OBJECTIVE: To define the behavioral profile of periventricular nodular heterotopia (PNH), a malformation of cortical development that is associated with seizures but reportedly normal intelligence, and to correlate the results with anatomic and clinical features of this disorder. METHODS: Ten consecutive subjects with PNH, all with epilepsy and at least two periventricular nodules, were studied with structural MRI and neuropsychological testing. Behavioral results were statistically analyzed for correlation with other features of PNH. RESULTS: Eight of 10 subjects had deficits in reading skills despite normal intelligence. Processing speed and executive function were also impaired in some subjects. More marked reading difficulties were seen in subjects with more widely distributed heterotopia. There was no correlation between reading skills and epilepsy severity or antiepileptic medication use. CONCLUSION: The neuronal migration disorder of periventricular nodular heterotopia is associated with an impairment in reading skills despite the presence of normal intelligence.
OBJECTIVE: To define the behavioral profile of periventricular nodular heterotopia (PNH), a malformation of cortical development that is associated with seizures but reportedly normal intelligence, and to correlate the results with anatomic and clinical features of this disorder. METHODS: Ten consecutive subjects with PNH, all with epilepsy and at least two periventricular nodules, were studied with structural MRI and neuropsychological testing. Behavioral results were statistically analyzed for correlation with other features of PNH. RESULTS: Eight of 10 subjects had deficits in reading skills despite normal intelligence. Processing speed and executive function were also impaired in some subjects. More marked reading difficulties were seen in subjects with more widely distributed heterotopia. There was no correlation between reading skills and epilepsy severity or antiepileptic medication use. CONCLUSION: The neuronal migration disorder of periventricular nodular heterotopia is associated with an impairment in reading skills despite the presence of normal intelligence.
Authors: M van Kogelenberg; S Ghedia; G McGillivray; D Bruno; R Leventer; K Macdermot; J Nelson; L Nagarajan; J A Veltman; A P de Brouwer; R J McKinlay Gardner; H van Bokhoven; E P Kirk; S P Robertson Journal: Mol Syndromol Date: 2010-01-08
Authors: Kristiina Tammimies; Isabel Tapia-Páez; Joëlle Rüegg; Gustaf Rosin; Juha Kere; Jan-Åke Gustafsson; Ivan Nalvarte Journal: Mol Endocrinol Date: 2012-03-01
Authors: Joanna A Christodoulou; Mollie E Barnard; Stephanie N Del Tufo; Tami Katzir; Susan Whitfield-Gabrieli; John D E Gabrieli; Bernard S Chang Journal: Epilepsy Behav Date: 2013-10-03 Impact factor: 2.937
Authors: Russell J Ferland; Luis Federico Batiz; Jason Neal; Gewei Lian; Elizabeth Bundock; Jie Lu; Yi-Chun Hsiao; Rachel Diamond; Davide Mei; Alison H Banham; Philip J Brown; Charles R Vanderburg; Jeffrey Joseph; Jonathan L Hecht; Rebecca Folkerth; Renzo Guerrini; Christopher A Walsh; Esteban M Rodriguez; Volney L Sheen Journal: Hum Mol Genet Date: 2008-11-07 Impact factor: 6.150