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Aim: To evaluate gross motor function and associated factors in children with congenital Zika syndrome (CZS).Method: Fifty-nine children (30 males, 29 females) with CZS at a mean (SD) age of 14.7 (3.9), months (range 5-29mo) were evaluated using the Gross Motor Function Measure (GMFM) and classified according to the Gross Motor Function Classification System (GMFCS). Neurological damage was evaluated by neuroimaging. The mothers' sociodemographic characteristics and general data on the children were obtained from interviews with the mothers and from the children's medical records. Correlational and multiple regression analyses were performed to identify factors associated with these children's motor function.Results: In 81% of the children, motor function impairment was severe, classified as GMFCS level V. The overall GMFM score ranged from 5 to 210 (median 18; interquartile range 11), with only four children receiving scores in the D and E dimensions. The factors found to affect motor function were the presence of severe malformations of cortical development and small head circumference at birth.Interpretation: Although motor impairment may be mild in some children, it is generally severe. Severe malformations of cortical development and small head circumference at birth were factors associated with poorer motor function, reflecting the greater severity of brain damage.What This Paper Adds: Motor impairment is severe in most children with congenital Zika syndrome (CZS). Motor skills are adequate or close to adequate for age in 7% of children with CZS. Severe malformations of cortical development are associated with poor motor control. Small head circumference at birth is also associated with poor motor control. [ABSTRACT FROM AUTHOR]

Aim: We aimed to systematically review the speech production, language, and oral function phenotype of bilateral perisylvian polymicrogyria (BPP), and examine the correlation between the topography of polymicrogyria and the severity of speech, language, and oral functional impairment.Method: A systematic search of MEDLINE, Embase, and PubMed databases was completed on 26th October 2017 using Medical Subject Heading terms synonymous with BPP and speech, language, or oral motor impairment. In total, 2411 papers were identified and 48 met inclusion criteria.Results: Expressive and receptive language impairment and oral structural and functional deficits are frequent in BPP. Expressive deficits are frequently more severe than receptive. Only one study used formal assessments to demonstrate the presence of speech disorder, namely dysarthria. Seven studies reported an association between diffuse BPP and more severe language impairment.Interpretation: Findings confirmed that language deficits are common in BPP, though assessment of the specific speech phenotype is limited. The paucity of high quality studies detailing the specific communication phenotype of BPP highlights the need for further investigation. Improving understanding of this phenotype will inform the development of targeted therapies and lead to better long-term outcomes.What This Paper Adds: Speech, language, and oral functional impairments are common in individuals with bilateral perisylvian polymicrogyria. Posterior polymicrogyria is associated with a less severe language impairment than anterior polymicrogyria. Deeper investigation of speech is needed to understand implicated networks in this malformation. [ABSTRACT FROM AUTHOR]

Aim: Polymicrogyria (PMG) is one of the most common forms of cortical malformation yet the mechanism of its development remains unknown. This study describes the histopathological aspects of PMG in a large series including a significant proportion of fetal cases.Method: We have reviewed the neuropathology and medical records of 44 fetuses and 27 children and adults in whom the cortical architecture was focally or diffusely replaced by one or more festooning bands of neurons.Results: The pial surface of the brain overlying the polymicrogyric cortex was abnormal in almost 90% of cases irrespective of the aetiology. This accords with animal studies indicating the importance of the leptomeninges in cortical development. The aetiology of PMG was highly heterogeneous and there was no correlation between cortical layering patterns and aetiology. PMG was almost always associated with other brain malformations.Interpretation: The inclusion of many fetal cases has allowed us to examine the early developmental stages of PMG. The study indicates the significance of surface signals responsible for human corticogenesis and the complex interaction between genetic and environmental factors leading to this common endpoint of cortical maldevelopment. [ABSTRACT FROM AUTHOR]

Aim Many neurodevelopmental disorders co-occur yet are rarely studied in terms of brain development. Developmental coordination disorder ( DCD) and attention-deficit-hyperactivity disorder ( ADHD) co-occur at a high frequency and are associated with functional and structural brain alterations. The objective of this study was to examine whether the effects of comorbid motor and attention problems influence cortical thickness in children and whether the pattern of changes for concurrent disorders is distinct from the alterations seen in single disorders. Method A total of 34 children (19 males, 15 females, mean age 9y 9mo, range 8-17y) who met the criteria for DCD ( n=14), ADHD ( n=10), or DCD+ ADHD ( n=10) were recruited into the study. Fourteen participants with typical development (eight males, six females, mean age 11y 9mo, range 8-17y) were also recruited for comparison. Participants underwent neuropsychological assessment and magnetic resonance imaging. Cortical thickness analysis was performed to determine the patterns of cortical thinning in each disorder, which was then compared across groups. Results Children with comorbid DCD+ ADHD demonstrated more widespread decreases in cortical thickness than participants with a diagnosis of DCD or ADHD alone. Cortical thinning was found to be concentrated in the frontal, parietal, and temporal lobes, and was correlated with measures of motor and attentional functioning. Interpretation The co-occurrence of DCD+ ADHD was associated with a distinct global pattern of regional cortical thickness decrease, highlighting the unique neurobiology of comorbid neurodevelopmental disorders. This novel feature of concurrent DCD and ADHD may help inform diagnostic definitions and provide clues to both the shared and the isolated genetic and environmental origins of motor and attention disorders. [ABSTRACT FROM AUTHOR]

Aims Hemimegalencephaly ( HME) is a rare congenital malformation of cortical development, usually associated with developmental delay and severe epilepsy. This condition has rarely been reported in adults. The aim of this study was to examine and compare neurological findings in adult patients with HME. Method We retrospectively examined adult patients with HME by evaluating the presence of neurocutaneous disorders, current cognitive development, seizure control, and documentation of therapies for seizure management and outcomes. Results Five patients were included in the study (three males, two females; mean age 23y 9mo [ SD 6y 1mo], range 18-34y). Four patients had HME that was associated with neurocutaneous syndromes and the remaining patient had isolated HME. Two patients required surgical treatment for seizures in childhood. One patient had no intellectual disability, while one had mild, and three severe intellectual disability. All patients presented motor deficits ranging from mild hemiparesis in two patients to non-ambulation in one patient. Patients in whom seizure onset occurred after the 7 years of age had better seizure control and psychomotor development in adulthood than patients in whom seizure onset occurred in the first year of life. Interpretation In our small sample of adults with HME, age at seizure onset, cognitive disability, and seizure control were found to be associated. [ABSTRACT FROM AUTHOR]

This commentary is on the original article by Jansen et al. on pages 39–48 of this issue. [ABSTRACT FROM AUTHOR]

The author discusses better definition of Tourette syndrome which is a neuropsychiatric disorder often associated with behavioral disorders. He includes the possibility that the motor phenotype of syndrome encompasses more than tic disorders which includes dysfunction of different areas of the basal ganglia and even cerebral cortex of patients. He also mentions the differences in finding related to dysfunction through Positron Emission Tomography (PET) imaging.