Presentation
Intermittent seizures, afebrile.
Patient Data
Selected CT images demonstrating asymmetry of the cerebral hemispheres with enlargement of the left one, associated with increased amount of gray matter and less gyration.
Selected MRI images demonstrating classic features of left hemimegalencephaly, involving the frontal, parietal and occipital lobes and to lesser extent the temporal lobe.
Selected images demonstrating post-operative changes related to a left modified functional hemispherectomy with partial resection of left temporal pole, left amygdala, and perisylvian left frontal lobe.
Case Discussion
27 day old, afebrile boy presented with intermittent seizure, where that CT raised the suspicion of hemimegalencephaly. One day later the MRI confirmed the diagnosis.
About 2 years later the patient underwent left modified functional hemispherectomy.
The patient developed new-onset of seizure five months after surgery, where the MRI showed macroscopically a complete disconnection, however, the diffusion tensor imaging (tractography) showed residual interhemispheric connecting fibers through the anterior corpus callosum and anterior commissure.
Hemimegalencephaly is a rare malformation characterized by enlargement of one cerebral hemisphere with an increased amount of the gray matter and thickening of the cortical ribbons. This might affect either one lobe or the entire hemisphere. Asymmetric appearance of the cranium may indicate it. The main clinical manifestations include hemiparesis, seizure and developmental retardation.
An afebrile child presenting with seizure should raise suspicions of developmental issues, such as hemimegalencephaly. We point out the importance of imaging modalities in detecting the operable causes of seizure.
Case contributed by Dr. Christine Saint-Martin, Associate Professor in the Neuroradiology and Pediatric Radiology at McGill University.
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