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Didier Scavarda, Philippe Major, Anne Lortie, Claude Mercier and Lionel Carmant

Object

Ischemic cerebral vascular accidents (CVAs) in children result in epilepsy in 25% of patients, which is refractory in 7% of cases. Repeated seizures worsen the global and cognitive prognosis of these patients. To evaluate the prognosis of epilepsy and cognitive development in children with refractory seizures following a CVA, the authors retrospectively studied the effectiveness of periinsular hemispherotomy in the treatment of these patients.

Methods

Between March 1995 and November 2007, 8 children who suffered from stroke-induced refractory epilepsy underwent a periinsular hemispherotomy. All patients' charts were reviewed in a retrospective manner. Age at the time of the CVA, imaging studies, cause of the ischemic event, onset of the first seizure, patient's handedness, the extent of the parenchymal damage, electroencephalography findings, type of epileptic seizures, number of seizures per day, number of antiepileptic medications, preoperative neuropsychological evaluation, and surgical outcome with regard to the patient's seizure activity were analyzed.

Results

There were 7 boys and 1 girl in this study. The mean age at stroke was 23 months (range birth–5 years). The mean age at onset of epilepsy was 22 months (range 0–60 months). The mean age at the time of the hemispherotomy was 7 years (range 54–130 months). The average delay prior to the hemispherotomy was 5 years and 3 months (range 23–115 months). Prior to surgery, the average number of seizures per day was 35 (range 5–100). The average number of antiepileptic medications introduced before the hemispherotomy was 8 (range 6–12). Six patients required only 1 surgical intervention and 2 necessitated 2 separate operations:1 underwent a 2-staged hemispherotomy and the other underwent a prior callosotomy. There were no reported surgical complications in this series. Seven children are seizure free. However, the remaining child, after a 3.5-year disease-free interval, has recently started having seizures. No child demonstrated an improvement in neuropsychological evaluation.

Conclusions

The periinsular hemispherotomy must be considered an alternative in the therapeutic approach to stroke-induced pediatric refractory epilepsy. It is effective in controlling seizure activity. The authors believe the delay before hemispherotomy must be shortened in children with post-CVA refractory epilepsy.

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Simona Mihaela Florea, Alice Faure, Hervé Brunel, Nadine Girard and Didier Scavarda

The embryological development of the central nervous system takes place during the neurulation process, which includes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD.

This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5–S1 level, with a thickened filum terminale.

The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale.

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Didier Scavarda, Tiago Cavalcante, Agnès Trébuchon, Anne Lépine, Nathalie Villeneuve, Nadine Girard, Aileen McGonigal, Mathieu Milh and Fabrice Bartolomei

OBJECTIVE

Hemispherotomy is currently the most frequently performed surgical option for refractory epilepsy associated with large perinatal or childhood ischemic events. Such an approach may lead to good seizure control, but it has inherent functional consequences linked to the disconnection of functional cortices. The authors report on 6 consecutive patients who presented with severe epilepsy associated with hemiplegia due to stroke and who benefitted from a new, stereoelectroencephalography-guided partial disconnection technique.

METHODS

The authors developed a new disconnection technique termed “tailored suprainsular partial hemispherotomy” (TSIPH). Disconnection always included premotor and motor cortex with variable anterior and posterior extent.

RESULTS

At a mean follow-up of 28 months, there were no deaths and no patient had hydrocephalus. Motor degradation was observed in all patients in the 2 weeks after surgery, but all patients completely recovered. The 6 patients were seizure free (Engel class IA) at the last follow-up. No neuropsychological aggravation was observed.

CONCLUSIONS

TSIPH appears to be a conservative alternative to classic hemispherotomy, leading to favorable outcome in this series.