictal headache episodes during a 180-day period. During follow-up visits, systematic history regarding peri-ictal headaches was taken. Results A total of 913 seizure and 325 peri-ictal headache episodes were noted during the study. Post-ictal headaches were most common, occurring in Conclusion Peri-ictal headaches are a significant health problem for patients with epilepsy. The most common type are post-ictal headaches, and they are most likely to appear after a generalised seizure.

Bole glowy stanowią istotny problem wśrod populacji pacjentow z padaczką. W zalezności od okresu ich wystepowania dzielimy je na bole glowy miedzynapadowe i okolonapadowe. Kryterium podzialu okolonapadowych bolow glowy jest czasowa zaleznośc ich wystepowania w stosunku do napadu padaczkowego. W artykule przedstawiono najnowsze kryteria diagnostyczne bolow glowy przypisanych napadom padaczkowym, stworzone przez Miedzynarodowe Towarzystwo Bolow Glowy. Scharakteryzowano rowniez wszystkie typy okolonapadowych bolow glowy, zarowno te zawarte w trzeciej, najnowszej Miedzynarodowej Klasyfikacji Bolow Glowy, jak i te, ktorych w tej klasyfikacji nie wyszczegolniono.

The data on the prevalence of the Restless Legs Syndrome/Willis -Ekbom disease (RLS/WED) in the population of teenagers is scarce. The aim of this study was to determine RLS/WED occurrence in adolescents, its diagnostic accuracy, family history, clinical characteristics and impact on everyday functioning.A group of 2379 pupils (aged 13-18 y.o.) from 6 randomly selected secondary schools in Gdańsk, Poland were screened for RLS/WED with the use of a questionnaire. In order to verify the diagnosis and perform additional tests (neurological examination, psychological evaluation, biochemical blood tests, demographic questionnaire, International RLS rating scale/IRLSS, Epworth daytime sleepiness scale). all of the respondents with RLS/WED suspicion and their parents were asked for a consultation by a child neurologist. Both children and parents with RLS/WED diagnosis were tested with actigraphy at home for at least two consecutive nights.Two thousand and ninety seven students (88,15%) filled the questionnaire correctly (1171 girls and 926 boys, 56% and 44%). Sixty four respondents were suspected of having RLS/WED (3,1%), however, 36 of them were diagnosed as RLS/WED-mimics (mainly positional discomfort). Finally, 21 (1%) were diagnosed with definite idiopathic RLS/WED. The average age of symptom onset was 10.96 years. The severity was moderate in the most of the cases (61.9%) and the course of the disease was intermittent in all of them. Family history was positive in 80%. Abnormal actigraphy (PLMS index5/h) was present in 80%. Blood level of ferritin was low (50 ng/ml) in 85%. Excessive daytime sleepiness and school problems affected almost half of them. The presence of RLS/WED symptoms was associated with disrupted sleep, behavioral problems (irritability, aggression, hyperactivity), attention deficit and lowered mood. No correlation between RLS/WED and attention deficit hyperactivity disorder (ADHD), nocturnal enuresis or primary headaches was found. Thirty eight percent of the patients sought medical help, but none of them obtained proper diagnosis nor treatment of RLS/WED.In this study restless legs syndrome affected 1% of Polish teenagers, in the majority of cases was idiopathic and associated with positive family history. It affected sleep and everyday functioning. Neurological consultation is essential to avoid false positive diagnoses of RLS/WED in teenagers.

Background The Prechtl General Movement Assessment (GMA) predicts various neurological and developmental disorders while also documenting therapeutic effects. Aims To describe the temporal organization of fidgety general movements in infants with mild to moderate postural asymmetries and/or tonus regulation problems, and to analyze to what extent the temporal organization of fidgety movements will change after physiotherapy. Study design Repeated measure design. Participants Twelve infants (five females) with mild to moderate postural asymmetries and/or tonus regulation problems were admitted for an early intervention program. The gestational age ranged from 27 to 40 weeks (Median, 36 weeks; nine infants born preterm) with birth weights ranging from 740 g to 3500 g (Median, 2590 g). Measures Fidgety movements and their temporal organization were measured using the Prechtl GMA at 9 to 19 weeks post term age (Median, 14 weeks) before and after an early motor training procedure. The movements of one of the infants were analysed using a computer-based approach, measuring the mean and standard deviation of quantity of motion, height of motion and width of motion. Results Seven infants had sporadic fidgety movements, and five had intermittent fidgety movements. None had continual fidgety movements before the intervention was initiated. After intervention, the temporal organization of fidgety movements increased in all infants. The observations of these movements were supported by computer-based analysis. Conclusion The study indicates that early intervention increases the temporal organization of fidgety movements in infants with postural asymmetries and/or tonus regulation problems. The clinical significance of this finding needs to be further evaluated.

Introduction Damage to the central nervous system in children with infantile cerebral palsy (ICP) can cause aberrant and uncoordinated muscle contraction resulting in postural instability. Hippotherapy is a method of motor rehabilitation, rooted in neurophysiology, which utilizes the natural motion of a horse's gait to entrain trunk stability in the seated rider. Aim The aim of this research was to assess the effect of hippotherapy in improving trunk stability in children with spastic ICP. Material and methods The research was conducted on 24 children with ages ranging from 2 to 18 years (average age: 10.63 ± 4.95) who suffer with spastic ICP. Each child underwent physiotherapeutic assessment twice, an initial assessment at the start of the study period and a follow up assessment after 3 months of hippotherapy. All study subjects undertook 2 sessions of hippotherapy each week for a period of 3 months. Results and discussion An increase in the maximum tension of the rectus abdominis muscle was observed in 17 out of 24 participants (81%). Further analysis of response to therapy according to subtype of ICP revealed an improvement in 13 out of 16 children with spastic diparesis and in 3 out of 7 children with tetraparesis. Improved trunk stability was seen in 6 out of the 11 children who were mobile with a wheelchair, and in 11 out of 12 independently mobile children. Conclusions This study proves that hippotherapy has a significant impact on improving the trunk stability of children with ICP.

Background Pediatric ischemic stroke is an important cause of morbidity and mortality. As previous studies of children after stroke showed, dyslipidemias were very common in Polish and other European populations. Thus, looking for genetic factors predisposing to pediatric stroke, its symptoms, and outcome, we have analyzed 2 polymorphisms of the upstream stimulating factor 1 (USF-1) gene. Materials and Methods The study group consisted of 82 children with stroke, 156 parents, and 146 controls. We used 2 alternative methods: the case–control model and the analysis of families using the transmission disequilibrium test. The 2 polymorphisms, rs2516839 and rs3737787, were genotyped using the TaqMan Pre-Designed SNP Genotyping Assay. The Statistica 10.0 software was used in all statistical analyses. Results We did not observe any statistical differences in genotype and allele frequencies between patients and controls. There were also no significant differences in the transmission of alleles from the parents to the affected children. However, we have observed that the TT genotype of the rs2516839 polymorphism was more common in patients with epilepsy and dysarthria, whereas the TT genotype of the rs3737787 polymorphism was more frequent in the group of patients with a decrease in intellectual functioning. Conclusions Our study did not show any associations between the 2 analyzed polymorphisms of the USF-1 gene and pediatric ischemic stroke. However, we have observed an influence of specific genotypes on the outcome of stroke, including epilepsy, dysarthria, and a decrease in intellectual functioning.

Purpose The most common causative factors of CSF otorrhea in children are injuries and congenital abnormalities of the temporal bone. Spontaneous CSF leak as a consequence of congenital temporal bone defects may result in recurrent meningitis. Diagnosis and management of such an entity are particularly difficult in early childhood. Materials and methods The aim of this study was to investigate clinical features and to discuss possible methods of treatment of spontaneous CSF otorrhea in children. Results Severe unilateral sensorineural hearing loss or total deafness was found in children with CSF otorrhea. CT and MRI of the temporal bones revealed dehiscences in the walls of the tympanic cavity and defects of the inner ear, which were confirmed intraoperatively. Lateral petrosectomy and closure of the fistula with muscle tissue and fat obliteration cavity were performed. The children remain free of otorrhea and recurrences of meningitis. Conclusion The diagnosis of spontaneous otorrhea in children is based on the severe unilateral sensorineural hearing loss and presence of CSF in the middle ear cavity. It may be successfully treated by means of lateral petrosectomy with obliteration of the vestibule with muscle tissue and tympanic cavity with fat tissue.

Background The association of 9p21.3 locus single nucleotide polymorphisms with arterial ischemic stroke in adults was demonstrated in many studies, but there are no studies in pediatric arterial ischemic stroke patients. We investigated whether the 9p21.3 locus polymorphism, namely rs10757278, is associated with the arterial ischemic stroke risk in children. Methods The study group consisted of 335 individuals: 80 children with arterial ischemic stroke, their biological parents (n = 122), and 133 children (age and sex matched) without any symptoms of arterial ischemic stroke as a control group. The rs10757278 polymorphism was genotyped using the TaqMan® Pre-designed SNP Genotyping Assay (Applied Biosystems). Two different study design models were used: family-based association test (transmission-disequilibrium test) and case-control model. Results There were no statistically significant differences in the distribution of genotypes and alleles of the rs10757278 polymorphism between groups of children with arterial ischemic stroke and controls. The frequency of both transmitted alleles in transmission-disequilibrium test analysis was identical (50%). The A allele carrier state (AA+AG genotype) was more frequent in arterial ischemic stroke children with hemiparesis than in patients without this symptom (94.5% versus 68.0%, P = .004). Conclusions There is no evidence to consider the 9p21.3 locus polymorphism as a risk factor for childhood arterial ischemic stroke.

The 677C>T polymorphism within methylenetetrahydrofolate reductase (MTHFR) gene is related to an elevated level of homocysteine. Thus it may be considered as a genetic risk factor in ischemic stroke. Apparently studies of this type of polymorphism in childhood stroke have shown conflicting results. We performed meta-analysis of all the data that are available in relation with MTHFR polymorphism and the risk of ischemic stroke in children. We searched PubMed (last search dated December 2010) using “MTHFR polymorphism”, “ischemic stroke” “child”, “children”, “pediatric stroke” as keywords and reference lists of studies and reviews on the topic. Finally, 15 case–control studies corresponded to the inclusion criteria for meta-analysis. These studies involved the total number of 822 children and adolescents after ischemic stroke and 1,552 control subjects. Fixed or random effects models were used depending on the heterogeneity between the studies. The association between ischemic stroke and 677C>T polymorphism within MTHFR gene was observed in three of the studies. The pooled analysis showed that TT genotype of MTHFR gene is more common in stroke patients than in controls (p = 0.0402, odds ratio = 1.57, 95 % confidence interval 1.02–2.41). The Egger’s test did not reveal presence of a publication bias. The results based on a sizeable group of cases and controls have proved that the 677C>T polymorphism in MTHFR gene is associated with the development of ischemic stroke in children.

Background The association between headache and stress is discussed. A powerful source of stress and the ways of relation between stress and headache can differed. The individual vulnerability to stress may be related to one's temperament. Objective The purpose of the present study was to evaluate the role of temperament traits in children with episodic tension-type headaches (ETTH). Methods We examined 120 children, 6–16 years of age, in the Department of Developmental Neurology of the Medical University of Gdansk. All children were diagnosed with ETTH. The control group consisted of 60 age-matched children without headaches. The following instruments of assessment were used: survey-interview, EAS Temperament Survey (Buss and Plomin), and Stress Response Scale (Chandler). The results were evaluated using statistical analysis. Results Our results showed differences in temperament traits in children with ETTH compared to children without headaches. The former demonstrated greater temperament instability, i.e., higher emotionality, an intensified level of fear, a lower level of vigour, and a higher level of shyness compared to the control group. Conclusion Children with ETTH have some different traits than children without headache.

Objective – To evaluate the contribution of antiphospholipid antibodies (aPL) and thrombomodulin (Thm) in the pathogenesis of stroke and migraine in children. Materials and methods – Ninety children were included in the study: 30 children (4–15 years) after an ischemic stroke of an unknown etiology; 30 migrainous patients (8–15 years), who were hospitalized in the Department of Developmental Neurology, Medical University of Gdansk, Poland, and 30 healthy children of the same age. Results – The statistical analysis showed an increase in the values of anticardiolipin antibodies (aCL), anti β2-glycoprotein 1 (β2-GP1) and Thm in children with stroke and migraine than in the control group. The resultant values were higher, but stayed at standard. Conclusion – The possible role of prothrombotic factors in individual cases of pediatric stroke and migraine cannot be excluded.

Moderate hyperhomocysteinemia is one of the risk factors of pediatric stroke. Methylenetetrahydrofolate reductase (MTHFR) is an important enzyme, which regulates homocysteine metabolism, and some polymorphisms of gene encoding this enzyme are associated with a decreased activity of the enzyme. The aim of the study was to assess an association between the A1298C polymorphism and pediatric stroke. We also evaluated a possible synergistic effect of A1298C and C677T polymorphisms of this gene. The study group consisted of 88 children after ischemic stroke, 142 of their parents and 111 controls. The A1298C polymorphism was genotyped using the restriction fragment length polymorphism method. We used 2 study designs: a case–control model and a family-based association test. The Statistica 7.1 and EpiInfo 6 softwares were used in all analyses. We did not observe any statistically significant differences either in the transmission of the A allele in the family-based test or in the frequency of the A allele in the patients group compared with the controls. We also did not notice any significant additive or synergistic effects between the A1298C and C677T polymorphisms. An analysis of the results obtained in this study and a critical review of previously published studies indicate that the A1298C polymorphism of the MTHFR gene is not related to ischemic stroke in children.

Background/purpose Data on the role of the −455G > A polymorphism of the gene encoding β fibrinogen subunit ( FGB ) and the Thr312Ala polymorphism of the gene for the α fibrinogen subunit ( FGA ) in childhood ischemic stroke are insufficient. Therefore the aim of the study was to evaluate a possible association between these two polymorphisms and arterial ischemic stroke. Methods The study group consisted of 85 children after ischemic stroke, 146 of their parents and 159 controls. Both polymorphisms were genotyped using the restriction fragment length polymorphism method. Two study designs were used: a case–control model and a family-based transmission-disequilibrium test. Statistica 7.1 and EpiInfo 6 softwares were used in all analyses. Results In the TDT test, a tendency to a higher transmission of the 312Ala allele of the FGA gene and the −455A allele of the FGB gene was observed, however, it was statistically non-significant. The frequencies of alleles and genotypes of both FGA and FGB genes polymorphisms did not differentiate children from both groups also in the case–control model. Additive or synergistic effects between FGA and FGB genes polymorphisms were not observed. Conclusion An analysis of the results obtained in this study and a critical review of previously published data indicate that examined gene polymorphisms are not related to ischemic stroke in children.

BACKGROUND The investigation of a possible association between the FII, FV, FVII, and FXIII genes polymorphisms and pediatric ischemic stroke (IS). METHODS The study group consisted of 392 individuals, including 81 children with IS, their biological parents (n=162), and 149 control children. The polymorphisms were genotyped using polymerase chain reaction-restriction fragments length polymorphism method. The relation between analyzed polymorphisms and the disease was tested by 2 independent methods: family-based association test-transmission/disequilibrium test (TDT) and classic case-control model. RESULTS We did not observe any preferential distribution of any analyzed allele from parents to the affected children. For the FVII gene polymorphism, there was a trend toward a higher frequency of the R allele. In a case-control model, the differences between the patients and controls in the frequency of the Q allele, Q allele carriers, and RR homozygotes lay close to the border of statistical significance (P=0.08). There were no significant differences in genotype and allele distribution between patients and controls in case of other polymorphisms. CONCLUSIONS Analyzed polymorphisms of coagulation factors are not significant determinants of pediatric IS in the studied population; however, these findings require a confirmation in a larger group of participants.

Ischemic stroke remains one of the top ten causes of death in children. There is evidence for the role of pro-inflammatory cytokines, such as IL-6 and the -174GC promoter polymorphism of the IL-6 gene, in the occurrence and outcome of stroke in adults. The aim of the present study was to determine a possible association between the -174GC IL-6 polymorphism and occurrence of paediatric stroke, its symptoms and outcome.The study group consisted of 340 individuals: 80 stroke children, 122 parents of patients and 138 controls. The -174G/C polymorphism was genotyped using the RFLP method. For the analysis of the relationship between genotypes and stroke we used two alternative methods: the case-control model and the transmission test for linkage disequilibrium using data from families.We observed no differences in the transmission of alleles from parents to children. We also did not find any statistical differences in distribution of genotypes and alleles between patients and controls. However, the analysis showed that post-stroke epilepsy was genotype-dependent. All children with epilepsy were G allele carriers and none of them was a CC homozygote whereas about 25% of children without epilepsy had the CC genotype.Our study did not show any associations between the IL-6 -174 GC polymorphism and the occurrence of stroke but we observed a relation between post-stroke epilepsy and the G allele carrier-state.

Pediatric ischemic stroke, though relatively rare, remains an important medical problem since 20-40% of patients have recurrent strokes and 50-85% of them suffer from long-term neurological deficits. Approximately 20-50% of the affected children have prothrombotic disorders, therefore upon looking for possible genetic causes of the disease we focused on the plasminogen activator inhibitor (PAI-1)--the major inhibitor of fibrinolysis. The aim of the present study was to investigate a possible association between the -675_-674insG PAI-1 gene polymorphism and pediatric ischemic stroke. The study population consisted of 343 individuals: 70 children with ischemic stroke, 140 their biological parents and 133 control children. The PAI-1 gene polymorphism was genotyped using the restriction fragment length polymorphism and was visualized by AgNO₃ staining. The transmission/disequilibrium test showed exactly the same transmission of alleles from parents to the affected children (37:37). The case-control model also did not reveal any statistical significance in alleles and genotypes distribution between patients and control children. The obtained results suggest that the 4 G/5 G polymorphism of the PAI-I gene is not a risk factor of ischemic stroke in Polish children.

Background and purpose Reactive oxygen species play an important role in the physiology and pathology of cerebral arteries, including ischaemic stroke. The cytochrome b-245 alpha gene ( CYBA ) encodes cytochrome b-245 alpha light chain (p22phox peptide), a critical element of NAD(P)H oxidases, the most important source of superoxide anion in the cerebral arteries. To search for genetic factors associated with paediatric ischaemic stroke, the possible association between CYBA gene C242T polymorphism and the disease was evaluated. Material and methods The study group consisted of 238 individuals: children with ischaemic stroke ( n = 70), their biological parents ( n = 118) and children without any symptoms of stroke ( n = 50). The C242T polymorphism was genotyped using polymerase chain reaction – restriction fragment length methodology. To evaluate the possible association between polymorphism and stroke, the transmission disequilibrium test and the case-control method were applied. Results The C242 allele was transmitted more frequently than 242T (62.2% vs. 37.8%) but observed frequencies did not differ significantly from expected ( p = 0.10). There were also no significant differences in allele and genotype distribution between patients and control subjects (patients: CC – 50.0%, CT – 38.6%, TT – 11.4% vs. controls: CC – 52.0%, CT – 36.0%, TT – 12.0%). Conclusions The study did not show that the C242T polymorphism of the CYBA gene is a risk factor of ischaemic stroke in children.

Genes related to platelet and arterial endothelial function have been recently considered as independent risk factors for stroke. We aimed to analyze a relationship between the E-selectin 98G > T polymorphism and stroke in children and to observe the transmission of E-selectin alleles from heterozygous parents to their affected children. We studied 59 children after stroke, 112 parents, and 87 healthy children. The E-selectin 98G > T polymorphism was analyzed with the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. The frequency of the 98T allele in patients was almost twofold lower than in controls (5.1% vs. 9.8%, p = 0.145, odds ratios (OR) = 0.49) as well as carriers of the 98T allele (19.5% in controls vs. 8.5% in cases, p = 0.067, OR = 0.38). The G allele of the E-selectin 98G > T polymorphism was more frequently transmitted to the children after stroke compared to the T allele (68% vs. 32%). In conclusion, we did not confirm the relationship between the 98G > T polymorphism of the E-selectin gene and childhood ischemic stroke. There is still a need for further studies.

A quantitative systematic review recently discussed the role of thrombomodulin (Thm) and antibeta2-glycoprotein I (beta2-GPI) in cerebral strokes in adults. Little is known about the problem in children. The aim of the study was to see if there is a difference in the values of Thm and beta2-GPI in children with ischemic stroke.Seventy patients were included, comprising 40 children who had had ischemic stroke of unknown etiology hospitalized from January 1995 to December 2005 at the Department of Developmental Neurology, Chair of Neurology Medical University of Gdańsk, and 30 healthy volunteers (no autoimmunologic disease or headache in interview). The concentrations of thrombomodulin (Thm) and antibeta2-glycoprotein I (beta2-GPI) in A, M, and G immunoglobulins were determined according to an immunoenzyme method (ELISA).None of the investigated subjects had elevated levels of beta2-GPI. The patients with stroke had significantly higher Thm values than the healthy group.This finding of elevated levels of thrombomodulin in cases of pediatric cerebral stroke could help in measuring the extent or duration of parenchymal brain injury, or even perhaps response to future therapeutic maneuvers. All these implications may aid not only in the diagnosis and management of acute ischemic stroke, but encourage prophylactic action to prevent probable stroke relapse.

Hemiplegic migraine is a specific form of migraine with aura including hemiparesis. It is a rare condition of unclear aetiology. The authors present a case of a 14-year-old boy with persistent foramen ovale, suffering from sporadic hemiplegic migraine. The authors broadly describe the symptoms and types of hemiplegic migraine as well as underlining diagnostic and therapeutic difficulties of the attacks. The patient suffered from recurrent severe headaches, vomiting and co-existing focal neurological symptoms, including alternant hemiparesis. On the basis of the history, thorough clinical observation and numerous accessory investigations a diagnosis of sporadic hemiplegic migraine was established. However, the last attack, with prolonged neurological deficits, was treated as a complication of migraine and defined as persistent aura without infarction. The authors also broadly discuss a differential diagnosis, including other stroke-like incidents, vascular diseases, immunological and mitochondrial disorders that mimic the symptoms and clinical course of sporadic hemiplegic migraine.

Ischemic stroke in children is relatively rare, but it remains an important medical problem. Previous studies on Polish children have implicated dyslipidemias as significant risk factors in stroke. To search for genetic factors associated with the disease, the possible association between apolipoprotein E gene epsilon polymorphism and childhood stroke was evaluated. The study population consisted of 243 individuals: 72 children with ischemic stroke and 100 of their biological parents and 71 children without any symptoms of stroke. The apolipoprotein E gene epsilon polymorphism was genotyped using restriction fragment length polymorphism methodology. To analyze the possible association between this polymorphism and stroke, the transmission disequilibrium test and the case-control model were used. No preferential distribution of any allele from parents to the affected children was observed. There were also no significant differences in genotype and allele distribution between patients and control subjects. Study findings did not confirm that epsilon polymorphism of the apolipoprotein E gene is a risk factor of ischemic stroke in children.

Restless legs syndrome (RLS) is one of the most common neurological diseases. Even though there is a large progress in its diagnosis, pathophysiology and treatment, it still remains recognised too rarely. It is elderly people who mostly suffer from restless legs syndrome, but it is established that the disease may begin in childhood. The aim of this study is to present the diagnostic criteria and differential diagnosis in cases of this syndrome, in children. Correct diagnosis of restless legs syndrome is a starting point for epidemiological studies on the incidence of RLS in children.

Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias. The aim of the study was assessment of the most frequent symptoms on the basis of long term observation of children with NF1 and presentation of implemented diagnostic and therapeutic procedures.In our department there are 149 children (71 boys and 78 girls) aged from 7 months to 18 yrs with diagnosed or suspected NF1. Each child is carefully followed up every 6 months on outpatient basis. Paediatric, neurological and opthalmological examinations are performed during the first visit and in cases of any new symptoms. Number of Lisch nodules, vision field, audiogram, dermatological evaluation of skin abnormalities as well as orthopaedic examination are also investigated. In any case of NF1 without neurological symptoms, MRI of the brain and spine is carried out every 2 years. Moreover, each child is consulted in the Genetic Clinic.Cafe-au-lait spots were observed in all 149 children, freckling of the armpits in 40, peripheral neurofibromas in 30, Lisch nodules in 2 patients. Secondary symptoms and complications such as mental retardation (9 cases) and epilepsy (10 cases), cognitive disorders and learning disabilities (21), abnormalities in MRI examination (53), benign or malignant CNS tumours (9), scoliosis (99) were diagnosed. In 5 patients malignant neoplasms occurred (3.4%) including: RMS--2 cases, Triton tumour--1 case, MPNST--1 case. Two children died of disease progression, one of treatment complications (sepsis) and two children are alive.1. Patients with NF1 need regular specialist medical care. 2. Continuous education of the families with this disease is necessary. 3. Diagnostic and therapeutic procedures recommended for patients with NF1 need to be implemented at different levels of health care.

Cerebral vascular anomalies have been rare pathology of the brain in childhood. The most frequent clinical symptoms of them are: intracerebral hemorrhage, subarachnoid hemorrhage, headache, neurological deficits and epilepsy.Because of very few reports about epilepsy in cerebral vascular anomalies in children the authors continued studies in this subject.The subject of the study were 20 patients in the age between 5 to 15 years with cerebral vascular anomalies hospitalized in the Department of Developmental Neurology Medical University of Gdansk in the years 1980-2001. The clinical state and CT, MRI, angiography and EEG recordings were evaluated in all the patients. Arteriovenous malformations (AVM) were diagnosed in 13 (65%), in 5--angioma cavernous (25%), in 2--aneurysms (10%).In 4 children epilepsy was the first symptom of cerebrovascular anomalies. Epileptic seizures were observed in 4 patients in the acute stage of the disease. In 6 children epilepsy was the consequence of arteriovenous malformation.Epilepsy occurred in 50% of children with vascular malformations. Among cerebrovascular anomalies, arteriovenous malformations are the most frequent cause of epilepsy in the group of examined children.

Objective In order to compare the cognitive functions in children with newly diagnosed temporal (TLE) versus frontal lobe epilepsy (FLE) and control group which consisted of healthy children the following prospective study was performed. Material and methods 39 children with TLE 24 children with FLE and 24 healthy subject were included in study. Each child had neuropsychology assessment using age-normed and validated instruments. The applied test battery consisted of measures assessing both intelligence as well as executive and motor skills. The children were reassessed one and two years. Results In all epilepsy patients there was no evidence of anatomical brain damage. There were no differences in mean age and gender between the groups. There was no significant difference in global IQ scores between the groups. Children with FLE had significantly lower scores than the other two groups in non-verbal memory tasks, presented higher attention deficit and had slower performance speed. The TLE group performed significantly worse as compared with control group in verbal learning and performance speed with no differences in attention. The correlations between the results of test and the localization of epileptic foci were performed. In long term outcome the “frontal” group performed significantly worse than other groups. detailed analysis of cognitive impairment was performed. Conclusion The children with new onset FLE present with more severe cognitive and attention problems in course of disease compared with TLE group, however the TLE group differ significantly in some measures with control group.

Objective Data from the literature show that 34–47% patients with epilepsy suffer from periictal, mainly postictal headache. There are poor information about correlations between type of headache with children's age and sex, type of seizures, type of eeg changes, localization of headaches and differentiation if the headache is preictal, ictal or postictal in children. The aim of the study was to evaluate the frequency of the preictal, ictal and postictal headaches in children with epilepsy and correlation between these symptoms with types of seizures and changes in eeg. Methods The prospective study conducted in the Department of Developmental Neurology in Medical University of Gdansk, include 30 patients, 6–17 years old, with diagnose of epilepsy (according to ILAE criteria) and periictal headaches. The study group was divided into three subgroups according to the headache: preictal, ictal or postictal. Results In 76% children focal seizures, in 40% secondary generalized and in 16% primary generalized seizures were observed. 73% patients suffered from postictal headache, ictal headache occurred very rarely (7%). The mean intensity of the pain measured with the VAS scale was 6.5. We found the correlation between lateralization of headache and interictal changes in eeg in patients with focal seizures. Conclusion The frequency of periictal headaches correlates with type of seizures and type of changes in eeg.

There are only a few investigations in the literature, that address the occurrence and the role of anticardiolipin antibodies (aCL) in children with migraine. The results of those studies are often contradictory. The aim of the study was to determine if the values of aCL in children with migraine differ from the control group. We tried to assess whether the type of migraine (with aura or without aura) had the influence on those values.Sixty patients (mean age: 10.9+/-3.3 years), including 30 children with migraine hospitalized from January 2000 to December 2003 in the Department of Developmental Neurology Medical University of Gdańsk and 30 healthy children, were studied. The values of aCL in class IgA, IgM and IgG were assessed by the immunoenzymatic method (ELISA test).The values aCL in IgA and IgG class were significantly different between the migraineurs and control group. The mean value of aCL in patients with migraine was 8.7+/-1.27 U/ml, while in the control group--3.81+/-1.74 U/ml. The positive values of aCL in class IgG were found in 11 (37%) children with migraine, and positive values of aCL in class IgM were noted in 6 (20%) cases in the same group. The type of migraine had no influence on the values of aCL.Children with migraine present with the higher values of aCL than the control group. The mean values of aCL were within the normal range, therefore their role in pathogenesis of migraine remains unclear. The further observation is needed to assess the reliable role of higher values of aCL in pathophysiology of vascular disorders.

Headaches which can last for many months and even years belong to the most frequent consequences of closed head injuries in children. The method which makes it possible to demonstrate the presence and extent of the nervous tissue damage caused by trauma is the regional cerebral blood flow (rCBF) evaluation with single photon emission computerized tomography (SPECT). In the present study the regional cerebral blood flow (rCBF) was assessed 10-15 days after trauma, 3 months and one year in cases of brain concussion. We tried to establish whether there was a correlation between changes in rCBF and the occurrence of posttraumatic headache.SPECT was applied in 32 children, aged 6-16, 10-15 days, and then 3 and 12 months after brain concussion. In all children no changes were found in CT and MRI examinations.In the studied group in the early period after trauma, blood flow impairment was found in 21 children, mostly in frontal areas. One year after trauma the rCBF improved in 11 children, in 10 cases the pattern was normal. In a group of 4 children with headache one year after brain concussion, three of them still presented the impairment of blood flow.SPECT shows a great sensitivity and usefulness in the assessment of consequences of head trauma and it can explain some posttraumatic complaints in children.

SIR–Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the paediatric population, characterized by unilateral painful ophthalmoplegia of, typically, a relapsing-remitting course and a substantial response to corticosteroids. The symptoms of THS are not specific and clinical criteria themselves do not guarantee a firm diagnosis. Neuroimaging or biopsy confirming granulomatous inflammation is essential to ad iagnosis of THS. Although diagnostic criteria were established by the International Headache Society in 1988, then revised in 2004 (Table I), 1 THS remains a diagnosis of exclusion of other causes of painful ophthalmoplegia, (e.g. neoplasms, aneurysms, cranial neuropathy [diabetes mellitus], opthalmoplegic migraine, intracranial vasculitis, pseudotumor of the orbit, basal meningitis, or cavernous sinus thrombosis). 2

Patent foramen ovale (PFO) may act as a causal or triggering factor for migraine attacks. A recent quantitative systematic review of the relationship between PFO and migraine has revealed that the already existing data which imply increased prevalence of PFO in patients with migraine attacks, increased prevalence of migraine attacks in patients with PFO, and improvement (even total abolition) of migraine attacks, after PFO closure in migraineurs are supported by little evidence. 3,4 The authors of this review, however, state that no conclusion can be drawn about the efficacy of PFO closure in migraine treatment, as further studies are needed. To our knowledge, we report the first case of a 14-yearold male whose SHM attacks disappeared after PFO closure. The 14-year-old male was admitted urgently to the Department of Developmental Neurology, Medical University of Gdansk, with severe headache (most prominent in fronto-temporal region bilaterally) accompanied by nausea and vomiting preceded by visual disturbances, dysarthria, weakness of the left limbs, and facial asymmetry that appeared about an hour before admission. Twelve months earlier he had been diagnosed in our clinic with probable sporadic hemiplegic migraine (the results of paediatric and neurological examination, neuroimaging, laboratory tests, and electroencephalograph were all negative).