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Amyotrophic lateral sclerosis (ALS) is a progressive, incurable, neurodegenerative disease affecting the upper and lower motor neuron, which inevitably leads to the impaired fitness of patients and therefore deterioration of their quality of life., Aim: The aim of the study was a comparative assessment of two forms of ALS in terms of limb function and electrophysiological parameters of peripheral nerves., Materials and Methods: 20 persons participated in the study, where 10 suffered from bulbar-onset ALS and 10 had limb-onset ALS. Patients were examined clinically and electrophysiologically three times at three-month intervals. Rene Zazzo's card test and Mira Stambak's line-drawing test as well as the 10-metre walk test were used to assess limb function. The results of electrophysiological conduction in the area of nerves of upper and lower limbs were subjected to statistical analysis, as were the results of tests used to assess limb function., Results: The comparative analysis of the obtained results demonstrated that patients with limb-onset ALS showed significant deterioration of conduction in proximal nerve sections compared to patients with bulbaronset ALS. Clinical reflection of this was impairment of limb function with a tendency for progression of these changes over time., Conclusions: Progressive impairment of conduction in the area of proximal nerve sections and ventral roots of spinal nerves is reflected by the increasing disability of limbs in patients with ALS. In the course of the disease, patients with limb-onset ALS show both worse nerve conduction and limb function than patients with bulbar-onset ALS., (© 2018 MEDPRESS.)

Objective: Introduction: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting upper and lower motor neurons, presenting with various manifestations, leading to progressing disability, with poor prognosis, and with no options for successful treatment. In its classic form the central and peripheral motor neurons are simultaneously affected at the beginning; the bulbar-onset ALS successively involving other parts of the nervous system is slightly less common. The aim: To demonstrate a correlation between electrophysiological parameters of peripheral nerves and loss of manual dexterity in the ALS., Patients and Methods: Materials and methods: The analysis covered results of electrophysiological tests of motor conductivity in median and ulnar nerves, and results of the Mira Stambak and Rene Zazzo tests normally used to evaluate lateralisation, while in this study they were adapted to verify manual dexterity. The study covered 20 patients with clinically confirmed or possible ALS determined on the basis of the EI Escorial criteria. Half (10 people) of the studied group had limb-onset ALS, while the other half had the bulbar-onset ALS., Results: Results: When evaluating a correlation between the results of electrophysiological tests and the results of the Mira Stambak and Rene Zazzo tests, a significant relationship was found between a reduction in an amplitude of the compound muscle action potential (CMAP) and deterioration in manual dexterity in the subjects, with a tendency for progression in these deviations, but with their interdependency maintained., Conclusion: Conclusions: 1. A loss of motor cells in the anterior horns of the spinal cord is reflected in the deterioration of manual dexterity in ALS patients. 2. A significant correlation is found between a loss in manual dexterity and an increase in changes in motor conductivity.

Introduction: Guillain-Barre syndrome (GBS) is an acute polyradiculoneuropathy with a variable clinical presentation and is the most common cause of acute palsy in children. The classic presentation is an acute monophasic, nonfebrile, postinfectious illness manifesting as an ascending flaccid palsy with areflexia. Aim. Analysis of children with GBS in 6 years observation., Material and Methods: In the present study we describe a population of 21 children (19 boys and 2 girls) with GBS hospitalized in the years 2009-2014 in our department. In this group 20 children (95%) had flaccid palsy of limbs, 13 children (62%) had previous respiratory or digestive infections. 17 (81%) children were treated by plasmapheresis, 3 (14%) by intravenous immunoglobulin with good outcome., Conclusions: For 6 years mechanical ventilation was not used in children with GBS.

Background: Ischemic strokes in children are rare. The etiology and risk factors for ischemic stroke in children and adolescence differ from those typical in adults. Clinical symptoms depend on the location, extent of damage of the central nervous system and age. The aim of the study was to compare the clinical picture of ischemic stroke in children at two periods., Material and Methods: The study included children who had a stroke: 32 children (group I) in the years 1968-1998 and 27 children (group II) in 2010-2015. In each case medical history, physical and neurological examination, laboratory tests and neuroimaging were taken. To determine the location of ischemia in children The Oxford Community Stroke Project classification was used. To determine the score of hemiparesis the Ashworth scale was used., Results: Pregnancy, delivery, neonatal period and neurological history in the majority of children enrolled to the study, was unbounded. Etiology of stroke in first group was found in 20 children. Diagnosis of ischemic stroke was made on the basis of angiography in 13 children, while the remaining 19 based on head CT. TACI were found in 11 children, while PACI had 21 children. In the Ashworth scale I score of paralysis was observed in 14 children; 2 score in 5 children. 3 score had 11 children and 2 children 4 score of paralysis. In the second group, the etiology of stroke was found in 6 children. Ischemic stroke in all children was diagnosed based on CT scans. Additionaly, 24 children had MRI, 4 children had CT angiography and in 5 children MRA. TACI was diagnosed in 3 children, in the next 21 children PACI and POCI in other 3 children. The Ashworth Scale score 1 paralysis were observed in 8 children and score 2 at 16. 2 children had paresis score 3, in 1 paralysis had score 4. No child in group I and II, had hemorrhagic stroke. In the pharmacological treatment of the acute phase of stroke steroidotherapy and diuretics were used, in children with infection antibiotic therapy was introduced. Antithrombotic therapy was administered in 2 children. Neuroprotective treatment (piracetam) and rehabilitation therapy was used. Children with ischemic stroke in group I were hospitalized an average of 80 days. Hospitalization time in group II was an average of 23 days., Conclusions: 1. In spite of dynamic development of laboratory diagnosis detection of the etiology of ischemic stroke in children has not improved. 2. Computed tomography is still a primary diagnostic tool in the diagnosis of stroke in children. 3. In both groups of children hospitalization in the years 1968-1998 and 2010-2015 clinical consequences after ischemic were observed. 4. Majority of children after ischemic stroke has a small degree of paralysis. 5. Better availability of outpatient multidisciplinary rehabilitation treatment enabled significantly shortened hospitalization of children in 2010-2015.

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.

Background: Myasthenia gravis (MG) is an autoimmunologic disorder. It is characterized by various clinical symptoms and their dependency upon the exertion and the rest as well., Material and Methods: Between 2002-20014 in the Neurophysiology Laboratory at the Chair of Pediatric and Adolescent Neurology, Jagiellonian University in Krakow, the electrophysiological repetitive nerve stimulation study were performed in 44 children. The clinical picture and positive electrophysiological test were the ground to diagnose MG in 19 of them (12 girls and 7 boys). The mean age at onset of MG in the examined group was 12.8 years. In 5 patients the diagnosis was completed using edrophonium test. In 14patients the titer of AchRAb was also tested. In 18 patients with MG the radiological examinations of the chest were performed. In 14/19 patients with MG the control electrophysiological testing was performed 2 to 8 months after the first one., Results: The gen-ralized MG was diagnosed in 14 patients, and ocular in 2 of them. The amplitude of electro-physiological testing was normal during the first response in all patients, but the decrement of amplitude 4:1 in patients with MG was 26% to 88%. In 3 patients with MG the persisted thymus, while in 7 hypertrophy of thymus, in 2 thymoma nad inl thyinflammation of the thymus was detected. In 5 children the result of chest examination was normal. Among 5/8 patients (62.5%) with positive AChRAb, in one ocular MG was diagnosed, in the other 7 generalized MG. The titer of AChRAb was between 0.4 and 30.8 nmol/l (mean 9.44 nmol/l), and the decrement of amplitude 4:1 was 22% to 58%.. In the treatment pirydostygmine bromide (Mestinon) was used in all children, however in 4 of them together with azathioprine, and in 7 with steroids. In the treatment of myasthenic crisis in 5 patients plasmapheresis was performed. In 42% of patients thymectomy was performed during the first year after diagnosis. The clinical remission was succeed in 90,9% patients. The electrophysiological control examination detected the decrement of amplitude 4:1 in 1% to 80% (mean 36%). The electrophysiological remission, correlating with clinical remission was achieved in 2 patients. In 6/10 patients with clinical remission, the decrement of amplitude 4:1 in electrophysiological examination continued to be at the level similar to initial examination. In 1 patient remission was complete allowing significant reduction of doses of Mestinon, even though in the electrophysiological test the decrement was 80%., Conclusions: (1) Among hospitalized children, the generalized myasthenia was the most common. (2) Neurophysiological studies plays still fundamental role in diagnostic methods in the diagnosis of myasthenia gravis in children. (3) The correlation of the clinical state and electrophysiological results was not established.

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.

Background: Epilepsy in first year of life needs constant attention due to diagnostic and therapeutic difficulties., Aim: The aim of the study was to identify cause of symptomatic epilepsy in infants from młopolskie and podkarpackie provinces hospitalized in Pediatric Neurology Clinic of Children and Adolescents Neurology Cathedra UJ in Cracow., Material and Methods: 110 children with epilepsy aged from 1 week to 24 months hospitalized between 1st of January 2006 and 31st of December 2009. The 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.

Neuroblastoma (NBL) is one of the most common childhood malignancies. Presence of untypical symptoms is characteristic for this malignancy; therefore it may mimic many other illnesses, both malignant and nonmalignant. Particular attention should be focused on opsoclonus-myoclonus syndrome (OMS, Kinsbourne's syndrome). In this paper three children with NBL previously diagnosed with OMS are presented. There is a need to test children with OMS for NBL as more than 30% of them are diagnosed with a tumor of neural origin. Children with NBL suffering from OMS have usually a favorable outcome with respect to their malignancy. However, the neurological and cognitive deficits are present for a long time and even they may become more intensive in spite of immunosuppressive therapy.

Background: In the population of children and adolescents, epilepsy affects 0.5-1% of individuals; approximately 3% of general population suffer from non-epileptic seizures, while endocrine disorders are several times more frequent. All of the above factors result in a relatively common non-accidental occurrence of endocrine disorders, epilepsy and neuropsychiatric disorders. However, structural central nervous system (CNS) abnormalities that cause both endocrine and neurologic disorders seem to be markedly less common. No reports addressing this problem are available in the literature., Aim of the Study: 1) Assessment of the frequency of non-coincidental occurrence of epilepsy and endocrine disorders in inpatients and outpatients with structural CSN abnormalities managed in Department Endocrinology. 2) Presentation of diagnostic and therapeutic difficulties in these patients, and 3) An attempt at defining a common etiology of both disorders., Material and Methods: A retrospective analysis of the medical records of the patients with coincidence of endocrine disorders and epilepsy and psycho-neurologic disorders (treated in Chair and Department of Children's and Adolescents Neurology, University Children's Hospital of Krakow or in another pediatric neurology center) and with organic CNS abnormalities (treated or followed up as inpatients and outpatient of Department of Pediatric Surgery, Children's University Hospital of Krakow, was performed. The patients were selected from among several thousands of children treated as inpatients and outpatients of the Department., Results: Various forms of symptomatic and idiopathic epilepsy and other psychoneurological disorders (disorders of behavior and emotions, obsession-compulsion syndromes, stereotypias, aggression, compulsive ideas and movements, anorexia or hypothalamic obesity) coincident with one or more endocrine disorders such as precocious or delayed puberty, multihormonal pituitary deficiency, panhypopituitarism and secondary hypothyroidism were detected in 42 patients with suprasellar arachnoid cyst (7 patients), septo-optic dysplasia (8 patients), craniopharyngioma (15 patients), glioma of the optic chiasm in neurofibromatosis type 1 (NF-1) (12 patients). There were no endocrine disorders in any of the ten patients with hamartoma of the hypothalamus and CPP. Endocrine and/ or neurological disorders did not resolve or were progressive after neurosurgery. Of 42 patients, a group of seven children representative for individual disorders was selected. In those patients, the etiology of both endocrine disorders, epilepsy and neuropsychiatric disorders was suspected to be common., Conclusion: 1. Various structural CNS abnormalities are the cause of concomitant epilepsy and endocrinopathy, although in some cases a direct impact of a genetic factor on the occurrence of both disorders or a mere coincidence cannot be ruled out. 2. Psychoneurological disorders usually precede the onset of endocrinopathy. 3. For this reason, MR or CT CNS imaging should be performed in any case of central neurological disorders, disorders of behavior, epilepsy, obsessive-compulsive syndrome, but also in patients with delayed psycho-motor development, delayed or accelerated growth and pubertal development. All of the above-mentioned manifestations may be symptoms of structural CNS abnormalities and their early treatment determines the child's future.

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.

Background: The term hypoparathyroidism refers to a group of disorders in which a relative or absolute deficiency of PTH leads to hypocalcemia and hyperphosphatemia., The Aim of the Study: Was to evaluate clinical symptoms in patients with hypoparathyroidism during normocalcemic period and to try to establish its etiology (electrolyte imbalance, organic central nervous system lesions, coincidence of tetany and epilepsy)., Material and Methods: The analysis included a group of 14 patients with hypoparathyroidism: 3 boys and 11 girls, aged from 12 months to 31 years (median 16.11 years), with duration of the disease 12 months to 26 years (median 10.9 years). In all the patients, the diagnosis was confirmed based on history, physical examination, results of biochemical and hormonal laboratory tests, radiological and neurological examinations. All the patients were followed by endocrinology specialists. Low phosphorus diet, calcium, magnesium, active vitamin D supplementation and management of other endocrine disorders were employed., Results: In 9 patients, pseudo-hypoparathyrodism was diagnosed; of this number, in 8 children, type Ia Albright syndrome was confirmed. Five patients were diagnosed as true hypoparathyroidism, two girls in this group were found to have autoimmune hypoparathyroidism as a component of the autoimmune polyglandular syndrome type 1, 2 others were diagnosed in infancy as congenital hypoparathyroidism and 1 girl had true hypoparathyroidism as a component of Kearns-Sayre syndrome. Five patients were referred to neurological department with epilepsy suspicion. In the medical history, 9 patients had generalized epileptic seizures, moreover, 1 girl manifested absence attack and balance disturbances. In 3 patients, EEG demonstrated changes typical of generalized seizure activity. In 5 patients on anti-epileptic management, additional calcium and active vitamin D treatment was initiated, allowing for achieving seizure remission. CT of the head and pituitary gland showed calcification foci in the central nervous system in 9 patients. Five patients of the eight individuals with Albright syndrome showed mild or moderate mental retardation confirmed by psychological testing., Conclusions: 1. Hypoparathyroidism leads to functional and morphological CNS changes. 2. Restoring metabolic balance through administration of calcium and active vitamin D preparations may obliterate the need for anti-epileptic treatment. 3. Calcification foci in the central nervous system seem to be associated with the duration of hypoparathyroidism. 4. No correlation has been observed between the extent and location of calcification foci and neurological abnormalities. 5. Hypoparathyroid patients with calcification foci in CSN require long-term multidisciplinary medical management and neurophysiological, imaging and neuropsychological monitoring.

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.

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.

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.

Background: In the population of children and adolescents, epilepsy affects approximately 1% of cases, nonepileptic seizures are seen in approximately 3%, and endocrine disorders are several times more common. For this reason, coincidence of endocrine disorders and epilepsy and psychoneurologic disorders is frequent. Much less common are structural abnormalities (tumors, developmental abnormalities), and especially non-structural CNS abnormalities, resulting in coincidence of both disorders. There are no reports available in the literature that would address the problem., Aim of the Study: 1) Assessment of the frequency of coincidental epilepsy and endocrine disorders in patients without structural CSN abnormalities treated as outpatients and inpatients of Department of Endocrinology University Children's Hospital of Krakow. 2) Presentation of diagnostic and therapeutic difficulties in these patients, and 3) An attempt at defining the common etiology of both disorders., Material and Methods: On the basis of ICD code patients with coincidance of endocrine disorders, epilepsy and psychoneurologic disorders were selected from several thousands of children treated between 2000 and 2009 in Pediatric Endocrinology Department. The neurologic disorders were diagnosed and treated in Chair and Department of Children's and Adolescents Neurology or in another pediatric neurology center., Results: Various forms of epilepsy (symptomatic or idiopathic) and other psychoneurological disorders (disorders of behavior and emotions, obsession-compulsion syndromes, stereotypias, aggression, autoaggression, or hypothalamic obesity) coincident with one or more endocrine disorders, such as growth disorders, disorders of pubertal development, obesity, thyroid diseases, adrenal diseases, hyperprolactinemia, hypoparathyroidism and ion metabolism disorders were diagnosed in 49 patients. The group included: i) children after cranial irradiation and chemotherapy due to medulloblastoma (3 patients), oligodenroglioma (1 patient), ependymoma (1 patient), optic chiasm glioma (2 patients), suprasellar germinal tumor (1 patient), ii) children with Hashimoto encephalopathy (2 patients), iii) children with Prader-Willi syndrome (20 patients), with Klinefelter syndrome (10 patients), with Albright syndrome (9 patients). Of the 49 patients, a group of 6 children representative for individual disorders was selected. In those patients, the etiology of both endocrine disorders, epilepsy and neuropsychiatric disorders was suspected to be common, and the diagnosis was usually delayed., Conclusions: 1. Cranial irradiation and chemotherapy, encephalopathy associated with Hashimoto disease and some of the syndromes with the chromosomal and genetic background are the causes of non-structural CNS abnormalities and coincidence of endocrinopathies, epilepsy and psychoneurologic disorders. 2. MR/CT CNS imaging should be performed in any case of central neurological disorders, disorders of behavior, epilepsy or seizures, but also in patients with delayed psycho-motor development, delayed or accelerated growth and pubertal development. All of the above-mentioned manifestations may be symptoms of structural CNS abnormalities and their early treatment determines the child's future. 3. Excluding structural CNS abnormalities allows for forming suspicions associated with diseases resulting in non-structural disorders of the CNS function, predisposing to coincidence of endocrine and neurological disorders. 4. In the diagnosis of Hashimoto's encephalopathy, a decisive factor is exclusion of structural, infectious, traumatic and metabolic causes, intoxications, epilepsy and presence of neuropsychiatric symptoms in patients with high level of against TPO antibodies. In cases of steroids resistance, a good therapeutic effect may be achieved by plasmapheresis, Rituximab therapy and progestagene inhibition of the menstrual cycle.

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.

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.

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.

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.

Introduction: The Verbal Fluency Test is one of the easiest method in the neuropsychological evaluation of the frontal and temporal lobes' functioning. The amount of reasearch considering children's performance is still small compared to the adult population. The test lacks polish norms (as well as norms for children in other countries, except for unique cases)., Aim of the Study: it was to present possible methods of quality and quantity analysis of the Verbal Fluency Test, and the statistical interpretation of children's performance, depending on the general result, age and diagnosis., Material and Methods: the research was done on a group of 80 children, aged 6-17, including 50 girls and 30 boys, who were hospitalized during the yeras 2007/2008 in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology Jagiellonian University in Krakow. The children were diagnosed with epilepsia (44 children) or headache (36 children). The Verbal Flunecy Test was used in the neuropsychological evaluation among other methods, such as Rey Osterrieth Compex Figure test, Clock test, and intelligence tests WISC-R and WAIS-R(PL)., Results: the results confirm the charakter of the method, as a executive rather than memory function measure. The general result was influenced mainly by the ability to switch between specific subcategories. The general result correlated with age and gender, also children with headache performed better than children with epilepsia., Conclusions: Apropriately interpreted, especially considering quality analysis, the Verbal Fluency test is a valuable tool in the differential diagnosis in children, and detection of subtle weakening in the development of certain cognitive abilities. It is crucial to gather appropriate normative data for the population of children in Poland, which would enable the test's use in more general practice, as one of the early detection methods in the diagnosis of developmental disorders.