Suboptimal multisensory processing in pediatric migraine without aura: a comparative, cross-sectional study.

Alterations of sensory processing in migraine are well known. There is some evidence to suggest that multisensory processing is altered in migraine as well, but the area is underexplored, especially regarding pediatric migraine. A visual and an audiovisual version of the Rutgers Acquired Equivalence Test paradigm was administered to pediatric patients with migraine without aura (aged 7-17.5 years) and to age- and sex-matched controls. The application of audiovisual stimuli significantly facilitated associative pair learning in migraine-free children and adolescents, but not in pediatric migraine patients. The results of this study corroborate the hypothesis that multisensory processing is altered in pediatric migraine without aura.

Visually guided associative learning in pediatric and adult migraine without aura.

INTRODUCTION: The Rutgers Acquired Equivalence Test is a visually guided equivalence learning paradigm that involves rule acquisition and generalization. Earlier we found impaired performance in this paradigm among adult migraine patients without aura. The aim of the study was to investigate if similar impairments can be found already in the pediatric form of the disease and to compare the performance of the pediatric study population with that of an adult study population. We hypothesized that the deficits observed in adults would be observable already in the pediatric population. METHODS: Twenty-seven children and adolescents newly diagnosed with migraine without aura and 27 age- and sex-matched healthy controls were tested with the Rutgers Acquired Equivalence Test. Their performance data were compared to each other and those of an earlier adult study population involving 22 patients and 22 age- and sex-matched healthy controls. Four parameters characterizing performance in the two main phases of the paradigm were calculated for each of the four groups. Performance parameters were compared with Mann-Whitney U test. RESULTS: In contrast to the decreased performance of the adult patients in the Rutgers Acquired Equivalence Test, no significant difference was found between pediatric patients and controls in any phase of the paradigm. CONCLUSION: Children living with migraine without aura do not exhibit the same cognitive deficits in the Rutgers Acquired Equivalence Test as their adult counterparts. It can be hypothesized that the deficit of equivalence learning is not an inherent feature of the migrainous cognitive profile, rather the result of the interference of the disease with normal development.

[Maternal and neonatal vitamin B12 deficiency detected by expanded newborn screening]. / Anyai és újszülöttkori B12-vitamin-hiány felismerése kiterjesztett újszülöttkori szuréssel.

INTRODUCTION: Infant vitamin B12 deficiency can manifest as a severe neurodegenerative disorder and is usually caused by maternal deficiency due to vegetarian diet or pernicious anaemia. Its early recognition and treatment can prevent potentially serious and irreversible neurologic damage. Biochemically, vitamin B12 deficiency leads to an accumulation of methylmalonic acid, homocysteine, and propionylcarnitine. Expanded newborn screening using tandem mass spectrometry may identify neonatal and maternal vitamin B12 deficiency by measurement of propionylcarnitine and other metabolites in the dried blood spot sample of newborns. AIM: To summarize our experiences gained by screening for vitamin B12 deficiency. METHOD: Clinical and laboratory data of vitamin B12-deficient infants diagnosed in Szeged Screening Centre were retrospectively analysed. RESULTS: In Hungary, expanded newborn screening was introduced in 2007. Since then approximately 395 000 newborns were screened in our centre and among them, we identified four newborns with vitamin B12 deficiency based on their screening results. In three cases an elevated propionylcarnitine level and in the fourth one a low methionine level were indicative of vitamin B12 deficiency. We also detected an additional vitamin B12-deficient infant with neurological symptoms at 4 months of age, after a normal newborn screening, because of elevated urinary methylmalonic acid concentration. Vitamin B12 deficiency was secondary to maternal autoimmune pernicious anaemia in all the five infants. As a result of the recognized cases the incidence of infant vitamin B12 deficiency in the East-Hungarian region was 1.26/100 000 births, but the real frequency may be higher. Conslusions: Optimizing the cut off values of current screening parameters and measuring of methylmalonic acid and/or homocysteine in the dried blood spot, as a second tier test, can improve recognition rate of vitamin B12 deficiency. Orv Hetil. 2017; 158(48): 1909-1918.

Age-related prevalence and features of migraine headache in Hungarian schoolchildren and adolescents.

BACKGROUND: Differences occur in certain features of childhood and adult migraine, such as the duration and location. However, few studies have been reported of the changes in other symptoms during childhood. AIMS: The aims of this study were to establish the prevalence of migraine headache in children in Hungary, and to investigate the changes in prevalence of migraine and migraine symptoms in a wide paediatric age range. METHODS: We conducted a school-based study with the use of a questionnaire. RESULTS: 7361 7-18-year-old students participated. The 1-year prevalence of migraine was 12.5% (9.2% in boys and 15.4% in girls). With the criterion of a headache duration of 4 h for 15-18-year-olds and of 1 h below the age of 15, the overall prevalence decreased to 9.1%. The prevalence of migraine increased steadily from young childhood to late adolescence in both boys and girls. The frequency and duration of headache increased, whereas vomiting and nausea became less prevalent with advancing age in both genders. The prevalence of uni/bilaterality, photophobia and phonophobia increased only in girls, while that of a pulsating character did so only in boys. CONCLUSIONS: The migraine characteristics displayed by the studied population proved similar to those experienced in other countries. The duration of headache applied in the diagnosis of migraine exerts a great impact on the prevalence data. The features of migraine change with advancing age, a situation demanding consideration in studies on migraine in children of different ages.

Sturge-Weber syndrome: clinical and radiological correlates in 86 patients.

BACKGROUNDS AND PURPOSE: To correlate the extent of the leptomeningeal angiomatosis with clinical features in Sturge-Weber syndrome (SWS). METHODS: The study group consisted of 86 consecutive patients aged two months to 56 (mean 7.9 +/- 10.3) years with SWS and epilepsy. Clinical and MRI data were analyzed. RESULTS: Based on the extent of leptomeningeal angiomatosis, patients were divided into two subgroups: 43 patients had hemispheric angiomatosis and atrophy, whereas, another 43 had focal involvement. Nine of the 43 hemispherial patients (10%) showed bilateral involvement: all of these bilateral cases demonstrated dominance in a single side with hemispheric leptomeningeal angiomatosis and contralateral focal extension. Hemispheric and focal subgroups were clinically different. Patients with hemispheric SWS were younger at the age of epilepsy onset (p < 0.001) and age at MRI examination (p < 0.05). Neither gender, lateralization, duration of epilepsy, appearance of secondarily generalized seizures, nor seizure frequency revealed a significant difference between subgroups. CONCLUSION: Bilateral involvement is frequent and occurs in cases with a hemisperic involvement on one side. The age of epilepsy onset is related to the extent of leptomeningeal angiomatosis. Patients with hemispheric form of SWS presented with earlier age of seizure onset. Focal pial angiomatoses do not tend to progress (a longer duration is not associated with more frequent hemispheric involvement). Other variables including seizure frequency and secondary generalized tonic-clonic seizures are not associated with the extent of angiomatosis.

Delayed development of visual motion processing in childhood migraine.

INTRODUCTION: Altered visual processing has been observed in adult migraineurs. But because visual processing has not been studied in paediatric cases, it is not known whether such visual system alterations are already present in early development. We therefore used a dynamic visual task to investigate motion detection threshold in paediatric migraine. METHODS: Fourteen migraineurs and 21 controls participated in the study (age range: 8-17 years). The minimal percentage of coherently moving dot stimuli at which subjects were still able to detect coherent movement (absolute threshold) was determined using a random dot kinematogram paradigm. RESULTS: Motion coherence detection threshold was higher in migraineurs (p < 0.05). This difference between groups was more pronounced at younger ages, but migraineurs seem to catch up with healthy controls over the years. CONCLUSIONS: Children with migraine exhibit a delayed development of visual motion processing. This might be a useful supplementary biomarker in paediatric migraine.

Development of visual contour integration in children with migraine without aura.

INTRODUCTION: As migraine attacks pose insult to cerebral circulation and ion homeostasis, migraine has the potential to interfere with the development of different brain structures, producing functional deficits. It is known that visual contour integration (CI) is a function with a protracted development. Therefore, we sought to establish whether migraine interferes with its development. METHODS: Forty-eight migraineurs (without aura) and 48 age- and sex-matched controls participated in the study, divided into three cohorts by age. Stimuli were presented on cards with a contour consisting of Gabor patches embedded in random noise. Difficulty was varied by the manipulation of relative noise density. The task was to identify and show the contour. RESULTS: A significant difference was found between the performance of migraineurs and controls in the 10-14-year-old and 15-18-year-old cohorts (p < 0.05). Development between all three cohorts was significant in the control group (p < 0.017), while it was not significant in migraineurs between 6 and 14 years. Correlation between age and CI threshold was stronger in controls than in migraineurs. CONCLUSION: Children with paediatric migraine exhibited a less marked development in the Gabor patch-based CI task.

Corpus callosum anomalies: birth prevalence and clinical spectrum in Hungary.

Data regarding the epidemiology of callosal anomalies are contradictory. We performed a population-based retrospective survey to study the birth prevalence and clinical features of agenesis/hypoplasia of the corpus callosum and accompanying central nervous system and somatic abnormalities in southeastern Hungary between July 1, 1992 and June 30, 2006. Among 185,486 live births, 38 patients (26 boys and 12 girls) manifested agenesis/hypoplasia of the corpus callosum, corresponding to a prevalence of 2.05 per 10,000 live births (95% confidence interval, 1.4-2.7). Callosal anomalies were isolated in 18 patients, and were associated with other central nervous system malformations in five children. Both central nervous system and noncentral nervous system abnormalities were evident in seven patients, whereas callosal dysgenesis was accompanied only by somatic anomalies in eight children. Five of 18 patients with isolated agenesis/hypoplasia of the corpus callosum remained asymptomatic. Developmental delay, intellectual disability, or epilepsy occurred in all patients, except one, when callosal anomalies were combined with other brain or somatic abnormalities. Five patients with multiplex malformations died. Callosal anomalies form a clinically significant and relatively frequent group of central nervous system malformations.

Holoprosencephaly in hungary: birth prevalence and clinical spectrum.

A retrospective population based survey of patients born with holoprosencephaly in South-Eastern Hungary between July 1, 1992 and June 30, 2006 was performed. All live birth cases with craniofacial and non-craniofacial abnormalities were included in the study. A total of 9 patients (5 boys and 4 girls) were found with holoprosencephaly among 185 486 live births, which correspond to a birth prevalence of 0.49 per 10,000 live births (95% confidence interval [CI]: 0.17-0.80). These figures were similar to those ones found in New York State and several European regions. In our series one newborn had trisomy 13. Eight patients did not have chromosomal abnormalities on routine testing, 4 of them had craniofacial abnormalities only and another 4 showed non-craniofacial anomalies as well. Three patients died in the neonatal period and another one in childhood. Patients surviving the neonatal period had intellectual and motor handicap, and epilepsy.

Is the development of visual contrast sensitivity impaired in children with migraine? An exploratory study.

INTRODUCTION: Impairment of visual contrast sensitivity is a well-known phenomenon in adult migraineurs. Little is known, however, about whether contrast sensitivity deficits are already present in children with migraine. METHODS: We conducted an exploratory study with 18 children with migraine without aura, in which we tested our subjects' visual contrast sensitivity. Eighteen age- and sex-matched healthy children served as controls. RESULTS: Among the youngest subjects (6-10 years) we found no significant differences at any of the spatial frequencies tested, as compared to the controls, whereas from the age of 10 on, migraineurs exhibited significantly poorer contrast sensitivity, especially at the lower spatial frequencies. CONCLUSION: To our knowledge, we are the first to report on such a deficit in children, and we conclude that our findings might be interpreted as reflecting an increased vulnerability of the visual system to migraine attacks as part of the migrainous endophenotype.

Epidemiology and clinical spectrum of schizencephaly in south-eastern Hungary.

The epidemiology and clinical spectrum of schizencephaly in south-eastern Hungary have been surveyed in a retrospective population-based study. A total of 10 patients (6 boys and 4 girls) were found with schizencephaly among 185 486 live births in a period of 14 years (July 1, 1992 to June 30, 2006), which means a birth prevalence of 0.54 per 10 000 (95% confidence interval [CI]: 0.20-0.87). The schizencephaly was unilateral in 7 cases (with closed lips in 5 and open lips in 2 patients) and bilateral in 3 children (with closed lips in 2 and open lips in 1). The septum pellucidum was absent in 5 cases; however, optic nerve hypoplasia was not found in these patients. Delayed development and intellectual disability were observed in all patients, except 2 with unilateral closed lip schizencephaly. Epilepsy was diagnosed in 3 patients (2 with unilateral and 1 with bilateral schizencephaly).

Primary microcephaly in Hungary: epidemiology and clinical features.

AIM: To describe the population-based epidemiological characteristics and clinical features of primary microcephaly in Hungary. METHODS: A retrospective survey of patients born with microcephaly in a region (Dél-Alföld - South Great Plain) in Hungary between July 1, 1992 and June 30, 2006 was performed. Patients with microcephaly and without any environmental or obstetric risk factors and/or dysmorphism (primary microcephaly) were included in the study. The birth prevalence of primary microcephaly per 10,000 live births was calculated. RESULTS: Ten patients (8 girls and 2 boys) were found with primary microcephaly among 185,486 live births, which corresponds to a birth prevalence of 0.54 per 10,000 live births (95% confidence interval: 0.20-0.87). Developmental delay and intellectual disability were the main clinical features. Dyskinesia was seen in one and epilepsy was diagnosed in two patients. The MRI revealed simplified gyral pattern in all patients. CONCLUSION: Primary microcephaly is a very rare brain malformation, although the birth prevalence found in this survey is slightly higher than the few figures published earlier. As more and more genes and mutations responsible for primary microcephaly are discovered, the ascertainment of these rare cases is mandatory to provide the parents with genetic counselling.

99-mTc-HMPAO single photon emission computed tomography examinations in genetically determined neurometabolic disorders.

UNLABELLED: The aim of our study was to determine regional cerebral blood flow (rCBF) abnormalities in different types of enzymopathies. PATIENTS AND METHODS: Among the patients with genetically determined enzymopathies 3 patients had aminoacidopathies, and 11 had different types of encephalopathies, from which 10 had mitochondrial encephalomyopathy (MEMP), and 1 patient had hyperuricaemic encephalopathy. Besides the mentioned 14 patients, 1 had ceroid lipofuscinosis and another patient had tuberous sclerosis. The further distribution of the MEMP patients' group was the following--5 patients had MEMP with lactic acidosis, 5 had Leigh's disease (subacute necrotizing encephalopathy), from which 1 had cytochrome-c-oxidase deficiency (COX). Additionally in all patients were performed cerebral MRI and SPECT examination 10 min. after intravenous administration of 20 Mbq/kg 99 mTc-HMPAO. RESULTS: Fourteen out of 16 SPECT findings were pathologic, showing decreased focal frontal/temporal/temporoparietal cerebral blood perfusion. Aminoacidopathic group--all the 3 patients revealed pathologic signs from the aminoacidopathic patients' group. Among them the ornithine transcarbamylase (OTC) heterozygous female patient with left-sided hemiparesis caused by hyperammonemic stroke at 10 month-age, showed right sided temporoparietal, occipital and left frontal hypoperfusion, nearly 6 years after the cerebral vascular attack. This finding might be resulted because of diaschisis. Mitochondrial encephalo-myopathic (MEMP) group--all the four patients with MEMP and lactic acidosis showed focal hypoperfusion in the temporal region, while the perfusion was normal in the COX deficient patient and in 2 Leigh's disease (subacute necrotizing encephalopathy) patients. In the remaining 1 Leigh's patient frontotemporal hypoperfusion was found. In all patients there were non specific structural abnormalities detected by MRI: cortical and subcortical atrophy, and scattered demyelination foci. In the case of ceroid lipofuscinosis the MRI showed cerebral atrophy and cerebellar hypoplasia, and the SPECT showed right frontal and occipital hypoperfusion, bilateral parietal physiological riping process. The patient with tuberous sclerosis showed bilateral temporo-occipital hypoperfusion. CONCLUSION: (1) SPECT images demonstrated hypoperfusion rCBF changes in 14 out of all 16 patients. (2) Regional cerebral/cerebellar hypoperfusion was detected by SPECT in mitochondrial encephalomyopathies, with lactate acidosis and aminoacidopathies giving high informative value about the cerebral perfusion.

Cerebellar hypoperfusion in infantile neuroaxonal dystrophy.

An identical abnormal pattern was detected by means of (99m)Tc-hexamethyl-propyleneamine-oxime single-photon emission computed tomography in two siblings with infantile neuroaxonal dystrophy. The markedly decreased cerebellar perfusion, along with the early motor symptoms, characteristic magnetic resonance imaging and pathologic findings, points to a preferential cerebellar involvement in this disease. A relative increase in the perfusion to the basal ganglia correlated with the magnetic resonance imaging abnormalities, highly resembling that of Hallervorden-Spatz disease in one of the males, at this site.