RESUMEN
Double cortex syndrome is an uncommon familial syndrome with X-linked dominant inheritance and most commonly presents with developmental delay and seizures. We present a case of a 14-year-old girl who came to neurology department of the hospital with severe generalized tonic-clonic fits and loss of consciousness. The mother of child gave history of uneventful antenatal period and labor. There was history of immediate cry and normal APGAR score. She was achieving milestones normally until at the age of 3 years when she suffered decline in her speech and vision. She had problems with learning with lack of concentration during her schooling. Physical examination was also unremarkable. Her lab values including complete blood count, serum calcium, and arterial blood gas tests, all were within normal limits. Electroencephalogram showed significant changes suggestive of epilepsy. Magnetic resonance imaging of brain showed continuous band of gray matter that was located deep and paralleling the cortex in both cerebral hemispheres suggestive of band heterotopia or double cortex syndrome. She was discharged and prescribed antiepileptics; and was advised regular outpatient follow-up.
RESUMEN
Objective Approximately 5-10% of preschool age children are considered developmentally disabled. Brain Magnetic Resonance Imaging (MRI) plays a key role in the diagnostic evaluation in these children. Many congenital or acquired brain anomalies are revealed with MRIs. Although the majority of these abnormalities are sporadic but patients with subcortical band heterotopia or double cortex syndrome have sex-linked inheritance. We are going to present the first case in Iran from Ahvaz city, which was presented with status epilepticus associated with developmental delay and finally diagnosed as double cortex syndrome, because band heterotopia cases especially for continuous or generalized form is rare. A 4.5-year-old developmentally delayed girl was admitted for generalized tonic clonic seizure attack of 1 hr, upward gaze, locked mouth, and urinary incontinence (status epilepticus) in the child neurology ward. She had a history of recurrent seizures that started as febrile seizures since she was 12 months of age and had frequent admissions for having recurrent seizure attacks. She was the only child of consanguineous parents with negative family history of any neurologic problems. She was a product of uneventful term pregnancy, vaginal delivery with a low Apgar score at birth who was admitted for six days in the neonatal ward for hypotonia and cyanosis. At 4.5 years of age, she had HC: 45cm (<3%) Length: 102 cm (25-75%), and BW: 18kg (75%). She was able to sit, walk with support, speak a few words, and communicate with others. A physical exam was unremarkable. Lab data including CBC, blood biochemical, and urinalysis results were all within normal limits, but the electroencephalography (EEG) revealed generalized poly spike-wave discharges. A brain MRI showed corpus callosal dysplasia, generalized band heterotopia, and polymicrogyria. She was discharged home with oral valproate and regular outpatient follow-ups. In the diagnostic evaluation of developmentally delayed and epileptic children, a brain MRI is strongly recommended for accurate diagnosis of anomalies such as neuronal migration disorders (band heterotopia) and others, because appropriate therapeutic management, prognosis, prevention, and genetic counseling for prenatal diagnosis are dependent on definite diagnosis of the proband case.