Souza AFA, Amado NI, Menezes NPM, Haun SAJ, Teles MPC, Ferreira Vila NJPJ, Costa da Costa J

Language: English
References: 33
Page: 115-119
PDF size: 119.75 Kb.

ABSTRACT

The disturbances of neural migration (cortical dysplasias) are a group of relatively rare diseases of the central nervous system. Among the diffuse or generalized forms, the Double Cortex Syndrome (DCS) is on the other side of severity. We report the case of a 14 year-old female patient, with a hard control epilepsy. In the Cranial Magnetic Resonance (CMR), a continuous lamina of neurons was noted in a subcortical situation. This lamina was separat-ed from the suprajacent cortex by an organized white matter, which is compatible with a double cortex configuration. The migration disturbances are caused by interferences in the activity between neuroblast and glial fibra, or with neuroblastic movimentation through glial fibra during central nervous tissue’s maturation. Symptoms vary from normal to a severe growth disturbance or mental retardation. Almost all patients course with epilepsy, which begins in childhood. The DCS diagnosis is made by characteristic pictures in Magnetic Resonance (MR). The EEG alterations are extremely variable. Functional Magnetic Resonance is useful in the investigation of the double cortex role in the normal cerebral functioning. Some patients give a good response with antiepileptic medication. Surgical treatment is controversial.

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