Surgical Resection for Intractable Epilepsy in “Double Cortex” Syndrome Can Yield Adequate Results

To the Editor: We read with interest the article by Bernasconi et al. (1) reporting a case series of intractable epilepsy in “double cortex” syndrome, or subcortical band heterotopia, in which they concluded that focal surgical resection of epileptogenic tissue yields inadequate results. However, we have observed a patient with this syndrome who underwent anterior temporal lobectomy and who has experienced a good outcome. In this 33-year-old woman, complex partial seizures developed at age 7 years. She described an aura of nausea and an urge to spit repeatedly or go to the washroom. She would stare and on occasion had focal twitching of the left arm. In childhood she had two to three seizures a month, and as a teenager, she had secondarily generalized convulsions. As an adult, her seizures increased with menstrual periods and occurred up to 7 times a month with medications. She had tried phenytoin (PHT), phenobarbital (PB), valproic acid (VPA), carbamazepine (CBZ), vigabatrin (VGB), and clobazam (CLB) without effective control of seizures. On neurologic examination, she had a minor left facial droop and slightly brisker reflexes on the left side. Long-term video-EEG demonstrated interictal right temporal discharges and seizures with build-up of rhythmic activity over the right frontal temporal region at onset. Magnetic resonance imaging (MRI) was read at the time as normal, although in retrospect, it demonstrated typical features of a bilateral band heterotopia. Family history includes a mother with medically intractable epilepsy and an almost identical structural abnormality on MRI, two brothers who died in infancy with microcephaly, and a healthy brother and sister without seizures. Based on her seizure intractability and investigations demonstrating right temporal onset, she had a right anterior temporal lobectomy performed in 1998. In follow-up immediately after surgery, she was dramatically improved with only occasional brief episodes of hand numbness and no other seizures. The patient experienced a single postoperative generalized tonic–clonic seizure in 1999 after attempted withdrawal of medications. Five years after surgery, she was having only nocturnal complex partial seizures confined to the week before menstruation. Although genetic testing has not been performed, the classic features of an X-linked dominant pedigree in association with the typical MRI findings of band heterotopia in the patient and her mother are strongly suggestive of a mutation in the doublecortin gene located on chromosome Xq22.3-q23 (2–4). Although Bernasconi et al. (1) reported uniformly poor outcomes in six patients with double cortex syndrome who had focal resective surgery, our patient continues to demonstrate a meaningful, worthwhile improvement in seizure control (Engel class IID) (5) 5 years after right temporal resection. Russo et al. (6) also recently reported a good outcome in a patient with band heterotopia who underwent a focal temporal resection. We believe that although surgical resection in subcortical band heterotopia may have a low success rate as shown by Bernasconi et al. (1), one should not conclude that this procedure cannot yield adequate results, given that a good outcome in some individuals is possible. Subcortical band heterotopia should not preclude consideration for focal resective surgery in intractable epilepsy.

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