Drug-resistant epilepsy in children with hemophilia: how can we do it surgically?

BACKGROUND: Drug-resistant epilepsy can occur in patients with intracranial hemorrhage (ICH) caused by hemophilia, there is a paucity of literature reporting the surgical treatment of these patients because of the high risk of bleeding and comprehensive management such as factor replacement during the period of perioperation. METHODS: The data of 216 children with drug-resistant epilepsy who underwent surgically treatment in the Pediatric Epilepsy Center of the Capital Institute of Paediatrics were retrospectively reviewed. Seizure response and procedure complications were evaluated. Two cases children with hemophilia underwent surgical treatment at 29 months (case 1) and 6 years of age (case 2) were identified and followed up. RESULTS: Both children have achieved seizure free without complications such as bleeding or infection after 28 months (case 1) and 21 months (case 2) follow-up. CONCLUSION: For children with drug-resistant epilepsy associated with hemophilia, surgery that meets certain conditions can improve the prognosis safely and effectively.

Negative or positive imaging: ganglioglioma in a boy with epilepsy.

Ganglioglioma is a rare primary tumour of the central nervous system, which characteristically contain both neuronal and glial neoplastic components mainly in children and adolescents. The most common clinical presentation is refractory epilepsy. The imaging findings of ganglioglioma are obvious and varied. However, ganglioglioma with normal neuroimaging is rare. We report a 12-year-old boy presented with intractable focal epilepsy with normal CT and almost negative MRI. The epileptogenic focus was found to be located in the left posterior superior temporal gyrus by comprehensive evaluation including PET-CT imaging and stereo electroencephalography monitoring. The epileptogenic focus was resected, and the histological examination of the surgical specimen confirmed ganglioglioma. He was seizure-free at last follow-up 14 months after surgery.

Deep Brain Stimulation Electrode Reconstruction: Comparison between Lead-DBS and Surgical Planning System.

BACKGROUND: Electrode reconstruction for postoperative deep brain simulation (DBS) can be achieved manually using a surgical planning system such as Surgiplan, or in a semi-automated manner using software such as the Lead-DBS toolbox. However, the accuracy of Lead-DBS has not been thoroughly addressed. METHODS: In our study, we compared the DBS reconstruction results of Lead-DBS and Surgiplan. We included 26 patients (21 with Parkinson's disease and 5 with dystonia) who underwent subthalamic nucleus (STN)-DBS, and reconstructed the DBS electrodes using the Lead-DBS toolbox and Surgiplan. The electrode contact coordinates were compared between Lead-DBS and Surgiplan with postoperative CT and MRI. The relative positions of the electrode and STN were also compared between the methods. Finally, the optimal contact during follow-up was mapped onto the Lead-DBS reconstruction results to check for overlap between the contacts and the STN. RESULTS: We found significant differences in all axes between Lead-DBS and Surgiplan with postoperative CT, with the mean variance for the X, Y, and Z coordinates being -0.13, -1.16, and 0.59 mm, respectively. Y and Z coordinates showed significant differences between Lead-DBS and Surgiplan with either postoperative CT or MRI. However, no significant difference in the relative distance of the electrode and the STN was found between the methods. All optimal contacts were located in the STN, with 70% of them located within the dorsolateral region of the STN in the Lead-DBS results. CONCLUSIONS: Although significant differences in electrode coordinates existed between Lead-DBS and Surgiplan, our results suggest that the coordinate difference was around 1 mm, and Lead-DBS can capture the relative distance between the electrode and the DBS target, suggesting it is reasonably accurate for postoperative DBS reconstruction.

Efficacy and tolerability of lamotrigine in the treatment of focal epilepsy among children and adolescents: a meta-analysis.

BACKGROUND: Epilepsy is the most common chronic neurological disease in children, and focal epileptic seizures are the most common subtype. Unlike the data supporting treatment options for adults with epilepsy, evidence regarding the most effective first-line drug therapy for focal epilepsy in children and adolescents is limited. While lamotrigine is a therapeutic option for adults, there are disagreements surrounding its efficacy and tolerability in the younger population. Therefore, we performed a meta-analysis to determine if there was sufficient evidence to support a more definitive recommendation. METHODS: We undertook electronic search strategies using Medline via Ovid SP, Embase via Ovid SP up to February 05, 2021. We also searched relevant articles through Chinese BioMedical Literature (CBM), Chinese National Knowledge Infrastructure (CNKI), WANFANG, and VIP databases up to February 05, 2021. Study selection and data extraction were performed by 2 authors independently. The randomized controlled trials on focal epilepsy in children were included, and we made risk of bias judgments based on the methods endorsed by The Cochrane Collaboration. We used fifty percent or greater reduction in seizure frequency as an indicator of efficacy, the incidence of adverse events and treatment withdrawal as indicators of tolerability. The strength of the correlation was assessed via risk ratios (RRs) and their 95% confidence intervals (95% CIs). RESULTS: A total of 7 randomized trials involving 757 participants fulfilled the eligibility criteria. Of the 7 trials, 3 were placebo-controlled, and 4 compared lamotrigine with carbamazepine or oxcarbazepine. Lamotrigine was significantly more effective than placebo in achieving ≥50% reduction in seizure frequency, but its efficacy was not significantly different from that of carbamazepine or oxcarbazepine (lamotrigine vs. placebo: RR 2.95, 95% CI, 1.88 to 4.61; lamotrigine vs. carbamazepine/oxcarbazepine: RR 0.95, 95% CI, 0.85 to 1.05. There was significant difference in the incidence of overall adverse events between the lamotrigine- and carbamazepine/oxcarbazepine-treated groups (RR 0.64, 95% CI, 0.45 to 0.90). CONCLUSIONS: Lamotrigine was effective in reducing the seizure frequency when used as an add-on treatment in children with focal epilepsy, but current evidence does not suggest that lamotrigine is superior to carbamazepine/oxcarbazepine as monotherapy. For overall adverse events, lamotrigine has significantly fewer than carbamazepine/oxcarbazepine, suggesting that lamotrigine has better tolerability.

Primary anaplastic large cell lymphoma of the central nervous system in a child: A case report.

INTRODUCTION: To report the clinical characteristics of primary central nervous system T-cell lymphoma with anaplastic lymphoma kinase-1 (ALK-1) positive in an 8-year-old male. PATIENT CONCERNS: The patient presented cognitive impairment, dizziness, vomiting, fever, and convulsions during the disease, followed by progressive and persistent severe headache, progressive increase of intracranial pressure, indifference, disorder of consciousness, mild increase in white blood cells in cerebrospinal fluid, progressive decrease of sugar, progressive increase of protein, abnormal signal of left parietal-occipital, local meningeal enhancement, and cerebrospinal fluid cytology. DIAGNOSIS: He was diagnosed with ALK-1-positive central nervous system T-cell lymphoma. INTERVENTIONS: Meropenem and vancomycin were administered to counter the infection, while dexamethasone alleviated the inflammation. OUTCOMES: The patient died of cerebral hernia due to intracranial hypertension in the eighth week of the disease. CONCLUSIONS: PCNS ALK-1-positive anaplastic large cell lymphoma is extremely rare. Also, it is difficult to distinguish from central meningeal lymphoma and central nervous system infection, which might lead to delayed diagnosis. However, early diagnosis depends on the pathological diagnosis of brain tissue biopsy.