TY - JOUR
T1 - Bilateral central macrogyria
T2 - Epilepsy, pseudobulbar palsy, and mental retardation—a recognizable neuronal migration disorder
AU - Kuzniecky, Ruben
AU - Andermann, Frederick
AU - Tampieri, Donatella
AU - Melanson, Denis
AU - Olivier, Andre
AU - Leppik, I.
PY - 1989/6
Y1 - 1989/6
N2 - The neuronal migration disorders comprise several morphological entities that are recognizable during life using current imaging techniques. We studied 4 patients who had a characteristic bilateral central rolandic and sylvian macrogyria. The patients had pseudobulbar palsy with oromotor incoordination and developmental delay and were mildly retarded. Minor seizures developed between the ages of 8 and 9 years. Subsequently, atonic drop attacks became the predominant epileptic pattern. Epileptogenic electrographic abnormalities were secondary generalized or multifocal. The lesions were detected by computed tomography and magnetic resonance imaging in all patients. Bilateral symmetrical areas of thick cortex surrounding a large sulcus were seen. This syndrome consists of specific clinical, imaging, electroencephalographic, and epileptic features. It can be suspected clinically and confirmed by imaging studies. Callosotomy in two patients helped the intractable seizures.
AB - The neuronal migration disorders comprise several morphological entities that are recognizable during life using current imaging techniques. We studied 4 patients who had a characteristic bilateral central rolandic and sylvian macrogyria. The patients had pseudobulbar palsy with oromotor incoordination and developmental delay and were mildly retarded. Minor seizures developed between the ages of 8 and 9 years. Subsequently, atonic drop attacks became the predominant epileptic pattern. Epileptogenic electrographic abnormalities were secondary generalized or multifocal. The lesions were detected by computed tomography and magnetic resonance imaging in all patients. Bilateral symmetrical areas of thick cortex surrounding a large sulcus were seen. This syndrome consists of specific clinical, imaging, electroencephalographic, and epileptic features. It can be suspected clinically and confirmed by imaging studies. Callosotomy in two patients helped the intractable seizures.
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U2 - 10.1002/ana.410250604
DO - 10.1002/ana.410250604
M3 - Article
C2 - 2500888
AN - SCOPUS:0024323989
SN - 0364-5134
VL - 25
SP - 547
EP - 554
JO - Annals of Neurology
JF - Annals of Neurology
IS - 6
ER -