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Purpose The aim of the treatment of epileptic seizures is to achieve a seizure-free state without adverse effects. The mainstays of seizure treatment are anticonvulsant medication, diet therapy, and surgery. Antiepileptic drugs and a ketogenic diet are associated with various types of medical adverse effects, including chronic kidney disease (CKD). We aimed to identify the characteristics of pediatric epilepsy patients who developed CKD and to determine the possible mechanisms. Methods We included 816 patients who underwent medical treatment for epilepsy and were followed-up for at least 3 years at a tertiary hospital. The patients were divided into CKD and non-CKD groups. The data were assessed using a multivariate Cox proportional hazards model to identify the factors associated with CKD among patients undergoing epilepsy treatment. Results Initial high serum creatinine levels (hazard ratio [HR], 13.927; P=0.010), microscopic hematuria on initial urinalysis (HR, 10.047; P=0.001), developmental delay (HR, 11.929; P=0.000), and interictal epileptiform discharges on initial electroencephalography (generalized interictal epileptiform discharges: HR, 38.395, P=0.003; focal interictal epileptiform discharges: HR, 19.252, P=0.006) were associated with increased CKD risk. Conclusion CKD was more likely to develop in patients who presented with initial kidney dysfunction and developmental delay, and was related to epilepsy itself. The abovementioned factors may increase CKD risk through decreased brain function, which may lead to decreased activity and, hence, to relatively poor hygiene and voiding function. Moreover, patients with pre-existing kidney disease were more vulnerable to CKD development.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient’s clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas. [ABSTRACT FROM AUTHOR]

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program. [ABSTRACT FROM AUTHOR]

Purpose: Headache is a frequent neurological symptom in school aged individuals and recurrent headache has significant disabling effects among children and adolescents that manifest as school absenteeism, decreased extracurricular activities, and poor academic performance, as shown in previous studies. In Korea, there has not yet been a population-based study of headache-related disability in children and adolescents. We sought to estimate headache-related disability and investigate relevant predictors of disability due to headaches among schoolchildren in South Korea. Methods: This was a cross-sectional school-based study. We surveyed 5,039 (boys 2,405, girls 2,634) students aged 6-18 years. Among 1,465 students with headache, six hundred sixty-six schoolchildren (225 boys, 441 girls) completed all questionnaires. The questionnaires collected demographic data, in addition to headache specific questions consistent with International Classification of Headache Disorder criteria, 2nd edition. Disability was evaluated using the 6-question Pediatric Migraine Disability Assessment (PedMIDAS). Results: Six hundred sixty six school children and adolescents (225 boys, 441 girls) among 1,465 students with headaches completed all questionnaires. The percentage of headache sufferers with grade I disability was 88.6%. The mean (±standard deviation) PedMIDAS score was 5.11±11.17. There was a trend towards more severe disability in the older age groups, particularly among the 16 to18 year-olds. Students with migraine had the highest PedMIDAS scores (6.69±10.66) whereas students reporting other types of headache had the lowest scores (3.81±7.52). The predictors of headache-related disability were intensity (P=0.028), frequent headache (P=0.003), and longer duration of symptoms prior to presentation (P=0.008). Conclusion: A trend towards a more severe disability was observed in the older age group. Schoolchildren with migraine had the most headache-related disability. The predictors for headache-related disability were intensity, frequent headache, and longer duration of symptoms prior to presentation. [ABSTRACT FROM AUTHOR]

Valproic acid (VPA) is a widely used anticonvulsant drug having various adverse effect and drug interaction. In contrast, newer antiepileptic drug, lacosamide has been known to have minimal drug interaction. We report the second case of lacosamide-associated valproic acid toxicity presenting as hyperammonemia following febrile illness. Prior to hospital admission, this 13-year-old girl had been treated with multiple antiepileptic drugs including valproic acid for 11 years with no any adverse effect. Lacosamide had recently been added due to poorly controlled seizures. The patient presented with excessive drowsiness 18 days following an increase in lacosamide dosage up to 7.5 mg/kg/day. Laboratory tests revealed elevated levels of VPA(> 150 ug/ml) and hyperammonemia (232 N-μg/dL). Eight days prior to admission, the patient suffered a 1-day fever as a result of influenza A infection and did not take an antiviral agent. Lacosamide and valproic acid treatments were discontinued, while treatment with L-carnitine was added. After 4-days, ammonia levels decreased to 144 N-μg/dL, and the patient became alert. In conclusion, valproic acid toxicity may occur at any time during the stable course of medical treatment, though patients may be more susceptible following the addition of new anti-epileptic drugs or febrile illness. [ABSTRACT FROM AUTHOR]

The characteristic features of ring chromosome 14 syndrome are microcephaly, facial dysmorphism, ocular abnormalities, psychomotor retardation, and drugresistant epilepsy. Here, we report the case of a 2-year-old girl, diagnosed with ring 14 syndrome with mild clinical features. The child had microcephaly without facial dysmorphism, a slight language delay, and well-controlled seizures with a favorable progress. Her first seizure occurred at the age of 4 months and she has been seizurefree for 12 months since turning 3 years old. She exhibited only a mild language delay, which is still improving. It is important to note that ring chromosome 14 may manifest as early onset seizures with mild psychomotor delay, but without facial dysmorphism. These patients may follow a favorable clinical course. [ABSTRACT FROM AUTHOR]