Bilateral central macrogyria: epilepsy, pseudobulbar palsy, and mental retardation--a recognizable neuronal migration disorder.
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.
Kuzniecky
R
R
Department of Neurology and Neurosurgery, McGill University, Montreal, Canada.
Andermann
F
F
Tampieri
D
D
Melanson
D
D
Olivier
A
A
Leppik
I
I
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000601:surgery; D001927:Brain Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D002533:Cerebral Angiography; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000503:physiopathology; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D010243:Paralysis / Q000503:physiopathology; D012640:Seizures / Q000503:physiopathology; D014057:Tomography, X-Ray Computed