Alternating hemiplegia of childhood: New diagnostic options

A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides of the body. Age of onset is before 18 months and the common earliest manifestations are dystonic or tonic attacks and nystagmus. Hemiplegic episodes last minutes to days and the frequency and duration tend to decrease with time. Motor and intellectual development is affected, deficits may also develop later. Epileptic seizures occur in some patients. Neuroimaging of the brain usually reveals no abnormalities. The variability of individual clinical presentations and evolution of symptoms have made diagnosis difficult. Therefore the problems of misdiagnosis could account for the low prevalence of this syndrome. This paper hopes to present actual data on AHC, especially of the results of genetic research and new diagnostic tools

University children's neurophysiology in Krakow

W 50-letniej historii neurologii dziecięcej w Krakowie istotny udział ma również neurofizjologia. Autor przypomniał jej rozwój i ludzi, dzięki którym dokonywał się postęp w zakresie technik EEG, EMG, potencjałów wywołanych, biofeedbacku i badań układu autonomicznego. Wszystkie te dziedziny neurofizjologii są reprezentowane w działalności Uniwersytetu Jagiellońskiego. Autor przedstawił składowe nowoczesnej neurofizjologii dziecięcej oraz jej zastosowania w działalności naukowej i dydaktycznej. Wskazał na udział neurofizjologii uniwersyteckiej w realizacji finansowanych projektów badawczych.In a 50-year history of pediatric neurology development in Krakow, neurophysiology has a significant share.The author recalls her development and the people thanks to whom the progress has been achieved in the techniques of EEG, EMG, evoked potentials, autonomic research and biofeedback. All these areas of neurophysiology are represented in the activities of the Jagiellonian University. The author presents the components of modern neurophysiology of children and their use in research and teaching. He pointed to the participation of neurophysiology in funded research projects

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

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

Trudnoœci diagnostyczne objawów napadowych u chłopca z zespołem Parry’ego-Romberga

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

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

Trudności diagnostyczne objawów napadowych u chłopca z zespołem Parry'ego-Romberga

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.Zespół Parry'ego-Romberga charakteryzuje się postępującym zanikiem połowy twarzy, obejmującym tkankę podskórną, chrząstkę oraz struktury kostne. Z zespołem tym często związane są bóle głowy oraz padaczka, jednakże leżąca u ich podstawy patofizjologia nadal nie jest znana. W niniejszej pracy przedstawiono przypadek dotyczący 12-letniego chłopca z zespołem Parry'ego-Romberga i jamistością rdzenia, u którego występowały napady bardzo silnych bólów głowy oraz napady padaczkowe. Napadom bólów głowy towarzyszyły obustronne objawy autonomiczne oraz hiperwentylacja, po których zazwyczaj występowały napady częściowe złożone, a czasami wtórnie uogólnione napady toniczne. Przeprowadzono szczegółową diagnostykę obrazową [badanie za pomocą rezonansu magnetycznego (RM) głowy, oczodołów i rdzenia kręgowego, angiografię RM głowy i spektroskopię RM móżdżku]. EEG wykazał zmiany zlokalizowane po stronie przeciwnej do zajętej procesem chorobowym. Wdrożono leczenie przeciwpadaczkowe karbamazepiną, którego początkowy efekt był niewielki. Zamiana leku na okskarbazepinę okazała się skuteczna. U opisanego pacjenta zaobserwowano interesujący związek napadów bólów głowy, objawów autonomicznych i napadów padaczkowych. Jest to prawdopodobnie pierwszy opis zespołu Parry'ego-Romberga z towarzyszącą jamistością rdzenia

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 1.5 T 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

Influence of the cognitive-behaviorak therapy on P300 potential in children with psychogenic nonepileptic events and tension headache : preliminary results

Podstawy: W leczeniu napadów rzekomopadaczkowych i napięciowych bólów głowy u dzieci stosowana jest psychoterapia. Umysłowe funkcjonowanie dzieci wiąże się z endogennymi parametrami neurofizjologicznymi. Cel: Celem tej pracy było ustalenie, czy endogenny potencjał poznawczy P300 u dzieci z napadami rzekomopadaczkowymi i napięciowymi bólami głowy jest różny i czy zmienia się on pod wpływem terapii poznawczo-behawioralnej. Materiał i metody: Badaniami objęto dotychczas grupę 12 dzieci z napadami rzeko­ mopadaczkowymi i 8 z napięciowymi bólami głowy. U wszystkich zastosowano 8-1 O sesji psychoterapeutycznych. Potencjał P300 zarejestrowano przed przeprowadzeniem terapii i po niej oraz dodatkowo w obu cyklach po 3-minutowej hiperwentylacji. Oce­niono czas reakcji, liczbę błędów i odsetkowe skupienie uwagi u dzieci. Wyniki: Średnie wartości wszystkich wyników potencjału P300 były lepsze w gru­ pie dzieci z napięciowymi bólami głowy niż u dzieci z napadami rzekomopadaczko­ wymi. Im krótszy był czas reakcji w pierwszym badaniu, tym wyższy był wskaźnik uwagi i krótszy czas reakcji w kolejnym badaniu w grupie z napadami w odróżnieniu od dzieci z napięciowymi bólami głowy. W obu badanych grupach zastosowanie hiper­ wentylacji wiązało się z wyraźnym wydłużeniem czasu reakcji w badaniu P300, przy niezmieniających się pozostałych parametrach (liczby błędów i procent uwagi). Wnioski: Wstępne wyniki badań zdają się wskazywać na różnicę parametrów potencjału P300, chociaż nieznamienną statystycznie, u dzieci z napadami rzekomo­ padaczkowymi i napięciowymi bólami głowyBackground: Psychotherapy is being used in the treatment o f nonepileptic psychogenic seizures and tension headaches in children. Intelectual functioning of children 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 headache, and whether it changes under the influence o f the cognitive-behavioral psychotherapy. Matherial and methods: The study included so far a group o f 12 children with nonepileptic psychogenic seizures and 8 children with tension headache. The P300 potential was registered before and after the therapy and additionally in both cycles after a 3 minutes hyperventilation. The reaction time, the amount o f mistakes and the\ud percentage o f attention focus was measuerd in all children. Results: Medium scores o f all P300 measures were better in the group with tension headaches than in children w ith psychogenic nonepileptic events. The shorter was the reaction time in the first examination, the higher the attention score and the shorter the reaction time in the second examination in the nonpeileptic seizure group, but not in the tension headache group. In both tested groups hyperventilation related to a substantial prolongation o f the P300 reaction time, with other m easures remaining the same (mistake count and % attention). Conclusions: The preliminary results seem to show a difference in the P300 paramteres, although not one that is statistically significant, in children with nonepileptic seizures and tension headaches. It also applies to the influence o f cognitive-beahioral psychotherapy and hyperventilation