Malformations of Cortical Development

Cerebral cortex development is an extremely complex process overall and the current understanding is incomplete and constantly changing as our understanding of genetics and the processes each gene controls emerges from scientific research. Neural cells are produced in the subventicular zone of the pallial or dorsal germinal epithelium. They then differentiate and migrate radially or tangentially to the cerebral cortex and organize themselves to function.Malformations of cortical development (MCD) is a heterogeneneous group of disorders that cause defective cortical development. These disorders are broadly categorized into three areas of cerebral cortical development: neural cell proliferation, neural cell migration and cortical organization. There is wide variation in the radiographic features and clinical problems seen and individuals may have more than one of these broad classifications (i.e. have elements 1 or all 3 classifications such as polymicrogyri). Additionally, the brain may be affected with these malformations globally or focally (focal cortical development). Specific wording and classifications for MCDs are necessarily changing as more information is learned. MCDs that are more global or that are due to neural cell proliferation problems are often more severe.

The study was performed on a 3-year-old male came to the inpatient floor because of intractable emesis of non-bilious fluid and stomach contents and some watery diarrhea for 36 hours after exposure to a particularly virulent form gastroenteritis at his daycare. He was not tolerating any oral or gastrotube feedings and had not urinated for 10 hours. He also had a 10-15 second generalized tonic-clonic seizure in the emergency room that was similar to his usual seizures that generally occurred 2-3 times/week. He also had a bout of watery diarrhea in the emergency room. The past medical history showed him known to have microcephaly with some areas of polymicrogyri diagnosed as an infant, and seizures that were reasonably well-controlled on phenytoin.

The pertinent physical exam showed a male in mild distress with dry mucous membranes. His weight was down 1040 grams from a recent clinic visit. His heart rate was 112 beats/minute and respirations were 26/minute. His blood pressure was 76/54. His abdomen was very mildly tender diffusely without guarding or rebound tenderness. His gastric tube was in place. There was no organomegaly and his bowel sounds were hyperactive. Neurologically he was semi-noncommunicative. His cranial nerves were normal, DTRs were slightly hyperreflexic and his tone was good. This was his normal neurological examination and the rest of his examination was normal. The work-up showed a normal abdominal radiograph, electrolytes and urinalysis were consistent with mild dehydration, and his complete blood count was normal. His phenytoin level was therapeutic. The diagnosis of gastroenteritis and dehydration was made. In the emergency room he was given on dansteron and intravenous fluids but he still could not tolerate fluids and was admitted. Over the next 24 hours he began to tolerate fluids. He had another similar seizure but again had therapeutic levels and his parents and neurologist were comfortable monitoring him at home and potentially making medication changes by telephone.

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