Focal Cortical Dysplasia: A Review of Pathological Features, Genetics, and Surgical Outcome

Vincent Y. Wang, M.D. Ph.D.; Edward F. Chang, M.D.; Nicholas M. Barbaro, M.D.

Disclosures

Neurosurg Focus. 2006;20(1) 

In This Article

Pathological and Pathophysiological Features

Focal cortical dysplasias are part of an array of disorders described variously as disorders of cortical development, cortical dysplasias, cortical dysgenesis, or neuronal migration disorders.[42] Classification of these disorders depends on either their pathological characteristics or the proposed origin of the pathological elements (for example, neuronal migration). Histological findings in cortical dysplasia include architectural abnormalities such as cortical laminar disorganization and columnar disorganization. More severe forms of FCD are characterized by the presence of abnormal neuronal elements, such as immature neurons, dysmorphic neurons, giant cells, and balloon cells. Immature neurons are round homogeneous cells with large nuclei.[42] Dysmorphic neurons have distorted cell body, axon, and dendrite morphology caused by the accumulation of neurofilaments within the cytoplasm.[44,45] Giant cells are increased in size but are normal in shape and do not show an accumulation of neurofilaments.[42,47] Balloon cells are considered a hallmark of FCD, although they are not present in all patients with FCD. First described by Taylor, et al.,[50] these cells have an eosinophilic cytoplasm and an eccentric nucleus. Immunohistochemical studies have shown that balloon cells have both neuronal and glial characteristics. Many of these cells are immunopositive for vimentin, and others are immunopositive for glial fibrillary acidic protein, neuron-specific enolase, microtubule-associated protein, or neuronal-specific nuclear protein.[42,44,45,48]

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