Four patients with medically intractable epilepsy, pseudobulbar palsy and mental retardation and found to have bilateral central macrogyria on CT and MRI are reported from the Department of Neurology and Neurosurgery, McGill University, and the Montreal Neurological Institute and Hospital, Montreal, Canada; and the Department of Neurology, University of Minnesota, St. Paul Ramsey Hospital, St. Paul, MN. The pseudobulbar palsy was associated with oromotor incoordination, developmental delay and mild retardation. Minor seizures developed between the ages of eight and nine years and one patient had infantile spasms at three months of age. Electroencephalographic epileptogenic abnormalities were secondary generalized or multifocal. CT scans revealed symmetrical bilateral sylvian and rolandic macrogyria extending into the parietal regions. The cortex appeared thick and smooth with the underlying white matter diminished. The MRI confirmed the CT findings and showed that the abnormal cortex had a lower signal as compared to normal gray matter of frontal and occipital regions. The thick cortical structures surrounded a large central sulcus reminiscent of a fetal sylvian fissure. Two patients tried on multiple anticonvulsants continued to have frequent seizures whereas two treated by callosotomy had no subsequent drop attacks and improved behavior. The authors suggest that the clinical and imaging features of these patients indicate a distinct and specific syndrome and the malformations appear to result from specific derangement of neuronal migration. [1]

COMMENT. Neuronal migration disorders, including agyria (or lissencephaly), macrogyria (or pachygyria), polymicrogyria, schizencephaly, and other heterotopias of the gray matter are being recognized more frequently by the use of the magnetic resonance imaging technique. The role of neurosurgical treatment in patients with neuronal migration disorders is being explored. The early recognition and effective surgical management of these cases might improve prognosis and prevent the development of intractable epilepsy and mental deterioration.