A case report of an adolescent with cluster headaches following neck trauma: Coincidence or trigger?

Posttraumatic headaches usually have tension-type or migraine-like characteristics. A correlation between head trauma and cluster headaches (CH) has been previously reported. CH in children are rare and require thorough differential diagnosis. We present an original case of a 15-year-old boy with cluster headaches associated with allodynia probably evoked by a neck trauma. Severe headache attacks started one month after neck trauma. At the beginning clinical presentation of our patient's headaches was very misleading. Headaches were bilateral and associated with infection. Initial diagnosis of sinusitis was made. During further observation headaches have become unilateral with typical for CH associated symptoms and additionally with allodynia. Other causes of secondary CH like cervicogenic headaches, brain tumor and vascular malformation have been excluded. The boy has undergone prophylactic treatment based on flunarizine and gabapentin with good result. Possible pathogenesis of our patient's headaches has been proposed and diagnostic traps discussed.

[Exogenous evoked potentials in adolesccents with muftiple sclerosis]. / Potencjaly egzogenne u mlodocianych ze stwardnieniem rozsianym.

INTRODUCTION: Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disease of the central nervous system. The disease usually affects young people, with a peak onset between the ages of 20 and 40, although it may also occur in early childhood. MS is one of the most common reasons of disability in young people. Aim of the study. The aim of the study was a neurophysiological characterisation of patients with relapsing-remitting MS (RRMS) eligible to receive an. MATERIAL AND METHODS: In this study 23 patients have been included. According to initial symptoms two categories of patients were identified: in one group [group 1] (12123) there were patients with focal signs such as motor andlor sensory abnormalities while the second one [group 2] (11123) consisted of patients with retrobulbar optic neuritis. RESULTS: There were no significant differences in VEP latencies and amplitudes in both I and 2 group. In both groups a significant latency prolongation as well as P100 amplitude decrease has been observed in comparison to the control group. Furthermore, the study has shown that the average latencies of N75 and N135 in group 2 were prolongated when compared to the control group. In BAEP examination no statistically relevant differences have been observed between average latencies and interlatencies in group I and group 2 as well as between the two test groups and control group. In SSEP examination group I has demonstrated a substantial latency prolongation of P14, NIB and N20 when compared to the control group, and the same result for P14, N20, P25 and CCT has been detected in group 2. The average amplitudes of all waves in group I were insignificantly lower than in the control group. In group 2 an insignificant decrease of amplitudes P9 and P18 from the control group has been noticed. In contrast, P25 amplitude was significantly lower. CONCLUSIONS: I. Visual evoked potentials test is an effective neurophysiological method in a diagnosis of subclinical focal demyelination in CNS. 2. Auditory pathway is highly resistant to demyelination processes in CNS. 3. The presence of changes in somatosensory evoked potentials indicates a demand for further precise diagnosis of spinal location of MS.

[P300 potential in chldren with psychogenic nonepileptic events and tension headache]. / Potencjal P300 u dzieci z napadami rzekomopadaczkowymi i napieciowymi bólami glowy.

INTRODUCTION: Psychotherapy is being used as the primary treatment in nonepileptic psychogenic seizures and tension headaches in children. Children's intelectual functioning is related to certain endogenous neurophysiological parameters. AIM: The goal of this study was to establish whether the endogenous potential P300 is different in children with nonepileptic psychogenic events and with tension headaches, and whether it changes under the influence of the cognitive-behavioral psychotherapy. MATERIAL AND METHODS: The study included a group of 47 children: 20 with nonepileptic psychogenic seizures (18 girls and 2 boys), aged 11.09-17.11 years, and 27 children with tension headache (25 girls and 2 boys), aged 10.11-17.11 years. The P300 potential was induced using an auditory stimulus. The reaction time, the amount of mistakes and the percentage of attention focus was measured in all children. All children attended 8-10 psychotherapy sessions. The P300 potential was registered before and after the course of therapy, and additionally in both cycles also after a 3 minutes hyperventilation. RESULTS: Medium P300 parameters were closer to normal in the group of children with tension headaches rather than in the group with nonepileptic seizures. The shorter was the reaction time in the first measurement, the higher the attention score and the shorter the reaction time in the second measurement - this was visible in the results of children with nonepileptic seizures, in contrast to children with tension headaches. The use of hyperventilation caused a noticeable extension of the reaction time in the P300 measurement, with other components unchanged (mistake count and percentage of attention focus). CONCLUSION: The endogenous potential P300 does vary, although on a statistically insignificant level, in groups of children with tension headaches and nonepileptic seizures.

[The influence of cognitive-behavioral therapy on the P300 potential in children with psychogenic nonepileptic seizures and tension headache]. / Wplyw terapii poznawczo-behawioralnej na potencjal P300 u dzieci z napadami rzekomopadaczkowymi i napieciowymi bólami glowy.

UNLABELLED: Explaining associations between neurophysiological and neuropsychological parameteres in children and improving the measurement methods would lead to a better understanding of the pathogenesis and course of psychosomatic disease. Goal: clinical assessment of the efficacy of cognitive-behavioral therapy in the treatment of psychogenic no- nepileptic seizures and tension type headaches in children. Determining the influence of cognitive behavioral therapy on the cognitive P300 potential and whether P300 parameters in children correlate with neuropsychological parameters. MATERIAL AND METHODS: 20 children with nonepileptic psychogenic seizures and 30 children with tension type headaches, aged 11.3 - 17.11 years. The final diagnosis was made in the Paediatric Neurology Clinic. The P300 examination was performed before/after therapy, with/without hyperventilation. A fixed structure therapy was implemented (10 sessions, 90 minutes each), during two weeks of hospitalization or in an outpatient clinic (9 children with tension type headache). The psychological assessment comprised of temperament questionnaires, auditory and visual memory trials, executive function and attention trials, and in some cases also intelligence testing. RESULTS: More significant correlations were found in children with psychogenic seizures: attention parameters correlated negatively with reaction time, and this correlation tended to fade in the second examination, after psychotherapy. In children with tension type headache a statistically insignificant tendency was found of a positive correlation between those parameters. Medium P300 parameteres in this group were better. In 17/20 of children with psychogenic seizures a clinical improvement was observed, in 3 children the symptoms persisted in a 6 month follow up, but of a lower frequency. In 11/27 of chil- dren with tension headache the symptoms persisted, also with a lower frequency. CONCLUSION: cognitive-behavioral therapy is effective in the reduction of symptoms in many cases of psychogenic seizures and chronic tension type headache.

[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.

Dilatation of Virchow-Robin spaces in children hospitalized at pediatric neurology department.

BACKGROUND AND PURPOSE: Dilated Virchow-Robin spaces (dVRs) have been revealed by magnetic resonance imaging (MRI) in patients with various neurological disorders. However, their etiology and clinical importance have not been discovered yet. The aim of the study was to estimate dVRs occurrence in hospitalized children and determine dVRs localization and their association with different nervous system diseases. MATERIAL AND METHODS: Contrast-enhanced brain MRI examinations with the use of 1.5T GE device were performed in children with different diseases of nervous system, who were hospitalized at Pediatric Neurology Department, Chair of Children and Adolescent Neurology, Jagiellonian University in the years 2010-2011. The mean age of examined children was 11.58 years, and the studied group included 27 boys and 26 girls. RESULTS: Within two years, MRI examinations of the brain were performed in 1348 children and dVRs were found in 53 of them (3.93%). Among children with dVRs, 15 were diagnosed with headache (28.3%) and 18 with epilepsy (33.96%). Other diagnoses were less frequent and occurred in 37.7%. Generalized dVRs and those localized in the subcortical nuclei were most frequently found. CONCLUSIONS: Higher incidence of dVRs was found in children with headache and epilepsy. No association was found between localization of dVRs and symptomatology of different nervous system diseases except for large dVRs probably due to the pressure on the surrounding tissues.

Diagnostic difficulties of paroxysmal symptoms in a boy with Parry-Romberg syndrome.

Parry-Romberg syndrome is characterized by progressive unilateral facial atrophy affecting subcutaneous tissue, cartilage and bone structures. Headache attacks and epilepsy are commonly associated with this syndrome but the underlying pathophysiology is still unknown. A case of a 12-year-old boy with Parry-Romberg syndrome and syringomyelia suffering from severe headache attacks and epileptic seizures is reported herein. Headache attacks were associated with bilateral autonomic symptoms and hyperventilation and were usually followed by complex partial and sometimes by secondary generalized tonic seizures. Detailed neuroimaging examinations were performed (magnetic resonance imaging [MRI] of the head, orbits, and spinal cord, MR angiography, and MR spectroscopy of the cerebellum). The EEG pattern revealed localized discharges contralaterally to the affected side. Antiepileptic treatment with carbamazepine was instituted with minimal effect. Modification of treatment (replacement with oxcarbazepine) was successful. In the reported patient interesting correlation of headache attacks, autonomic symptoms and epileptic seizures was observed. Additionally we believe it is the first report of coincident syringomyelia and Parry-Romberg syndrome.

Effects of neurosteroids on the human corticotropin-releasing hormone gene.

Increased activity of hypothalamic-pituitary-adrenal (HPA) axis and hypersecretion of corticotropin-releasing hormone (CRH) are known to be important factors in pathogenesis of some stress-related diseases. Some neurosteroids exert anxiolytic and antidepressant effects probably by inhibition of HPA axis activity. The aim of our study was to find out if neurosteroids can directly affect human CRH gene transcription. The effect of allopregnanolone (ALLO), allotetrahydrodeoxycorticosterone (THDOC), pregnenolone (PGL), PGL sulfate (PGL-S), dehydroepiandrosterone (DHEA) and DHEA sulfate (DHEA-S) on CRH expression was determined in differentiated Neuro-2A cells stably transfected with plasmid containing a fragment of human CRH promoter (-663 to + 124 bp) linked to the chloramphenicol acetyltransferase (CAT) reporter gene. It was found that PGL (0.3-30 µM), ALLO (1-30 µM) and THDOC (1-30 µM) present in the culture medium for 5 days in the concentration-dependent manner inhibited CRH-CAT activity. These neurosteroids also inhibited forskolin-stimulated CRH gene transcription with similar potency. In contrast, PGL-S, DHEA and DHEA-S in a concentration from 0.01 to 10 µM had no effect on basal and forskolin-stimulated CRH activity. Further experiments revealed that wortmannin (an inhibitor of phosphatidylinositol 3-kinase; PI3-K) at concentrations of 0.01 and 0.02 µM did not change the inhibitory effect of ALLO (3 µM) and PGL (1 µM) on CRH gene transcription. Moreover, ALLO (3 µM) and PGL (1 µM) present in the culture medium for 5 days did not change the amount of active, phosphorylated form of protein kinase B (PKB, Akt) and extracellular signal-regulated kinase (ERK). The obtained results indicate that PGL, ALLO and THDOC inhibited basal and forskolin-induced CRH gene promoter activity in the differentiated Neuro-2A cells and that these effects did not depend on the activation of PI3-K/Akt and ERK-MAPK pathways.

[Visual and auditory evoked potentials parameters in multiple sclerosis in children]. / Parametry wzrokowych i sluchowych potencjalów wywolanych w stwardnieniu rozsianym wieku rozwojowego.

BACKGROUND: Evoked potentials (EP) visual (VEP) and auditory (BAEP) are used in diagnostics of demyelinating diseases, especially multiple sclerosis (MS). Elongation of the EP latencies and interlatencies has been found so far and sometimes change in the shape of the responses or decrease of their amplitudes has been registered as well. AIM OF THE STUDY: Characteristics of VEP and BAEP parameters in Polish children with MS. MATERIAL AND METHODS: Analysis included examination results of 10 patients with MS, at the age range from 13 to 17 years, 5 girls and 5 boys, hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology Jagiellonian University Collegium Medicum in Krakow, in the years 2004-2010. Control group consisted of 10 children with similar age and sex distribution. VEP were registered during monocular stimulation with black and white checkerboard pattern reversal (pattern reversal VEP). Responses were registered from three active electrodes O1, O2 and Oz (according to the 10-20 International System of Electrode Placement). Latency of the maximal positive deflection P100, preceding N75 component, following N135 and N75/P100 amplitude were analysed. BAEP were evoked during alternating stimulation of the left and right ear, with the use of acoustic stimulus ("click") at 70 dB HL. The responses were registered from two receiving electrodes localized on the mastoid processes and from reference electrode Cz (according to the 10-20 International System of Electrode Placement). Latencies of I, II and V deflection and interlatencies: I-III, III-V, I-V were analyzed. RESULTS: As far as VEP are concerned, significant elongation of P100 latencies was revealed in children from examined group when compared with control. N75/P100 amplitude differences were not statistically significant in both groups. BAEP analysis revealed significant elongation of III and V wave latencies and III-V, I-V interlatencies. CONCLUSIONS: Evoked potentials, visual and auditory are important paraclinical tests used in MS diagnostics in children. They are used in identification of clinically silent demyelinating foci.

[Specific aspects of child's electroneurography and electromyography: literature and own experience]. / Specyficzne aspekty elektroneurografii i elektromiografii dzieciecej: przeglad pismiennictwa i doswiadczenia wlasne.

BACKGROUND: Despite introduction of modern molecular diagnostics, electrophysiologic examinations still play an important role in the neuromuscular diseases diagnostics and in the disease course and convalescence process monitoring. EMG examination enables determination of location and character of peripheral impairment, establishment of neurogenic or myogenic impairment or neuromuscular transmission disorders and also determination if the process is acute or chronic, localized or generalized. EMG examination is also helpful in detection of subclinical pathology. In neurogenic processes it enables differentiation of spinal and peripheral nerve trunks impairment. MATERIAL AND METHODS: Electrophysiological demyelinization criteria commonly used in adults have limited application in children. In the youngest children electrodes with smaller surface and distance between the poles are used in conduction examination. This cause difficulties in interpretation of the obtained results and requires the use of own norms considering values obtained with the use of smaller stimulating and receiving electrodes. Many technical factors have to be considered in the correct evaluation of electroneurographic and electromyographic recordings, which in adults have significantly lower influence on the results interpretation. Normal EMG result in a small infant does not exclude myopathy especially congenital myopathy. On account of child anxiety usually accompanying the examination the scheme of EMG examination should be elastic. RESULTS: Authors illustrated specificity of these problems in children with own results.

[Structural MR of the brain changes in infants with epilepsy]. / Zmiany strukturalne w badaniu MR mózgu u dzieci z padaczka pierwszego roku zycia.

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 mlopolskie 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 group included 55 girls and 55 boys. On the basis of clinical characteristics and results of neuroimaging synptomatic epilepsy was diagnosed. Perinatal burdening was cause of epilepsy in 35/ 110. Other causes were identified in 34/ 110 children and in remaining 41/110 children the cause was not established. RESULTS: Developmental effects syndrome was diagnosed in 17 children, in cases 6 neurocutaneous syndromes, in 2 inflamatory CSN involvement and in 1 asphyxia were the cause of epilepsy. In children in infancy generalized seizures were diagnosed the most often and facal and polymorphic seizures were less often. CONCLUSIONS: 1. Widely using MR scan provides better recognition structural background of symptomatic epilepsy. 2. Brain atropy was the most often found change. 3. The most often cause of symptomatic epilepsy in the first year of life was pathology of perinatal period. 4. Generalized seizures were the most often manifestation of early childhood epilepsy.

[Structural MRI changes in children with epilepsy aged 2-18 years]. / Zmiany strukturalne w badaniu MR mózgu u dzieci z padaczka w wieku 2-18 lat.

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. MATERIAL AND METHODS: 119 children were included, within children hospitalized as urgent to exclude severe cause and children admitted for diagnostics, whose cerebral MRI revealed pathological changes. RESULTS: The most common anatomical change in MRI was asymmetry of ventricles, revealed in 32 children, within 13 with localized seizures. In 21 children non specific hiperintensive lesions of the white matter were detected, in 19 children seizures were not focal. Cortical atrophy was present in 11 children, seizures in 7 were focal. Venous angioma was diagnosed in 8 children (partial attacks were observed in 4). Dilated Virchow-Robin perivascular spaces were detected in 7 children, within 4 with partial seizures. Vasogenic scar or porencephaly was diagnosed in 6 children, and arachnoid cyst in 9, within 7 with focal seizures. In 4 children epilepsy was a result of hypoxic-ischemic damage, and in 3 resulted from neuroinfection. In 4 children brain neoplasm was detected (in 3 seizures were focal and in one status epilepticus occurred), in 3 other children pineal cyst was detected. In 9 children malformations of CNS were detected, and in single cases epilepsy was associated with Leigh syndrome or NF I, and in one child pituitary microadenoma was detected. CONCLUSION: Severe pathological process for surgical intervention manifested as focal attacks or status epilepticus, indicating the necessity of urgent neuroradiological examinations. In each case of the first attack or epilepsy neuroradiological tests are compulsory not as urgent, for confirming or excluding static lesions. This is important to establish prognosis and long-term management of the child with epilepsy. Children with idiopathic generalized epilepsy were not included, because hospitalization rarely is necessary.

[Arachnoid cysts and epilepsy in children]. / Torbiel pajeczynówki a padaczka u dzieci.

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. They are often the only structural lesions of the brain found in the child with epilepsy. There is still lack of univocal statement whether presence of ac determines type of seizures and focal paroxysmal changes in EEG recording. AIM OF THE STUDY: Estimation the association between cyst localization and changes in bioelectric activity in inter seizure EEG recording and type of epileptic seizures observed in children with ac. MATERIAL AND METHODS: Analysis included 14 children out of 35 with found pineal cyst hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology in Krakow, in the years 2007-2009. There were 4 girls and 10 boys at the age range from 1 month to 14 years. On the basis of clinical symptoms and EEG results diagnosis of epilepsy was established. RESULTS: Pc were usually localized in the temporal region and posterior cranial fossa and had characteristics of single lesions. In minority of children they were co-occurring with other brain development disorders. In children with pc generalized and polymorphic seizures were dominating and in EEG recording generalized paroxysmal changes. CONCLUSIONS: No association between presence and localization of pc and type of epileptic seizures and characteristics of changes in inter seizure EEG recording was found.

[Familial epilepsy]. / Rodzinne zachorowania na padaczke.

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. AIM OF THE STUDY: Research and analysis of epilepsy incidence in families of children hospitalized because of epilepsy. MATERIAL AND METHODS: The study included 18 children with diagnosed epilepsy and positive family history of epilepsy, hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology in the years 2007-2009. Apart from thorough pregnancy and delivery history, brain imaging examinations (MR, CT) and inter seizure EEG recording were performed. RESULTS: Positive family history of epilepsy was found in children at the age range from 1 month to 11 years. In the examined group predomination of older children (over 2 years of age) and girls (10/18) was observed. Epilepsy with generalized seizures was found in majority of patients from examined group. The siblings of the patients were the most often affected with the epilepsy. CONCLUSIONS: Positive family history of epilepsy in children with epilepsy is almost always associated with occurrence of generalized seizures. Even in twins different types of seizures may occur, which may result from structural brain lesions in one of them. In research of familial occurrence of epilepsy repetitive history taking and complementation is needed.

[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.

[Course of gelastic epilepsy in a boy with non operated hypothalamic hamartoma]. / Przebieg padaczki z napadami smiechu u dziecka z nie leczonym chirurgicznie hamartoma podwzgórza.

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. Pharmacological treatment is usually ineffective and surgical resection of the lesion is the only chance of clinical improvement. AIM OF THE STUDY: Presentation of the experience from 5-year observation of the patient with gelastic epilepsy and hypothalamic hamartoma and comparison of this observation with previously reported in the literature with special attention to modern surgical treatment techniques. MATERIAL AND METHODS: 6-year-old boy with gelastic epilepsy diagnosed in September 2004 at the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology. Clinical, neuroimaging and neurophysiological techniques were used. RESULTS: The boy was admitted to the Department because of the attacks of inadequate laughter, aggression and hyperactivity, treated unsuccessfully in the out-patient clinic. On the basis of clinical manifestation and results of MR of the brain, diagnosis of gelastic epilepsy with associated hypothalamic hamrtoma was established. During next 5 years the patient remained under constant multispecialistic care (neurological, neurosurgical, endocrinological, psychological). Laughter attacks were accompanied by complex partial seizures and temporarily by generalized tonic seizures as a result of wrong response to pharmacological therapy. Despite of treatment modification with the use of mono and polytherapy the complete control of the seizures was not achieved only partial reduction. Behavioral improvement was also not achieved. The risk of the operation of the lesion was to high due to its size and location and the boy was not qualified for surgical treatment. Analysis of the literature concerning the surgical treatment in the patients with drug resistant gelastic epilepsy and hypothalamic hamartoma indicates the need of further studies in this area to establish qualification criteria for each type of surgical technique in order to minimize the risk of operative complications. CONCLUSIONS: Diagnosis of gelastic epilepsy is often delayed due to untypical character of the seizures, treated as non-epileptic behavioral disorders. Drug resistance in this type of epilepsy cause search of better and better surgical techniques and establishment of criteria enabling to choose optimal method for every patient.

[Visual evoked potential parameters in multiple sclerosis in developmental age]. / Parametry wzrokowych potencjalów wywolanych w stwardnieniu rozsianym wieku rozwojowego.

INTRODUCTION: Abnormal results of visual evoked potentilas (VEP) are typical for demyelinating diseases of the central nervous system, including multiple sclerosis. Elongation of P100 latency was the most frquent finding and sometimes changes in the shape of VEP responses or decrease of the amplitudes were observed. AIM OF THE STUDY: Results of VEP in children with multiple sclerosis. MATERIAL AND METHODS: 11 patients in the developmental age with multiple sclerosis were included, the age range was 13 to 17 years, 6 girls and 5 boys, hospitalized at Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology, Jagiellonian University in the years 2004-2009. The control group consisted of 11 children with analogous age range and sex distribution. VEP were recorded during monoocular visual stimulation with black and white checkerboard pattern reversal (pattern reversal VEP). The responses were recorded from three active electrodes O1, O2 and Oz (according to the international 10/20 electrode placement system). Latencies of maximum positive deflection P100, preceding N75 component and following N135 and amplitude of N75/P100 were analyzed. RESULTS: Elongation of P100 latency in the examined group was statistically significant when compared with controls. Differences of N75/P100 amplitudes did not differ statistically between the groups. CONCLUSIONS: VEP are one of the most important paraclinic tests used in diagnosis of MS in children.

[Usefulness of electromyography in diagnostics of the neuro-muscular diseases]. / Przydatnosc badania EMG jako wskaznika dla dalszych badan w chorobach nerwowo-miesniowych.

INTRODUCTION: Electrophysiological examinations still play an important role in initial diagnostics of neuromuscular disorders and monitoring of the disease progress or recovery process. AIM OF THE STUDY: Evaluation of neurophysiological examinations usefulness in differential diagnosis, indicating diagnostic and/or therapeutic management in patients with suspicion of neuromuscular disorders. MATERIAL AND METHODS: 109 patients were included, hospitalized at Department of Pediatric Neurology Jagiellonian University and treated at Neuromuscular, Neurologic, Orthopedic and Rehabilitation Outpatient Clinics of the Children's Hospital in Krakow. 6 groups of patients were indicated: I-11 patients with benign acute childhood myositis, BACM), II-18 children with gait disturbances, III-36 patients with suspicion of neuropathy, IV-11 patients hospitalized with suspicion of muscular disorders, V-18 children with SMA suspicion and V-15 patients with suspicion of myasthenia (MG). Neurophysiological examinations were conducted within 5 years (from May, 2004 to May, 2009) with Keypoint device from MedtronicDantec. RESULTS: In the first group, EMG examination, performed in the acute phase of the disease, did not reveal any abnormalities. Indicators of inflammatory process were normal, however in 5 patients transient elevation of CK was found. Diagnosis of BACM in this group was established. Couple-month observation of children after BACM did not reveal deficits of muscular tone and strength, recurrence of the disease, or elevation of CK, despite consecutive respiratory tract infections. In 4/18 patients with gait disturbances EMG examination revealed abnormalities. In 1 patient myogenic injury of the muscles was found, in 3 conduction in motor fibers of examined peripheral nerves was disturbed. In 14/18 children EMG examination did not reveal any pathological changes. In 17/36 children from group III diagnosis of inflammatory neuropathy was established (Guillain-Barre Syndrome, GBS), in 2 chronic inflammatory demyelinating polyneuropathy, and in 2 others multifocal motor neuropathy with conduction block. In 7/36 patients familial sensory-motor polyneuropathy was diagnosed. Neuroboreliosis was cause of neuropathy in 2 children. In 1 child, segmental inflammation of anterior horns of the spinal cord evoked by Coxackie virus was revealed. Friedreich disease, Nieman-Pick disease, thoracic outlet syndrome was found in others. In 1 boy symptoms of polyneuropathy and encephaloptahy occurred in the course of tal intoxication. In group IV EMG examination showed myopathic injury of the muscles in 9 children. In 2 others the examination results were normal. Kearns-Syre syndrome was found in one of them and Duchenne disease in the second one, 16, 5 years old boy without pain complaints, Becker disease in 2 and in next 2 patients encephalopathy and in rare cases BaCM, congenital dystrophy and myotubular myopathy. SMA was diagnosed based on clinical manifestation and EMG examination in 18 patients. EMG examination showed lower motor neuron injury in every child with SMA type I and II. MG was diagnosed in 15 patients based on clinical manifestation and positive result of fatigability test. Ocular myasthenia was found in 2 patients, bulbar type of MG was found in 1 and systemic myasthenia in 12 children. In electrophysiological fatigability test amplitude of the first response was normal in every patient and decrease of amplitude in response 4:1 in patients with MG was from 26 to 88%. CONCLUSIONS: Electromyographic examination remains important diagnostic tool of neuromuscular disorders. In order to limit extension of differential diagnostics EMG should be performed in its early stage.

[Migrainous aura subtypes in hospitalized children]. / Rodzaje aury migrenowej u hospitalizowanych dzieci.

INTRODUCTION: Migraine with aura is characterized by reversible focal neurological symptoms preceding or accompanying headache. Visual aura is the most common type of aura and its patognomic symptoms are scintillating fortification migrating across the visual field or scintillating scotoma. However, the symptoms are not always so typical and clinical doubts are greater when negative symptoms (loss of vision, numbness or paresis) are present. Differential diagnosis of migraine with aura includes in the first place transient ischemic attack (TIA) as well as epilepsy. Diagnosis of migraine in the developmental age is more difficult and associated with unprecise description of the symptoms. Thorough history taking is crucial in migraine diagnosis and following management. Knowledge of migraine with aura symptoms, clinical differences associated with developmental age and features enabling differentiation with other disorders imitating migraine is very important. AIM OF THE STUDY: Description of aura types and characteristics of migraine headache and their accompanying symptoms in children. MATERIAL AND METHODS: 30 children were included, aged 8-17 years (mean 13,6 years), hospitalized at Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology, Jagiellonian University in 14 months of years 2008 and 2009. Clinical analysis was based on inquiry addressed to the patients and their parents. RESULTS: Migraine with visual aura was established in 9 patients, with somatosensory aura in 4, visual and somatosensory in 5, visual and dysphasic in 1, visual, somatosensory and dysphasic in 5, somatosensory and dysphasic in 2 and basilar type migraine in 4 patients. Consequently, symptoms of visual aura were present in 20/30 patients, in 9 of them it was the only type of aura and in 11 coexisted with other aura types. In more than half cases it manifested as simple visual phenomenons (spots, dots, frills, lines). Blurred vision was found in 5 patients (bilateral in 3, unilateral in 3) and hemianopia in 2 (bilateral in aura was observed in 16/30 patients and only in 4 of them it occurred without other aura symptoms. Dysphasic aura accompanied other aura types in 8 cases. Basilar type migraine was established in 4 patients. Unilateral migrainous headache occurred in 20/30 patients. Pain intensity was defined as very severe, severe and moderate in the following 10, 12 and 8 patients. Pulsating quality of the pain was found in 15 cases. Aggravation of headache by movement was observed in 22/30 patients. Nausea was the most common accompanying symptom present in 20/30 patients. Vomiting, photophobia and phonophobia occurred in 15 patients each. Coexistence of nausea and/or vomiting, photo- and phonophobia was reported in 12 cases. Vertigo and balance disturbances were frequently found additional symptoms and occurred in 11 and 2 patients and in 6 coexisted together. Positive family history of migraine was reported in 18/30 patients. CONCLUSIONS: 1. Visual and somatosensory aura were the most frequent types of aura in children; basilar-type aura occurred with the lowest frequency. 2. Unilateral headache with severe or very severe pain intensity, aggravated by movement was found in more than half cases. Pulsating quality of headache was present in half of the patients. 3. Accompanying symptoms (nausea, vomiting, photo- and phonophobia) occurred in the combination, fulfilling ICHD II diagnostic criterion D for migraine in less then half cases. Vertigo and/or balance disturbances, were commonly found symptoms with no association to aura type. 4. Familial occurrence of migraine was reported in more than half patients. 5. Another modification of migraine diagnostic criteria for children is needed. 6. History takes crucial role in the diagnosis of childhood migraine.

[Value of the experiment for developmental neurology]. / Znaczenie eksperymentu dla poznania zagadnien neurologii wieku rozwojowego.

Some neurological diseases cannot be at present efficiently treated, because of their unknown pathogenesis and the lack of appropriate drugs. The etiology of autism is not known and there is no drug for ameliorating basal symptoms of this disease. Some research was conducted to obtain an adequate rodent model of autism in which potential drugs can be studied. Therapeutic action of psychostimulant drugs in the attention deficit hyperactivity disorder (ADHD) are known for years, but because of their addictive properties, decision about their clinical use in a child is not easy. The precise recognition of their mechanism of action and determination of the maximal but safe doses should facilitate making the correct decision. Only about 70% children suffering from epilepsy are efficiently treated with one drug, while in the remaining the use of two or more drugs is necessary, which increases the the risk of side effects. Clinically more effective classical antiepileptic drugs can disturb cognitive functions in the child, therefore, on the basis of the present knowledge the experiments are under way with the aim of receiving drugs with strong anticonvulsant properties but without serious side effects. The introduction of efficient neuroprotective drugs, which may ameliorate secondary neuronal cell damage in various brain regions to the therapy is the main aim of numerous experimental works. At present it seems that neurotrophic factors can be useful but they must be administered centrally. Transplantations of transfected cells capable of producing neurotrophic factors or stem cells to the brain may be in the future an efficient method for improving brain function.