Special Considerations in the Management of Women with Epilepsy in Reproductive Years

Anti-seizure medications (ASMs) fail to prevent seizure recurrence in more than 30% of patients with epilepsy. The treatment is more difficult in premenopausal women with epilepsy (WWE) because changes in plasma estrogen and progesterone concentrations during the menstrual cycle often affect seizure frequency and intensity. Interactions between enzyme-inducin ASMs and hormonal contraceptives can lead to both a loss of seizure control and failure of contraception. Significant changes in the function of the liver and kidneys during pregnancy can accelerate metabolism and elimination of ASMs, causing breakthrough seizures. In addition, the teratogenic, cognitive, and psychological effects of ASMs on potential offspring have to be considered when choosing the best ASM regimen. Therefore, aspecialized approach is necessary for the treatment of premenopausal WWE.

Cognitive Effects of Lacosamide in Patients with Drug Refractory Focal Epilepsy: A Prospective Observational Study

Objectives: This study was conducted to obtain data on the cognitive effects of lacosamide in Indian population. Methodology: An open labelled prospective observational study in 22 patients who suffered from focal epilepsy. Results: All the pre and post lacosamide cognition scores showed statistically significant positive correlation in this study. Average initial seizure frequency per month was 3.56 (SD 2.58) and median frequency 2.5 seizures per month. Range being 1-8 per month. At the final follow-up at 6months, 87.5% of the study subjects had no seizures. In the remaining12.5% of patients, reduction in seizure frequency was observed. The difference in frequency is statistically significant (Wilcoxon Signed Ranks TestP <0.001). Conclusion: Excellent seizure control is observed in patients with refractory focal epilepsy treated with lacosamide. Also, lacosamide has no serious adverse effects or drug interactions. In this study, it is observed that unlike many AEDs, lacosamide contributed to significant improvement in cognition.

Case Report: Hemispherotomy in the First Days of Life to Treat Drug-Resistant Lesional Epilepsy

Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults such as stroke, ischemia, hemorrhage, and/or genetic defects. Rapid seizure control is particularly important for cognitive development. Since early surgical intervention and thus a short duration of epilepsy should lead to an optimal developmental outcome, we present our experience with hemispherotomy in an infant at the corrected age of 1 week.Methods: We report successful hemispherotomy for drug-resistant epilepsy in an infant with hemimegalencephaly at a corrected age of 1 week.Results: The infant was diagnosed with drug-resistant lesional epilepsy due to hemimegalencephaly affecting the left hemisphere. Given congruent electroclinical findings, we performed a left vertical parasagittal transventricular hemispherotomy after critical interdisciplinary discussion. No complications occurred during the surgery. Intraoperatively; 118 ml of red blood cells (30 ml/kg) and 80 ml of plasma were transfused. The patient has been seizure-free since discharge without further neurological deficits.Conclusion: We demonstrate that early epilepsy surgery is a safe procedure in very young infants if performed in a specialized center experienced with age-specific surgical conditions and perioperative management. The specific surgical difficulties should be weighed against the risk of life-long developmental drawbacks of ongoing detrimental epilepsy.

Phacomatosis pigmentovascularis with sturge-weber syndrome and congenital glaucoma: A rare case report

Phacomatosis pigmentovascularis (PPV) is a rare congenital disease characterized by the co-existence of cutaneous vascular malformation and pigmentary nevi with or without extracutaneous systemic involvement. Here, we present a 2-month old child diagnosed with phacomatosis cesioflammea type of PPV with Sturge-Weber syndrome and secondary congenital glaucoma of the left eye. She underwent combined trabeculotomy and trabeculectomy in the left eye for glaucoma and was started on anti-epileptics for seizure control following pediatric evaluation. Early screening and treatment initiation can prevent blindness and other systemic complications associated with PPV.

Epilepsy and pregnancy: identifying risks

Pregnancy is a time of physical, physiological and psychological challenge. For women with epilepsy, as well as its potential for joy and fulfilment, pregnancy may bring additional risks and difficulties. Clinicians must anticipate and prevent these complications, ensuring that pregnancy, delivery and motherhood proceed without obstetric or medical complications, using available evidence to balance individual risks of undertreatment and overtreatment. Here we review epilepsy management in pregnancy, identifying some of the known effects of epilepsy and its treatment on gestation, fetal malformation, delivery, and neurocognitive and behavioural development. We outline strategies to reduce obstetric and fetal complications in women with epilepsy, while recognising the sometimes competing need to maintain or improve seizure control. We reinforce the importance of identifying those at highest risk, who may require additional measures or safeguards.

Febrile seizures: perceptions and knowledge of parents of affected and unaffected children

Febrile seizures (FS) in children are common, but little is known about parents’ perceptions and knowledge of FS. We interviewed parents of children aged 6 months to 6 years affected by FS (FS group, 65 parents) or unaffected (control group, 54 parents). In the FS group, 32% said they knew their child had an FS when the first event occurred, and 89% described fear when the child had a seizure, with a median intensity of 10/10 (Q25/Q75: 9/10). Related to follow-up, 77% in the FS group (will) observe their child more carefully after the first seizure happened, and 63% (will) give antipyretics earlier at a median temperature of 38.2 °C (100.8 °F). In the FS group, 62% were unaware of FS before the first event (54% of control group did not know about FS thus far, n.s.). In the FS group, 20% would put a solid object in the mouth of a child having a seizure (control group, 39%, p = 0.030), and 92% would administer an available anti-seizure rescue medication (control group, 78%, p = 0.019). In the FS group, 71% feared that children with FS might suffocate (control group, 70%, n.s.).Conclusion: Information about FS and their management should be more available to improve parents’ coping and patient safety. What is Known:• Febrile seizures in children are common.• The prognosis of children suffering from febrile seizures is usually rather good. What is New:• Over half of parents had not informed themselves about febrile seizures so far; and only 32% of parents realized their child had a febrile seizure when it occurred.• Most parents described own fear with a median intensity of 10/10; and 63% (will) give antipyretics earlier at a median temperature of 38.2 °C (100.8 °F).

Epilepsy of Infancy With Migrating Focal Seizures (EIMFS): Expansion of Clinical Phenotypic And Genotypic Spectra

EIMFS is a rare early infantile epileptic encephalopathy with unknown etiology and poor prognosis. This study included 36 patients who were diagnosed with EIMFS. 17/36 cases had causative variants across 11 genes, including 6 novel EIMFS genes: PCDH19, ALDH7A1, DOCK6, PRRT2, ALG1 and ATP7A. 13/36 patients had ineffective seizure control, 14/36 patients had severe retardation and 6/36 patients died. Of them, the genes for ineffective seizure control, severe retardation or death include KCNT1, SCN2A, SCN1A, ALG1, ATP7A and WWOX. 17 patients had abnormal MRI, of which 8 had ineffective seizure control, 7 had severe retardation and 4 died. 13 patients had hypsarrhythmia, of which 6 had ineffective seizure control, 6 had severe retardation and 2 died. Also, 7 patients had burst suppression, of which 1 had ineffective seizure control, 3 had severe retardation and 3 died. This study is the first to report that ALDH7A1, ATP7A, DOCK6, PRRT2, ALG1, and PCDH19 mutations cause the phenotypic spectrum of EIMFS to expand the genotypic spectrum. The genes KCNT1, SCN2A, SCN1A, ALG1, ATP7A and WWOX may be associated with poor prognosis. The patients presenting with MRI abnormalities, hypsarrhythmia and burst suppression in EEG may be associated with poor prognosis.

Successful Use of Inhalational Anesthesia and Electroconvulsive Therapy in a Child with New Onset Prolonged Super-Refractory Status Epilepticus

The treatment of super-refractory status epilepticus (SRSE) and prolonged SRSE rests on urgent seizure control to minimize excitotoxic cerebral damage, other forms of neurologic damage, and multiple medical complications. To date no randomized controlled trials or clear-cut guidelines are available for the management of SRSE. We reported the case of a 10-year-old previously healthy male child patient who presented with a febrile illness and new onset prolonged SRSE that became refractory to multiple antiseizure medications (ASMs). Coma induction with anesthetic agents, 14 ASMs, ketogenic diet, immunotherapy failed to completely control the SRSE in our patient. On day 22, clinical and electroencephalographic seizure control was achieved with isoflurane inhalation anesthesia, which was continued for 3 weeks but was unable to be weaned. From day 57 onwards, electroconvulsive therapy was administered (total 14 sessions that resulted in complete control of seizures). He was discharged on the 80th day.