Neurol. praxi. 2012;13(1):47-52

Subcortical band heterotopia of the cerebral cortex with pharmacoresistant epilepsy

MUDr.Milan Labonek¹, MUDr.Marta Neklanová², MUDr.Jindřiška Burešová¹, doc.MUDr.Stanislav Buřval, Ph.D.³, doc.MUDr.Robert Kuba, Ph.D.⁴, prof.MUDr.Roman Herzig, Ph.D.¹, prof.MUDr.Petr Kaňovský, CSc.¹

¹ Neurologická klinika LF UP a FN Olomouc

² Dětská klinika LF UP a FN Olomouc

³ Radiologická klinika LF UP a FN Olomouc

⁴ Centrum pro epilepsie Brno, FN u sv. Anny, LF MU Brno

Subcortical laminar heterotopia of the cerebral cortex, also referred to as band heterotopia (BH) or double cortex, is a rather rare

developmental defect of the cerebral cortex characterized by the presence of large bands of grey matter below the surface of the

cortex. This malformation occurs mostly in women and is manifested as sporadic or familial cases with epilepsy and varying degrees

of mental retardation. The most common cause of the disease is a mutation in the doublecortin gene, resulting in BH in females and,

predominantly, in agyria-pachygyria and, only sporadically, in BH in males. Two female patients were followed for delayed psychomotor

development and pharmacoresistant epilepsy with partial seizures with occasional secondary generalizations. Magnetic resonance

imaging of the brain showed a typical presentation of BH. Multiregional epileptiform activity with an occasional generalized epileptiform

pattern was recorded on electroencephalography (EEG). An ictal pattern with secondary generalized epileptiform activity was

repeatedly detected during video-EEG monitoring. During many years of follow-up of the patients, a number of antiepileptic drugs

and their combinations have been tried. Due to comprehensive evaluation, including video-EEG monitoring and an analysis of drug

history, it has been possible to optimize the treatment with antiepileptic drugs and even to discontinue some of them. In one of the

patients, treatment with vagus nerve stimulation was indicated, which resulted in a significant reduction in atonic and in myoclonic

seizures. In terms of pharmacotherapy, BH is a very challenging area for an epileptologist, with a need for combined therapy using

antiepileptic drugs with their potential interactions and side effects; the possibilities for successful epilepsy surgery are largely limited

in this case. With that said, a certain hope of at least some improvement for these patients remains in VNS treatment or palliative

anterior callosotomy.

Keywords: band heterotopia, double cortex, pharmacoresistant epilepsy, mental retardation

Published: February 14, 2012  Show citation