Safety, tolerability, and efficacy of a widely available nusinersen program for Polish children with Spinal Muscular Atrophy.

Spinal muscular atrophy (SMA) is a devastating neuromuscular disorder with limited treatment options. Nusinersen is the first disease-modifying therapy to treat children and adults with SMA. This study aimed to review the safety, tolerability, and efficacy data of a nusinersen treatment program in Polish children. A total of 298 patients aged from 0 to 18 years were included in the nusinersen treatment program in Poland between March 1 and September 20, 2019. All patients were prospectively followed for at least one year. The mean age at treatment onset was 6.9 years. SMA type 1 symptoms were reported in 127 patients (43.5%), SMA type 2 symptoms in 68 cases (23.3%), and SMA type 3 in 93 patients (31.8%). No patient met the inefficiency criteria defined in the program. One year after treatment initiation, all patients assessed by the CHOP-INTEND scale had improved or remained stable. The mean change in CHOP-INTEND score was an increase of 8.9 points between baseline and after one-year treatment (p < 0.001). Except for 2 fatal cases, not related to the treatment, no serious adverse events were reported. The results of our study indicate that treatment with nusinersen is beneficial for children with SMA regardless of their age, baseline functional status, or the number of SMN2 gene copies. Therapy with nusinersen was effective and well tolerated by patients.

Clinical utility of complex assessment with evoked potentials in acute lymphoblastic leukemia survivors: comparison of various treatment protocols.

BACKGROUND: One of the greatest success of pediatric hematology is a prominent improvement of survival in acute lymphoblastic leukemia (ALL). Therefore, special attention needs to be paid to long-term side effects of the treatment such as neurotoxicity. One of the few diagnostic methods that allow an objective assessment of sensory systems are evoked potentials (EP). METHODS: The analyzed group consisted of 167 ALL long-term survivors, aged 4.9-28.4 years, without auditory, visual and sensory deviations. Patients were treated with New York (NY, n = 35), previous modified Berlin-Frankfurt-Münster (pBFM, n = 47) and BFM95 (n = 85) protocols. In order to assess the impact of radiotherapy on recorded EP, a joint analysis of NY and pBFM groups was performed. The control group consisted of 35 patients, aged 6-17 years. The analyzed patients underwent a complex assessment with visual EP (VEP), somatosensory EP (SEP) and brainstem auditory EP (BAEP) in accordance with current standards. RESULTS: ALL treatment contributed to the shortening of wave I latency (1.59 vs 1.90, P = 0.003) and prolongation of I-III (2.23 vs 2.04, P = 0.004) and I-V (4.57 vs 4.24, P = 0.002) interwave latencies of BAEP. A significant effect was also noticed in P100 (106.32 vs 101.57, P < 0.001) and N135 (151.42 vs 138.22, P < 0.001) latencies of VEP and N18 amplitude (3.24 vs 4.70, P = 0.007) and P25 latency (21.32 vs 23.39, P < 0.001) of SEP. The distribution of abnormalities between protocols was similar in BAEP (NY - 68.6%, pBFM - 61.7%, BFM95-69.4%, P = 0.650), VEP (NY - 68.6%, pBFM - 42.5%, BFM95-58.3%, P = 0.053) and significantly different for SEP (NY - 62.9%, pBFM - 36.2%, BFM95-53.0%, P = 0.045). The harmful effect of radiotherapy was most clearly marked in numerous disturbances of SEP parameters. CONCLUSIONS: The presented analysis indicates a high frequency of subclinical abnormalities in EP regardless of the analyzed protocol. To our knowledge current study is the largest and one of the most complex research examining the role of EP in ALL patients. The obtained results indicate the possibility of using a single, objective and non-invasive measurement of EP in ALL survivors in order to stratify the risk of developing sensory abnormalities in adulthood.

Polyneuropathy in Acute Lymphoblastic Leukemia Long-Term Survivors: Clinical and Electrophysiological Characteristics With the Impact of Radiotherapy.

Introduction: Acute lymphoblastic leukemia (ALL) is the most common childhood cancer with one of the highest survival rates. Long-term complications that occur after intensive oncological treatment often impair normal daily functioning. However, existing data on peripheral nervous system condition in ALL survivors remain conflicting. Materials and Methods: The study group consisted of 215 ALL survivors. Patients were treated with New York (NY, n = 45), previous modified Berlin-Frankfurt-Münster (pBFM, n = 64), and BFM95 (n = 106) protocols. Time elapsed between the end of the treatment and the control examination varied from 0.3 to 20.9 years. The analyzed patients underwent a neurophysiological analysis with electroneurography (ENG) of motor (median and peroneal) and sensory (median and sural) nerves as well as electromyography (EMG) of tibialis anterior, vastus lateralis, and interosseous I muscles. To estimate the influence of radiotherapy on recorded neurophysiological responses, a joint analysis of NY, and pBFM groups was performed. Results: Clinical symptoms of polyneuropathy were noted among 102 (47.4%) children during the ALL therapy and in 111 (51.6%) during follow-up. At the time of treatment, polyneuropathy was diagnosed in 57.8% participants from NY group, 35.9%-pBFM and 50.0%-BFM95 (p = 0.145). A significantly higher incidence of polyneuropathy was observed during a follow-up in the NY group (68.9%; p < 0.001 vs. pBFM, p = 0.002 vs. BFM95). The most frequent abnormality within all the protocols was demyelination (NY: 44.4%, pBFM: 59.4%, BFM95: 41.5%), in contrast to the least frequently registered isolated axonal changes. The negative influence of oncological treatment on neurophysiological parameters in ALL survivors was observed. Complex disorders of motor nerves, sensory nerves, and motor unit potentials were registered. Motor-sensory neuropathy was the most frequently found pathology in all analyzed protocols. The harmful effect of radiotherapy was also observed in EMG results. Conclusions: Detailed neurophysiological analysis in long-term childhood ALL survivors has shown generalized abnormalities in registered parameters. To our knowledge, the current study is the largest and one of the most comprehensive ones among those examining disturbances in ENG and EMG in this group of patients. Moreover, we are the first ones to demonstrate the negative influence of radiotherapy on peripheral nerve conduction parameters.

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.

[The links between neuropsychology and neurophysiology]. / Zwiazki miedzy neuropsychologia a neurofizjologia.

AIM: The aim of the study was to establish current scope of knowledge regarding associations between neurophysiological functioning, neuropsychology and psychoterapy. MATERIAL AND METHODS: A systematic review was performed including 93 publications from Science Server, which contains the collections of Elsevier, Springer Journals, SCI-Ex/ICM, MEDLINE/PubMed, and SCOPUS. The works have been selected basing on following key words: 'neuropsychology, neurocognitive correlates, electrodermal response, event related potential, EEG, pupillography, electromiography' out of papers published between 2004-2015. RESULTS: Present reports on the use of neurophysiological methods in psychology can be divided into two areas: experimental research and research of the practical use of conditioning techniques and biofeedback in the treatment of somatic disease. Among the experimental research the following have been distinguished: research based on the startle reflex, physiological reaction to novelty, stress, type/amount of cognitive load and physiological correlates of emotion; research on the neurophysiological correlates of mental disorders, mostly mood and anxiety disorders, and neurocognitive correlates: of memory, attention, learning and intelligence. Among papers regarding the use of neurophysiological methods in psychology two types are the most frequent: on the mechanisms of biofeedback, related mainly to neuro- feedback, which is a quickly expanding method of various attention and mental disorders'treatment, and also research of the use of conditioning techniques in the treatment of mental disorders, especially depression and anxiety. A special place among all the above is taken by the research on electrophysiological correlates of psychotherapy, aiming to differentiate between the efficacy of various psychotherapeutic schools (the largest amount of publications regard the efficacy of cognitive-behavioral psychotherapy) in patients of different age groups and different diagnosis.

[May neuroimaging findings of a child with multiple sclerosis surprise us? Clinical and radiological observation]. / Czy wynik neuroobrazowania u dziecka ze stwardnieniem rozsianym moze nas zaskoczyc? Spostrzezenie kliniczno-radiologiczne.

BACKGROUND: Multiple sclerosis (MS) is a chronic disorder characterized by presence of demyelinating changes in the central nervous system. The disease most often affects young adults with a female predominance, but the first symptoms can also occur in children. MS symptoms in childhood are characterized by a higher incidence of sensory, cerebellar and brainstem disorders compared to adults. They are frequently multifocal especially in the youngest age groups, requiring differentiation of acute disseminated encephalomyelitis. The diagnosis of MS is based on the 2010 McDonald criteria in both children and adults. Dissemination of disease in space and time documented by either clinical and/or radiological findings is necessary for diagnosis establishment. Additional tests used in MS diagnosis are examination of cerebrospinal fluid and examination of exogenous evoked potentials. CASE REPORT: We describe a case of 11-year-old boy who came to the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology Jagiellonian University in Krakow because of severe headache and dizziness, which occurred the day before admission. Neurological examination revealed the presence of balance disorders and signs of the pyramidal tract involvement. Head MRI revealed disseminated demyelinating lesions in typical for MS localization with the presence of one active, gadolinium-enhancing lesion. Examination of cerebrospinal fluid showed oligo-clonal bands. Results of exogenous evoked potentials were normal. The patient received high-dose intravenous (pulse) methylprednisolone therapy with good clinical outcome. At that time the patient did not meet requirements for interferon therapy financed from the state budget because of too young age. CONCLUSIONS: Pediatric-onset MS has a slightly different clinical presentation compared to adult-onset MS. The neuroimaging findings may be sometimes surprising showing a large discrepancy between advanced radiological changes and clinical outcome.

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.

Epilepsy and electrophysiological findings in polish twins with glycogenosis type IIIb.

Glycogen storage diseases are rare genetic disorders, mostly autosomal recessively inherited. Abnormal accumulation is because of the lack of one of the enzymes involved in glycogen metabolism. Neurological manifestation of the diseases involves muscle weakness and hypoglycemia-induced seizures. In this article, we present a history of twin sisters with unusual coincidence of glycogenosis type IIIb and epilepsy. Hypoglycemic background of seizures and organic changes of the central nervous system were excluded. Since the introduction of antiepileptic treatment, the patients have been seizure-free; however, paroxysmal electroencephalographic (EEG) changes have persisted. A high-protein and low-carbohydrate diet has protected them against hypoglycemia.

Neurofeedback therapy in patients with acute and chronic pain syndromes--literature review and own experience.

Pain management is based mainly on pharmacotherapy which has many limitations. Non-pharmacological techniques, like neurofeedback (EEG-biofeedback) are alternative methods of pain treatment. Data from literature confirm high efficacy of neurofeedback in pain syndromes treatment, chronic and acute as well. Neurofeedback plays an important role in management of post stroke, post traumatic headaches and in primary headaches like tension type headaches or migraine. Literature review and own experience indicate importance of number and frequency of performed neurofeedback trainings on treatment effectiveness. Satisfactory results have already been observed after 30 trainings however usually 40-60 training have to be performed. Effectiveness of such therapy in pain syndromes is usually good or less often acceptable (50% reduction of headaches). Children with tension type headaches (differently than adults) need reminder therapy every 6-12 months, otherwise recurrence of headaches is observed. Based on our own experience neurofeedback therapy seems to play role in neuropathic pain and cancer pain management.

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.

[Influence of visual stimulation on cerebral blood flow and visual evoked potentials in children with migraine with visual aura]. / Wplyw bodzca wzrokowego na przeplyw mózgowy i wzrokowe potencjaly wywolane u dzieci z migrena z aura wzrokowa.

BACKGROUND: Visual aura is the most common type of migrainous aura. It may occur as a single symptom or precede sensory or speech disturbances. Aura symptoms and order of their appearance may result from propagation of spreading depression phenomenon. Vascular disorders observed during migraine with aura attacks are probably secondary to neuronal changes. Simultaneous registration of cerebral bioelectric activation and changes evoked in cerebral circulation enables their objective estimation, detection of correlation and better understanding of migraine with aura pathogenesis. Studies with transcranial Doppler revealed impaired cerebrovascular response to various stimulations in migraine, especially migraine with aura patients. Combination of Doppler examination with registration of visual evoked potentials (VEP) enables estimation on neurovascular coupling. AIM OF THE STUDY: Estimation of cerebrovascular response to visual stimulation in migraineurs with visual aura. Examination of correlation between cerebral blood flow and VEP parameters. MATERIAL AND METHODS: 50 children at the age range 8-18 years, 8 with migraine with visual aura, 9 with visual and sensory aura, 8 with visual, sensory and dysphasic aura and 25 children from control group. Examination of cerebral blood flow parameters was performed with transcranial Doppler, with the use of continuous monitoring, enabling performance of simultaneous record. ing from vessels of both hemispheres. Systolic (SV), end-diastolic (EDV), mean (MV) velocities and pulsatility (PI) and resistive indexes (RI) were analyzed. Parameters of cerebral blood flow were recorded in middle cerebral arteries, at rest (1 measurement), during visual stimulation (2, 3 measurement) and directly after the end of the stimulation (4 measurement). Black and white checkerboard pattern rebersal was visual stimulator and during stimulation VEP were registered. N75, P100, N135 latencies and N75/P100, P100/N135 amplitudes were analyzed. Moreover correlation between visually evoked changes in cerebral blood flow (2-1 measurement) and VEP values was examined. RESULTS: Statistically significant differences in EDV and MV were found between controls and migraineurs with aura. In response to visual stimulation significant differences in cerebral blood flow velocities (SV, EDV, MV) were found and most often were observed direct after the beginning of the stimulation, however they did not differ significantly between the groups. Interactions between group and time was also not found in the following analyzed groups: migraine with visual, sensory and visual, sensory, dysphasic aura vs control, migraine with visual aura vs visual and sensory aura vs visual, sensory and dysphasic aura. Significant differences in P100 latencies and N75/P100 amplitudes were found between control and migraine with more than one aura symptom but no differences were found within migraine with aura subtypes. Moreover, in migraine with aura patients positive correlation between visually evoked changes in cerebral blood flow velocities and VEP amplitudes was detected. CONCLUSIONS: Visual stimulation has significant influence on cerebral blood flow velocities in both migraine with aura patients and controls and these changes are most often observed direct after the beginning of the stimulation. However, no differences in vascular response to visual stimulation between the groups were observed. On the other hand positive correlation between visually evoked changes in cerebral blood flow velocities and VEP amplitudes was found in migraine with aura patients suggesting increased neurovascular coupling in this group when compared with controls.

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

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

[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.

[The influence of child's behaviour on the course of neurological diagnosis]. / Wplyw zachowania dziecka na przebieg diagnozy neurologicznej.

INTRODUCTION: Among the huge amount of neurological diseases that cause diagnostic difficulties, there happens, from time to time an additional difficulty--disorders of psychogenic origin, and among those- conversion disorders. Until now there still is no satisfactionary explanation for the neurobiological basis of conversion. The explanations offered by psychology are also rather general, based on the tradition of psychoanalysis. Aside from how deeply one understands its mechanisms, when suspecting a psychogenic origin of the symptoms observed, one should be especially careful. It is far too easy falling in the trap, that hysteria sets for us, suggesting to be the patient's one and only disease that should be dealt with. AIM OF THE STUDY: To show the difficulties and dangers when making a diagnosis of functional disorders, both from the physician's and psychologist's point of view. MATERIAL AND METHODS: Presenting cases of 6 patients, whose behaviour and symptom presentation suggested a psychogenic background of the disorders, and only few of the psychological tests' results differentiated between the organic and the psychogenic group. RESULTS: In the presented group there were 3 children diagnosed with conversion disorder, and 3 diagnosed with neurological diseases. The psychological test results only partly differentiated between both groups, not allowing any interpretation to be based on quantitative results only. CONCLUSIONS: 1. Even though medicine has made such a huge step forward, conversion disorders can still cause serious diagnostic difficulties. 2. Leaving our knowledge about human mind aside, we are continously eager to base our judgement on the most vivid elements, that present to us most clearly. 3. That line of thinking puts us in jeopardy of missing the obvious: that a histerical person can also suffer from another condition of a very organic origin.

[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.

[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.

[Causes and diagnostics of dizziness of central origin in children]. / Przyczyny i diagnostyka zawrotów glowy pochodzenia osrodkowego u dzieci.

Dizziness and vertigo are symptoms caused by several etiological factors, they are result of general diseases, within anemia. They are associated with several diseases of the organ of hearing and balance, which are under laryngological care, and one of most important tests to distinguish vertigo of central and peripheral origin is videonystagmography. Several diseases of the central nervous system cause instability of posture and gait. Psychogenic feeling of being insecure and unsafe makes the diagnostics more complicated. Vertigo of central origin in children can be a first symptom of severe conditions, such as tumor of CNS, therefore the neuroradiological evaluation is necessary for early diagnosis, before the occurrence of increase of intracranial pressure symptoms. Vascular diseases, such as vertebro-basilar insufficiency or stroke are diagnosed more common in adults, still they are not excluded in children, and therefore vascular evaluation is necessary. The cerebrovascular ultrasound is a method significant in the diagnostics as well as in the monitoring of therapy of cerebral vascular diseases. The elecrophysiological evaluation, EEG or videoEEG is useful in the evaluation of paroxysmal conditions. Evoked potentials are helpful in diagnostics of demyelination of CNC. The serological tests (within to detect boreliosis) are infrequently performed; they may lead to correct diagnosis and causal treatment. The paper presents examples of diagnostic methods valid in children with dizziness of central origin.