1 40 2 Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month Backlog URL Address <a href="http://doi.org/10.1016/j.pediatrneurol.2006.07.004" target="_blank" rel="noreferrer">http://doi.org/10.1016/j.pediatrneurol.2006.07.004</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Links between abnormal brain structure and cognition in holoprosencephaly Publisher An entity responsible for making the resource available Pediatric Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2006 Subject The topic of the resource Child; Female; Humans; Male; Age Factors; Severity of Illness Index; Cognition; Sex Factors; Predictive Value of Tests; adolescent; Preschool; infant; Q3 Literature Search; Neuropsychological Tests; Brain/abnormalities; Cognition Disorders/pathology; Corpus Striatum/abnormalities; Epilepsy/pathology; Holoprosencephaly/pathology; Hypothalamus/abnormalities; Motor Skills Disorders/pathology; Thalamic Nuclei/abnormalities Creator An entity primarily responsible for making the resource Roesler CP; Paterson SJ; Flax J; Hahn JS; Kovar C; Stashinko EE; Jing H; Benasich AA Description An account of the resource Converging information on medical issues, motor ability, and cognitive outcomes is essential when addressing long-term clinical management in children with holoprosencephaly. This study considered whether adding more informative structural indices to classic holoprosencephaly categories would increase prediction of cognitive outcomes. Forty-two children with holoprosencephaly were examined to determine the association of deep gray nuclei abnormalities with cognitive abilities and the effect of motor skill deficits on cognitive performance. Additionally, a cognitive profile was described using the Carter Neurocognitive Assessment, an experimental diagnostic instrument designed specifically for young children with severe neurodevelopmental dysfunction. Findings indicated that nonseparation of the deep gray nuclei was significantly associated with the cognitive construct of vocal communication, but not with the cognitive constructs of social awareness, visual attention, or auditory comprehension. Importantly, motor skill deficits did not significantly affect performance on the Carter Neurocognitive Assessment. This study is the first investigation to provide a descriptive overview of specific cognitive skills in this group of children. The results also strongly suggest that this feature of the brain's structure does not predict all aspects of neurodevelopmental function. These findings contribute a critical component to the growing body of knowledge regarding the medical and clinical outcomes of children with holoprosencephaly. 2006 Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1016/j.pediatrneurol.2006.07.004" target="_blank" rel="noreferrer">10.1016/j.pediatrneurol.2006.07.004</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). Type The nature or genre of the resource Journal Article 2006 Adolescent Age Factors Backlog Benasich AA Brain/abnormalities Child Cognition Cognition Disorders/pathology Corpus Striatum/abnormalities Epilepsy/pathology Female Flax J Hahn JS Holoprosencephaly/pathology Humans Hypothalamus/abnormalities Infant Jing H Journal Article Kovar C Male Motor Skills Disorders/pathology Neuropsychological Tests Paterson SJ Pediatric Neurology Predictive Value of Tests Preschool Q3 Scoping Review Results Roesler CP Severity Of Illness Index Sex Factors Stashinko EE Thalamic Nuclei/abnormalities Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month Backlog URL Address <a href="http://doi.org/10.1212/wnl.59.7.1058" target="_blank" rel="noreferrer">http://doi.org/10.1212/wnl.59.7.1058</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Neuroanatomy of holoprosencephaly as predictor of function: beyond the face predicting the brain Publisher An entity responsible for making the resource available Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2002 Subject The topic of the resource Child; Female; Humans; Male; Adult; Prospective Studies; Magnetic Resonance Imaging; Linear Models; Chi-Square Distribution; Forecasting; adolescent; Preschool; infant; Q3 Literature Search; Nonparametric; Statistics; Tomography; Brain/pathology; X-Ray Computed; Face/pathology; Holoprosencephaly/pathology/physiopathology; Seizures/pathology/physiopathology Creator An entity primarily responsible for making the resource Plawner LL; Delgado MR; Miller VS; Levey EB; Kinsman SL; Barkovich AJ; Simon EM; Clegg NJ; Sweet VT; Stashinko EE; Hahn JS Description An account of the resource BACKGROUND: Despite advances in neuroimaging and molecular genetics of holoprosencephaly (HPE), the clinical spectrum of HPE has remained inadequately described. OBJECTIVE: To better characterize the clinical features of HPE and identify specific neuroanatomic abnormalities that may be useful predictors of neurodevelopmental function. METHODS: The authors evaluated 68 children with HPE in a multicenter, prospective study. Neuroimaging studies were assessed for the grade of HPE (lobar, semilobar, and alobar), the degree of nonseparation of the deep gray nuclei, and presence of dorsal cyst or cortical malformation. RESULTS: In general, the severity of clinical problems and neurologic dysfunctions correlated with the degree of hemispheric nonseparation (grade of HPE). Nearly three-quarters of the patients had endocrinopathies, with all having at least diabetes insipidus. The severity of endocrine abnormalities correlated with the degree of hypothalamic nonseparation (p = 0.029). Seizures occurred in approximately half of the children with HPE. The presence of cortical malformations was associated with difficult-to-control seizures. The presence and degree of dystonia correlated with the degree of nonseparation of the caudate and lentiform nuclei and the grade of HPE (p < 0.05). Hypotonia correlated with the grade of HPE (p < 0.05). Mobility, upper extremity function, and language correlated with the degree of nonseparation of the caudate, lentiform and thalamic nuclei, and grade of HPE (p < 0.01). CONCLUSIONS: Patients with HPE manifest a wide spectrum of clinical problems and neurologic dysfunction. The nature and severity of many of these problems can be predicted by specific neuroanatomic abnormalities found in HPE. 2002 Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1212/wnl.59.7.1058" target="_blank" rel="noreferrer">10.1212/wnl.59.7.1058</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). Type The nature or genre of the resource Journal Article 2002 Adolescent Adult Backlog Barkovich AJ Brain/pathology Chi-Square Distribution Child Clegg NJ Delgado MR Face/pathology Female Forecasting Hahn JS Holoprosencephaly/pathology/physiopathology Humans Infant Journal Article Kinsman SL Levey EB Linear Models Magnetic Resonance Imaging Male Miller VS Neurology Nonparametric Plawner LL Preschool Prospective Studies Q3 Scoping Review Results Seizures/pathology/physiopathology Simon EM Stashinko EE Statistics Sweet VT Tomography X-Ray Computed