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Background: This study aimed to compare the P3 component between patients who have migraines with aura and healthy subjects, and to compare different subtypes of migraine with aura relative to the complexity of migraine aura. Methods: Average Migraine Aura Complexity Score was calculated for each MwA patient. Visual oddball paradigm was used to elicit the P3 component. P3 amplitudes and latencies elicited from frequent and rare stimuli, as well as from difference wave, were compared with healthy subjects. Subsequently, subtypes of migraine with aura were compared and Average Migraine Aura Complexity Score was used to explore the connection between features of the P3 and complexity of migraine with aura. Results: 37 patients who have migraine with aura (16 with simple aura and 21 with complex aura) patients and 28 healthy subjects were studied. Patients who have migraine with aura had significantly prolonged latencies compared to healthy subjects (411 ± 39 ms vs 372 ± 34 ms, p < 0.001) relative to a rare condition. Patients who have complex aura significantly differs from patients who have simple aura (427 ± 34 ms vs 389 ± 35 ms, p = 0.004) and healthy subjects (372 ± 34 ms, p < 0.001) relative to P3 latency in a rare condition and the patients who have complex aura significantly differs from healthy subjects (442 ± 37 ms vs 394 ± 33 ms, p < 0.001) relative to P3 latency in difference wave. P3 latency from rare condition positively correlated with the Average Migraine Aura Complexity Score (p < 0.001). Conclusions: Visual oddball paradigm, particularly rare stimuli, could serve as a potential new tool for deep profiling of different clinical complexities among patients who have migraine with aura. Also, the present pattern of P3 components provided new evidence for the cognitive dysfunctions in patients who have migraine with aura. [ABSTRACT FROM AUTHOR]

Introduction: In 1962, Renpenning et al. published an article with 20 male patients from three generations with mental retardation. Scientists suggested that the syndrome with mutation mapped to the locus Xp11.2-p11.4 should be called Renpenning syndrome. The deletion/duplication of an AG dinucleotide on proximal Xp in the polyglutamine tract-binding protein 1 (PQBP1) gene causing frameshift in the fourth coding exon was identified as the most frequent mutation in this syndrome. Renpenning syndrome with asymmetric cerebellar hemispheres has not been reported previously. Case Presentation: In this case report, we presented an 11-year-old male with mild developmental delay and mild intellectual disability, microcephaly, dysmorphic face, short stature, and seizures. The following morphological abnormalities were detected: a wide nasal bridge, midfacial hypoplasia, short philtrum, low-set ears, low hanging columella, high palate, and narrow face. Neurological examination showed upper and lower extremities hypotonia with joint hypermobility. The patient had his first seizure at the age of seven, and he experienced a total of 10 seizures by the age 11. A systolic murmur of intensity 2/6 was present, and echocardiography showed chordae tendineae abnormalities in the left ventricle. Brain magnetic resonance imaging (MRI) showed asymmetric cerebellar hemispheres (mild right cerebellar hemisphere hypoplasia). A frameshift mutation in the polar reach domain (PRD) of the PQBP1 gene (c.459-462 delAGAG) was detected by exome sequencing. Conclusions: We showed the first case of genetically confirmed Renpenning syndrome in Serbia. Our patient had classical clinical manifestations for Renpenning syndrome as a consequence of frameshift mutation in the PRD of the PQBP1 gene. To the best of our knowledge, according to the literature, this is the first patient with Renpenning syndrome with asymmetric cerebellar hemispheres (mild right cerebellar hemisphere hypoplasia). [ABSTRACT FROM AUTHOR]

Background: Manifestations of typical migraine aura can be numerous. Investigation of its pathophysiological mechanisms can be challenging if a stratification of phenotypes is not performed. In this context, the Migraine Aura Complexity Score (MACS), recently developed, may help. Here we aimed to categorize migraine patients into homogenous groups using MACS and to compare those groups with respect to patients' characteristics and neuroimaging findings. Methods: Participants who have a migraine with aura (MwA) were interviewed after each attack in order to obtain the characteristics of migraine aura. Thereafter, we scored the complexity of their auras by MACS. The MACS was used to categorize patients into three groups: MwA-S (with simple aura), MwA-MC (with moderately complex aura), and MwA-C (with complex aura). The patient characteristics and estimated cortical thickness of regions of interest, which are potentially linked to the symptoms that develop during the aura, were used to compare these groups. Results: In total, 338 MwA attacks were recorded in analyzed groups. Scotoma was the most frequently reported symptom in the groups, followed by somatosensory aura in the MwA-C group and zig-zag lines in the MwA-MC and MwA-S groups. Patients in the MwA-C and MwA-MC groups had a thicker cortex in the left primary visual cortex with respect to MwA-S group. In addition, patients in the MwA-C group had a thicker cortex in several visual and somatosensory cortical regions relative to the MwA-S group. Conclusions: Our results show that the newly developed MACS can be used for the stratification of MwA patients, herewith allowing the better investigation of changes in migraineurs' brains. [ABSTRACT FROM AUTHOR]

Objectives: Currently, there is no scoring system for assessing the complexity of migraine aura. Our goal was to develop a Migraine Aura Complexity Score that synthesizes the quantity and quality of aura symptoms and to test its applicability in neuroimaging studies.Methods: Patients with migraine aura were interviewed in order to obtain characteristics of migraine aura. Explorative and confirmatory analyses were used to develop the Migraine Aura Complexity Score. Median values were derived from 10 consecutive migraine auras in each patient. The Migraine Aura Complexity Score was correlated with an average cortical thickness of different brain areas in studied patients. The Surface-based Morphometric Analysis approach was used to estimate cortical thickness.Results: This study included 23 (16 females and seven males) migraineurs with aura. Confirmatory factor analysis suggested the second-order model with three-factor measurement for grading migraine aura. The first factor is linked to higher cortical dysfunction during migraine aura, while the second is associated with the degree of involvement of primary visual and somatosensory cortices; the third linked symptoms of somatosensory aura and hand and head involvement. Positive correlation of Migraine Aura Complexity Score and averaged cortical thickness were found in the left and right hemispheres overall (r = 0.568, p = 0.007; r = 0.617, p = 0.003) and in some of their regions.Conclusions: This study demonstrates that the Migraine Aura Complexity Score could be a valuable tool for assessing migraine aura. The score could be used in neuroradiological studies in order to achieve a stratification of patients with migraine aura. [ABSTRACT FROM AUTHOR]

Although migraine represents one of the most common form of primary headache in the teenage population, most neurophysiologic studies are only on the adulthood. We investigated 38 teenage patients with migraine with aura, 17 male and 21 female, with a mean age of 16.2 years, comparing them with gender- and age-matched patients with migraine without aura and healthy subjects. Also, characteristics of aura were correlated with pattern-reversal visual evoked potential parameters. There was a significant difference in left and right eye N2 wave latencies between migraine with aura and migraine without aura patients or healthy controls. In migraine with aura and migraine without aura, 26.3% of patients had abnormal wave latency. Reported tunnel vision during the aura was correlated with lower N1P1 and/or P1N2 wave amplitudes. Also, higher amplitude in patients with migraine with aura correlated with younger age and earlier disease onset, whereas longer aura duration correlated with prolonged wave latency. Findings suggest that migraine subtypes may be differentiated on the basis of N2 wave latency prolongation. [ABSTRACT FROM AUTHOR]

Introduction: Aura occurs in 20-30% of patients with migraine. Some descriptions of aura go far beyond the most frequentvisual and sensory symptoms, suggesting the involvement of different cortical areas. The aim of this prospective studywas to evaluate the frequency and types of disorders of higher cortical functions (HCF) that occur during visual and/orsensory aura.Methods: We interviewed 60 patients with visual and/or sensory aura about HCF disorders of praxia, gnosia, memory,and speech, during aura. Patients were divided into two groups, with and without HCF disorders, and were compared interms of demographic data and aura characteristics.Results: From all 60 patients, 65% reported at least one HCF disorder during aura. The patients with HCF disorders hadlonger-lasting auras (28.51±16.39 vs. 19.76±11.23, p0.016). The most common HCF disorders were motor dysphasia(82.05%) and dysnomia (30.74%). Motor dysphasia was more often reported by patients with visual as well assensory aura (p=0.002). The number of HCF disorders correlated with the aura duration (p=0.003).Conclusion: According to our results, HCF disorders during aura occur more often than previously thought. The auraduration has some influence on the HCF disorders. [ABSTRACT FROM AUTHOR]

A response from the authors of the article "Pattern-reversal visual evoked potential parameters and migraine in the teenage population" in the 2016 issue is presented.

Background and Purpose: Various features of the cerebral cortex and white matter have been extensively investigated in migraine with aura (MwA), but the morphological characteristics of subcortical structures have been largely neglected. The aim of this study was to identify possible differences in subcortical structures between MwA patients and healthy subjects (HS), and also to determine the correlations between the characteristics of migraine aura and the volumes of subcortical structures., Methods: Thirty-two MwA patients and 32 HS matched by sex and age were analyzed in this study. Regional subcortical brain volumes were automatically calculated using the FSL/FMRIB Image Registration and Segmentation Tool software (https://fsl.fmrib.ox.ac.uk/fsl/fslwiki/Glossary). A general linear model analysis was used to investigate differences in the volume of subcortical structures between the MwA patients and HS. A partial correlation test was used to assess correlations between the volume of subcortical structures and characteristics of MwA., Results: The volumes of the right globus pallidus, left globus pallidus, and left putamen were significantly smaller in MwA patients than in HS (mean±SD): 1,427±135 mm³ vs. 1,557±136 mm³ ( p <0.001), 1,436±126 mm³ vs. 1,550±139 mm³ ( p =0.001), and 4,235±437 mm³ vs. 4,522±412 mm³ ( p =0.006), respectively. There were no significant relationships between subcortical structures and clinical parameters., Conclusions: These findings suggest that both the globus pallidi and left putamen play significant roles in the pathophysiology of the MwA. Future studies should determine the cause-and-effect relationships, since these could not be discriminated in this study due to its cross-sectional design., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2019 Korean Neurological Association.)

Patients with migraine with aura often experience a variety of visual and somatosensory phenomena and disturbances of higher cortical functions. Analysis of these alterations may provide important information about the involvement of different cortical regions in cortical spreading depression (CSD). We report five cases of migraineurs who experience unusually abundant clinical phenomena during auras. These patients were selected from a cohort of migraine with aura patients who were interviewed, using a specially designed questionnaire, to evaluate the presence of higher cortical dysfunctions. On the basis of the aura symptoms they reported, we attempted to infer the origin and the possible paths of CSD in each patient. According to their reported symptoms, CSD could begin in the primary visual cortex, in the primary somatosensory cortex or simultaneously in both, and propagate to the posterior parietal cortex, the temporal lobe and Broca's area. We believe that clinical descriptions of aura could play an important role in further investigations of the pathophysiology of migraine.