Hemispheric Surgery in Children with Refractory Epilepsy: Seizure Outcome, Complications, and Adaptive Function

Basheer, Sheikh Nigel; Connolly, Mary; Lautzenhiser, Aaron; Sherman, Elisabeth M. S.; Hendson, Glenda; Steinbok, Paul

doi:10.1111/j.1528-1167.2006.00909.x

Cited by 157 publications

(152 citation statements)

References 31 publications

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“…10 Hemispherectomy outcomes are usually good, with up to 100% of patients becoming seizure-free after the procedure. [3][4] Some authors have reported periods of seizure freedom as long as 17 years post-hemispherectomy in patients with SWS 11 . However these patients did not have recurrence of seizures for as long as they were followed.…”

Section: Discussionmentioning

confidence: 99%

“…Some form of hemispherectomy is usually performed for SWS-associated intractable epilepsy and most patients become seizure-free after surgery. [3][4][5] Another possible benefit of hemispherectomy is a better cognitive profile in patients that became seizure-free after such a procedure. However, very long follow-ups addressing seizure control and cognitive deficits in hemispherectomized patients are rare and they usually show good outcomes.…”

mentioning

confidence: 99%

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Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Andrade

McAndrews

Hamani

et al. 2010

Can. j. neurol. sci.

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Sturge-Weber syndrome (SWS) is a rare congenital disease associated with venous angiomatosis of the leptomeninges and ipsilateral, cutaneous facial port-wine stain (facial angiomatosis). Seizures are seen in more than 80% of cases, cognitive impairment in 50% and frequently glaucoma. 1-2 Seizures are the result of leptomeningeal angioma and underlying calcifications. These abnormalities are usually considered to be limited to one hemisphere. Frequently, seizures in SWS are refractory to medical treatment. In those cases surgical resection of the affected areas is recommended. Some form of hemispherectomy is usually performed for SWS-associated intractable epilepsy and most patients become seizure-free after surgery. 3-5 Another possible benefit of hemispherectomy is a better cognitive profile in patients that became seizure-free after such a procedure. However, very long follow-ups addressing seizure control and cognitive deficits in hemispherectomized patients are rare and they usually show good outcomes. Here we report on a SWS patient who become seizure-free after hemispherectomy performed in the first year of life, however, seizures recurred 29 years later without any identifiable cause. She also had cognitive decline before the onset of seizures. To the best of our knowledge this is the longest seizure-free interval between hemispherectomy and seizure recurrence in SWS.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Andrade

McAndrews

Hamani

et al. 2010

Can. j. neurol. sci.

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“…5,18,19 Hemispherotomy certainly circumvents the problem of widespread epileptogenic networks in the setting of early vascular injury and is one of the most effective forms of epilepsy surgery across all etiologies, with seizure freedom rates as high as more than 90%. 1,35,36 Hemispheric disconnection/anatomical resection has been the mainstay of surgical therapy for large perinatal porencephalic cysts after MCA stroke. 18,34,35 In this subgroup, outstanding seizure control rates with Engel Class IA outcomes in almost 90% of patients have been reported.…”

Section: Discussionmentioning

confidence: 99%

Surgical management of medically refractory epilepsy due to early childhood stroke

Ghatan

McGoldrick

Palmese

et al. 2014

PED

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Object The risk of developing epilepsy after perinatal stroke, hypoxic/ischemic injury, and intracerebral hemorrhage is significant, and seizures may become medically refractory in approximately 25% of these patients. Surgical management can be difficult due to multilobar or bilateral cortical injury, nonfocal or poorly lateralizing video electroencephalography (EEG) findings, and limited functional reserve. In this study the authors describe the surgical approaches, seizure outcomes, and complications in patients with epilepsy due to vascular etiologies in the perinatal period and early infancy. Methods The records were analyzed of 19 consecutive children and adults with medically refractory epilepsy and evidence of perinatal arterial branch occlusions, hypoxic/ischemic insult, or hemorrhagic strokes, who underwent surgery at the Comprehensive Epilepsy Center of Beth Israel Medical Center and St. Luke's-Roosevelt Hospital Center. Preoperative findings including MRI, video EEG, functional MRI, and neuropsychological testing were analyzed. The majority of patients underwent staged operations with invasive mapping, and all patients had either extra- or intraoperative functional mapping. Results In 7 patients with large porencephalic cysts due to major arterial branch occlusions, periinsular functional hemispherotomy was performed in 4 children, and in 3 patients, multilobar resections/disconnections were performed, with 1 patient undergoing additional resections 3 years after initial surgery due to recurrence of seizures. All of these patients have been seizure free (Engel Class IA) after a mean 4.5-year follow-up (range 15–77 months). Another 8 patients had intervascular border-zone ischemic infarcts and encephalomalacia, and in this cohort 2 hemispherotomies, 5 multilobar resections/disconnections, and 1 focal cortical resection were performed. Seven of these patients remain seizure free (Engel Class IA) after a mean 4.5-year follow-up (range 9–94 months), and 1 patient suffered a single seizure after 2.5 years of seizure freedom (Engel Class IB, 33-month follow-up). In the final 4 patients with vascular malformation-associated hemorrhagic or ischemic infarction in the perinatal period, a hemispherotomy was performed in 1 case, multilobar resections in 2 cases, and in 1 patient a partial temporal lobectomy was performed, followed 6 months later by a complete temporal and occipital lobectomy due to ongoing seizures. All of these patients have had seizure freedom (Engel Class IA) with a mean follow-up of 4.5 years (range 10–80 months). Complications included transient monoparesis or hemiparesis in 3 patients, transient mutism in 1 patient, infection in 1 patient, and a single case of permanent distal lower-extremity weakness. Transient mood disorders (depression and anxiety) were observed in 2 patients and required medical/therapeutic intervention. Conclusions Epilepsy surgery is effective in controlling medically intractable seizures after perinatal vascular insults. Seizure foci tend to be widespread and rarely limited to the area of injury identified through neuroimaging, with invasive monitoring directing multilobar resections in many cases. Long-term functional outcomes have been good in these patients, with significant improvements in independence, quality of life, cognitive development, and motor skills, despite transient postoperative monoparesis or hemiparesis and occasional mood disorders.

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“…Hemidisconnection provides a 63 to 85 percent seizure-freedom rate (1). Complication rates range from 24 to 41 percent, depending on the definition (12)(13)(14). Another potential benefit from surgery is that it frequently decreases medication burden and resulting side effects.…”

Section: Surgical Treatmentmentioning

confidence: 99%

Timing of Surgery in Rasmussen Syndrome: Is Patience a Virtue?

Hartman

Cross²

2014

Epilepsy Curr

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Rasmussen syndrome affects previously normal people and forever changes their lives and the lives of their families. Although understood as a probable autoimmune condition, medical treatment remains limited and surgery remains the only cure, although with inevitable functional consequences. Difficulties remain in deciding on the optimal timing of surgery. Here, we review data available to aid clinicians faced with making the decision of when to recommend hemispherectomy. Not all patients have rapidly progressive disease, however, and such patients may benefit from immunomodulatory treatment. Thus, a patient's clinical course requires careful evaluation in order to identify those who would benefit most from early surgery.

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Hemispheric Surgery in Children with Refractory Epilepsy: Seizure Outcome, Complications, and Adaptive Function

Cited by 157 publications

References 31 publications

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Surgical management of medically refractory epilepsy due to early childhood stroke

Timing of Surgery in Rasmussen Syndrome: Is Patience a Virtue?

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