TY - JOUR
T1 - Early onset myoclonic epilepsy and 15q26 microdeletion: observation of the first case
AU - Veredice, Chiara
AU - Bianco, Flaviana
AU - Contaldo, Ilaria
AU - Orteschi, Daniela
AU - Stefanini, Maria Chiara
AU - Battaglia, Domenica Immacolata
AU - Lettori, Donatella
AU - Guzzetta, Francesco
AU - Zollino, Marcella
PY - 2009
Y1 - 2009
N2 - The authors report the study of a 30-month-old girl with refractory myoclonic epilepsy associated with mental retardation, growth delay, peculiar facial appearance, and minor physical anomalies. Extensive genetic studies were performed, including an array-based comparative genomic hybridization (array-CGH) that showed a cryptic interstitial deletion of 15q (5 Mb) affecting the 15q26.1-26.2 region. Partial deletions of the long arm of chromosome 15, including the 15q26 region, were observed in syndromic associations that typically include congenital diaphragmatic hernia, but neurologic features were poorly described and epileptic seizures were never reported. Our findings suggest that genes for seizures could be included in the 15q26.1q26.2 deletion interval.
AB - The authors report the study of a 30-month-old girl with refractory myoclonic epilepsy associated with mental retardation, growth delay, peculiar facial appearance, and minor physical anomalies. Extensive genetic studies were performed, including an array-based comparative genomic hybridization (array-CGH) that showed a cryptic interstitial deletion of 15q (5 Mb) affecting the 15q26.1-26.2 region. Partial deletions of the long arm of chromosome 15, including the 15q26 region, were observed in syndromic associations that typically include congenital diaphragmatic hernia, but neurologic features were poorly described and epileptic seizures were never reported. Our findings suggest that genes for seizures could be included in the 15q26.1q26.2 deletion interval.
KW - Abnormalities, Multiple
KW - Adult
KW - Age of Onset
KW - Child, Preschool
KW - Chromosome Deletion
KW - Chromosomes, Human, Pair 15
KW - Comparative Genomic Hybridization
KW - Epilepsies, Myoclonic
KW - Female
KW - Hernia, Diaphragmatic
KW - Humans
KW - In Situ Hybridization, Fluorescence
KW - Intellectual Disability
KW - Magnetic Resonance Imaging
KW - Male
KW - Oligonucleotide Array Sequence Analysis
KW - Syndrome
KW - Abnormalities, Multiple
KW - Adult
KW - Age of Onset
KW - Child, Preschool
KW - Chromosome Deletion
KW - Chromosomes, Human, Pair 15
KW - Comparative Genomic Hybridization
KW - Epilepsies, Myoclonic
KW - Female
KW - Hernia, Diaphragmatic
KW - Humans
KW - In Situ Hybridization, Fluorescence
KW - Intellectual Disability
KW - Magnetic Resonance Imaging
KW - Male
KW - Oligonucleotide Array Sequence Analysis
KW - Syndrome
UR - http://hdl.handle.net/10807/31927
U2 - 10.1111/j.1528-1167.2009.02078.x
DO - 10.1111/j.1528-1167.2009.02078.x
M3 - Article
SN - 1528-1167
VL - 50
SP - 1810
EP - 1815
JO - Epilepsia
JF - Epilepsia
ER -