Subcortical band heterotopy or "double cortex" is a neuronal migration disorder characterized by the presence of a diffuse subcortical grey matter band separated from both lateral ventricles and overlying cortex, which can show simplified gyral pattern. The syndrome is usually associated with mutations in the DCX (Xq22.3-q23) gene, and much less frequently in the LIS1 (17p13.3) gene. This syndrome occurs very rarely in males. Common clinical features of SBH are variable degrees of mental retardation and epilepsy. About half of the patients suffer from generalized epilepsy (generalized tonic-clonic seizures, atypical absences, atonic seizures, myoclonic seizures, infantile spasms), often with the characteristics of Lennox-Gastaut syndrome, while the remaining patients have focal epilepsy. Overall, 65% of the patients studied have intractable epilepsy. In the scientific literature is well known that "double cortex" syndrome frequently leads to intractable epilepsy, as in our patient. We report here in one girl the occurrence of recurrent status epilepticus, whose particular electroclinical features have not been previously described.
|Translated title of the contribution||Recurrent status epilepticus in a patient with mental retardation and "double cortex"|
|Number of pages||3|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - May 2008|
ASJC Scopus subject areas
- Clinical Neurology