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Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach

Weiner, Howard L; Carlson, Chad; Ridgway, Emily B; Zaroff, Charles M; Miles, Daniel; LaJoie, Josiane; Devinsky, Orrin
OBJECTIVE: Tuberous sclerosis complex (TSC) is associated with medically refractory epilepsy and developmental delay in children and usually results from cortical tubers. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved when seizures are controlled at an early age. Previous reports have shown modest benefit from surgical resection of single tubers/seizure foci in older children; however, many children with TSC develop uncontrolled seizures before age 1. To identify patients who might benefit from surgery and to maximize outcome, we used a novel surgical approach in young children that consists of invasive intracranial monitoring, which is typically 3-staged and often bilateral. METHODS: Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the past 6 years, 25 patients (9 boys and 16 girls) had TSC. At the time of their first surgery at our institution, they were a median age of 4.0 years. A total of 31 separate admissions for epilepsy surgery in these 25 patients were identified. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively, and 22 patients underwent 3-stage surgeries. RESULTS: At 6 months or longer after the initial resection, 21 (84%) children were class I, 2 (8%) children were class II, and 2 (8%) children were class IV. At a mean follow-up of 28 months, 17 (68%) children were class I, 6 (24%) were class II, and 2 (8%) were class III. Four of the 5 children who initially were rejected as surgical candidates because of multifocality and who required initial bilateral electrode study are now seizure-free. CONCLUSIONS: This approach can help to identify both primary and secondary epileptogenic zones in young TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery. Long-term follow-up will determine whether this approach has durable effects
PMID: 16651302
ISSN: 1098-4275
CID: 64210

Pediatric epilepsy surgery: The recent NYU experience [Meeting Abstract]

Cahan, B; Bollo, RJ; LaJoie, J; Miles, D; Devinsky, O; Weiner, H
ISI:000236592500060
ISSN: 0022-3085
CID: 104254

Multi-staged epilepsy surgery in multifocal tuberous sclerosis complex [Meeting Abstract]

Carlson, C; Weiner, H; Ridgway, E; Zaroff, C; Miles, D; LaJoie, J; Kuzniecky, R; Devinsky, O
ISI:000236068104013
ISSN: 0028-3878
CID: 104258

Evolving concepts in pediatric epilepsy surgery: Tuberous sclerosis as paradigm [Meeting Abstract]

Weiner, HL; Miles, D; LaJoie, J; Devinsky, O
ISI:000231215100032
ISSN: 0022-3085
CID: 104260

Current treatments of epilepsy

Nadkarni, Siddhartha; LaJoie, Josiane; Devinsky, Orrin
Medical therapy is the mainstay for epilepsy, with most patients well controlled on a single antiepileptic drug (AED). In this non-refractory group, many patients have medication side effects and occasional seizures. Approximately 30% of patients with partial epilepsy and 25% of patients with generalized epilepsy are not well controlled on medications. These patients are often receiving multiple AEDs, with disabling seizures and side effects. Although second-generation AEDs are safer and better tolerated than the older AEDs, there are scant data to support significant advantages in efficacy. In VA studies with older AEDS, therapy with two AEDs improved seizure control in 40% of patients but seizure freedom was achieved in only 9%. A meta-analysis of the second-generation AEDs used as adjunctive therapies shows that 12% to 29% of patients had a 50% or greater reduction in seizure frequency. Surgery and the vagus nerve stimulator provide important therapeutic options in patients whose seizures are not controlled by AEDs. Special considerations about epilepsy care must be made in pediatric populations, those with developmental delays, women, and the elderly
PMID: 15994220
ISSN: 1526-632x
CID: 58984

Epilepsy surgery for children with tuberous sclerosis complex

Weiner, Howard L; Ferraris, Nina; LaJoie, Josiane; Miles, Daniel; Devinsky, Orrin
Tuberous sclerosis complex is associated with medically refractory seizures and developmental delay in children. These epilepsies are often resistant to antiepileptic drugs, can be quite severe, and usually have a negative impact on the child's neurologic and cognitive development. It is believed that functional outcome is improved if seizures can be controlled at an early age. The surgical treatment of intractable epilepsy in children and adults with tuberous sclerosis complex has gained significant interest in recent years. Previously published studies have shown a potential benefit from resection of single tubers, with most of the results noted in relatively older children. All of these reports support the idea that if a single primary epileptogenic tuber or region can be identified, then a surgical approach is appropriate. However, most children with tuberous sclerosis complex have multiple potentially epileptogenic tubers, rendering localization challenging, and they are therefore rejected as possible surgical candidates. We have used a novel surgical approach using invasive intracranial monitoring, which is typically multistaged and bilateral. This multistage surgical approach has been useful in identifying both primary and secondary epileptogenic zones in patients with tuberous sclerosis complex with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery in selected patients. Long-term follow-up will determine whether this approach has durable effects. We await better methods for identifying the epileptogenic zone, both noninvasive and invasive
PMID: 15563015
ISSN: 0883-0738
CID: 47796

Epilepsy surgery in young children with tuberous sclerosis [Meeting Abstract]

Weiner, HL; Lajoie, J; Miles, D; Devinsky, O
ISI:000220440900125
ISSN: 0022-3085
CID: 104265

Effects of seizures and their treatment on fetal brain

LaJoie, Josiane; Moshe, Solomon L
PURPOSE: To describe the effects of pregnancy on seizures, the effects of seizures during pregnancy on the fetus, and the effects of antiepileptic drugs (AEDs) on fetal brain and development. METHODS: The available literature was reviewed and summarized. RESULTS: There is a paucity of prospective studies. Retrospective studies indicate that, during pregnancy, alterations in seizure frequency can occur in an unpredictable fashion. Generalized tonic-clonic seizures may have adverse effects on the fetus. It is unclear whether complex partial seizures or absence seizures have negative consequences. AEDs may have potentially detrimental effects on the fetus and its subsequent development, but the full spectrum and clinical significance are under investigation. Monotherapy is strongly encouraged. CONCLUSIONS: Dealing with the pregnant epileptic patient is a difficult and challenging task. Although there are several risks for the mother and the fetus, most epileptic women bear normal, healthy children
PMID: 15610195
ISSN: 0013-9580
CID: 56087

Treatment of attention-deficit disorder, cerebral palsy, and mental retardation in epilepsy

LaJoie J; Miles DK
Epilepsy in childhood is often associated with other neurologic disorders, including attention-deficit/hyperactivity disorder, cerebral palsy, and mental retardation. A single pathologic process may explain both epilepsy and these associated disorders. However, in some cases, distinct etiologies may be present. Recognition of these problems is essential, as is individualized treatment. Proper classroom placement; behavior modification, speech, occupational, and physical therapies; pharmacological agents; and even surgical procedures have a role in the management of these comorbid disorders. Diagnostic criteria and therapeutic modalities used in these syndromes will be discussed
PMID: 12609321
ISSN: 1525-5050
CID: 34498