Sang Ook Nam

Longitudinal Change of Vitamin D Status in Children With Epilepsy on Antiepileptic Drugs: Prevalence and Risk Factors

BACKGROUND Our aim was to evaluate the prevalence and risk factors of vitamin D deficiency and the changes of vitamin D level among children with epilepsy on antiepileptic drugs. METHODS The levels of serum 25-hydroxy vitamin D were measured at the start of antiepileptic drugs and at 6- to 12-month intervals in children with epilepsy taking antiepileptic drugs in Pusan National University Childrens Hospital. Vitamin D deficiency was defined as 25-hydroxy vitamin D levels <20 ng/mL and insufficiency between 21 and 29 ng/mL. RESULTS A total of 143 children (103 boys and 40 girls) with the mean age of 7.4 ± 5.4 years were included. The mean follow-up duration was 1.8 ± 0.8 years. At the start of antiepileptic drugs and the last follow-up, vitamin D deficiency or insufficiency was recognized in 56.6% (81 of 143) and 79.0% (113 of 143), respectively (P < 0.01). The mean value of initial 25-hydroxy vitamin D was 31.1 ± 14.7 ng/mL, which was significantly decreased to 20.2 ± 14.9 ng/mL (P < 0.01) in the last follow-up. Polytherapy (-16.0 ± 13.6 ng/mL), longer duration of ≥2 years (-23.5 ± 9.1 ng/mL), tube feeding (-18.2 ± 14.5 ng/mL), and overweight with body mass index of eighty-fifth percentile or greater (-17.0 ± 12.1 ng/mL) had a significant negative effect for the longitudinal change of 25-hydroxy vitamin D. Age, etiologies, seizure outcomes, and type of antiepileptic drugs (enzyme-inducing versus nonenzyme-inducing antiepileptic drugs) did not affect the longitudinal decrease of 25-hydroxy vitamin D. CONCLUSIONS A high proportion of these children on antiepileptic drugs had hypovitaminosis D and a significant decrease between the initial and the last follow-up. Polytherapy and longer duration of antiepileptic drugs, tube feeding, and overweight were independently associated with longitudinally significant decrease of 25-hydroxy vitamin D.

Optic Neuritis in Korean Children: Low Risk of Subsequent Multiple Sclerosis.

BACKGROUND A relationship between optic neuritis and multiple sclerosis has previously been reported in non-Asian adults. We extended the investigation to Korean children. OBJECTIVES We compared the clinical features, laboratory findings, and visual outcomes of optic neuritis between prepubertal children and postpubertal adolescents and evaluated the conversion rate of optic neuritis to multiple sclerosis in Korean children. METHODS We conducted a retrospective analysis of children less than 18 years of age presenting with optic neuritis at Pusan National University Hospital between January 2002 and December 2013. Outcomes and clinical, ophthalmologic, magnetic resonance imaging, and laboratory findings were reviewed. RESULTS Twenty-six children (male:female, 1:1.2) were included. Follow-up duration was 16.3 ± 27.5 months in the prepubertal children (≤10 years, n = 13) and 8.2 ± 9.2 months in postpubertal adolescent (>10 years, n = 13) (P = 0.32). There was no significant difference between the prepubertal group and postpubertal group in clinical, ophthalmologic, magnetic resonance imaging, or laboratory findings. Of two patients (7.7%) with abnormal brain magnetic resonance images, one developed multiple sclerosis and the other developed acute disseminated encephalomyelitis. Of three patients (11.5%) with relapsing optic neuritis, two developed systemic lupus erythematosus and one developed multiple sclerosis. CONCLUSION The risk of developing multiple sclerosis after pediatric optic neuritis was low (7.7%). Abnormal brain magnetic resonance imaging and relapsing optic neuritis should alert the clinician to systemic or neurological disorders.

Melkersson-Rosenthal Syndrome With Hashimoto Thyroiditis in a 9-Year-Old Girl: An Autoimmune Disorder

BACKGROUND Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown cause. The classical triad of MRS is orofacial edema, recurrent facial paralysis, and a fissured tongue. PATIENT We present a 9-year-old girl with a recurrent peripheral facial paralysis. She experienced the first episode of a peripheral facial paralysis on the same side without orofacial swelling and lingua plicata 1 year ago. She was diagnosed with Hashimoto thyroiditis 9 months earlier, as confirmed by an endocrinologic investigation. RESULTS While the patient was hospitalized with recurrent facial paralysis, we found that serum levels of free thyroxine (1.3 ng/dL) and thyrotropin (0.4 uIU/mL) were within normal range, but the level of antithyroperoxidase antibodies (772.0 IU/mL) was very increased. She had been taking an oral prednisolone orally for 2 weeks. At the 1-month follow-up, the patients symptoms had completely disappeared. DISCUSSION The possible correlation between MRS and autoimmune disorders has been documented in only one report, which described an adult with autoimmune thyroiditis (Hashimoto thyroiditis) and MRS. We suggest that the co-occurrence of MRS and Hashimoto thyroiditis is not coincidental but linked to autoimmunity.

Idiopathic intracranial hypertension associated with polycystic ovarian syndrome.

Idiopathic intracranial hypertension is defined as increased intracranial pressure of unknown origin. Idiopathic intracranial hypertension associated with polycystic ovarian syndrome (PCOS) is a rare condition in adolescence. We report the case of a 14‐year‐old girl with sudden onset of decreased visual acuity, headache and menstrual irregularity. Clinical neurological examination and magnetic resonance imaging of the brain and orbit were normal. Lumbar puncture demonstrated an increased opening pressure of 31 cmH2O. Gynecologic investigation indicated PCOS. Her symptoms improved with medical and surgical treatment for the underlying PCOS.

Venous angioma may be associated with epilepsy in children

Purpose Venous angioma (VA) is the most common congenital abnormality of the intracranial vasculature. This study aimed to investigate the relationship between VA and epilepsy and to identify the characteristics of children with VA and epilepsy. Methods The records of all patients aged less than 18 years who underwent brain magnetic resonance imaging (MRI) at Pusan National University Hospital were retrospectively reviewed. Patients with isolated VA and patients with normal MRI were compared in terms of the prevalence of epilepsy. Results In total, 2,385 pediatric patients who underwent brain MRI were enrolled. Isolated VA was identified in 26 patients (VA group). Among the patients with normal MRI findings, 225 age- and sex-matched patients to the VA-group were assigned to the control group. Nine patients in the VA group (9 of 26, 34.6%) and 27 patients in the control group (26 of 225, 11.5%; P<0.001) had epilepsy. In the VA group, 20 patients (76.9%) had the VA in the cerebral hemispheres, and 6 patients (23.1%) had the VA in the brainstem and cerebellum. The latter showed a higher prevalence of epilepsy (5 of 6, 83.3%) than the former (4 of 20, 20.0%; P=0.004). Among the nine patients who had epilepsy with VA, patients whose VA involved the brainstem and cerebellum showed a significantly higher frequency of abnormal Electroencephalographic findings than patients whose VA involved the cerebral hemispheres (P=0.016). Conclusion VA, especially in the brainstem and cerebellum, might be associated with epilepsy.

Endovascular stenting of tracheoinnominate fistula after tracheostomy in a 14-year-old boy.

Tracheoinnominate artery fistula is a rare, fatal complication of tracheostomy, and prompt diagnosis and management are imperative. We report the case of tracheoinnominate artery fistula after tracheostomy in a 14-year-old boy with a history of severe periventricular leukomalacia, hydrocephalus, cerebral palsy, and epilepsy. The tracheoinnominate artery fistula was successfully treated with a stent graft insertion via the right common femoral artery. Endovascular repair of the tracheoinnominate artery fistula via stent grafting is a safe, effective, and minimally invasive treatment for patients in poor clinical conditions and is an alternative to traditional open surgical treatment.

Cerebrospinal fluid non-pleocytosis in pediatric enteroviral meningitis: Large-scale review

Lack of cerebrospinal fluid (CSF) pleocytosis has been reported in some children with enteroviral meningitis (EVM). The aim of this paper was to investigate the clinical spectrum and related factors in EVM with CSF non‐pleocytosis.

Comparison of conservative therapy and steroid therapy for Bell’s palsy in children

Purpose Bell’s palsy is characterized by sudden onset of unilateral facial weakness. The use of corticosteroids for childhood Bell’s palsy is controversial. This study aimed to identify clinical characteristics, etiology, and laboratory findings in childhood Bell’s palsy, and to evaluate the efficacy of corticosteroid treatment. Methods We conducted a retrospective analysis of children under 19 years of age treated for Bell’s palsy between January 2009 and June 2017, and followed up for over 1 month. Clinical characteristics, neuroimaging data, laboratory findings, treatments, and outcomes were reviewed. Patients with Bell’s palsy were divided into groups with (group 1) and without (group 2) corticosteroid treatment. Differences in onset age, sex, laterality, infection and vaccination history, degree of facial nerve palsy, and prognosis after treatment between the groups were analyzed. Results One hundred patients were included. Mean age at presentation was 7.4±5.62 years. A total of 73 patients (73%) received corticosteroids with or without intravenous antiviral agents, and 27 (27%) received only supportive treatment. There was no significant difference in the severity, laboratory findings, or neuroimaging findings between the groups. Significant improvement was observed in 68 (93.2%) and 26 patients (96.3%) in groups 1 and 2, respectively; this rate was not significantly different between the groups (P=0.48). Conclusion Childhood Bell’s palsy showed good prognosis with or without corticosteroid treatment; there was no difference in prognosis between treated and untreated groups. Steroid therapy in childhood Bell’s palsy may not significantly improve outcomes.

Comparison of Cerebrospinal Fluid Cytokine Levels in Children of Enteroviral Meningitis With Versus Without Pleocytosis

In viral meningitis, proinflammatory cytokines were detected at higher levels in the cerebrospinal fluid (CSF) and might play an important role in the inflammatory process. Our goal was to compare the cytokine profiles in the CSF of children of enteroviral meningitis (EVM) with versus without CSF pleocytosis. In total, 158 patients were enrolled in this prospective cohort study and were classified as EVM (group-A, n = 101), nonenteroviral aseptic meningitis (group-B, n = 27), and control (group-C, n = 30) groups. Of the 101 children with EVM, 71 had CSF pleocytosis (group-A1) and 30 had CSF nonpleocytosis (group-A2). Fifteen cytokines/chemokines in the CSF were measured simultaneously by immunoassay. Significant differences were found in interleukin (IL)-2, IL-6, and IL-8 levels in the CSF across the 3 groups, with the highest levels in group-A, followed by group-B and group-C. The levels of IL-1β, IL-2, IL-6, IL8, IL-10, interferon-γ, and tumor necrosis factor-α were significantly higher in the CSF of group-A1 than in that of group-A2. Group-A2 was significantly younger than group-A1 (3.4 ± 2.8 years versus 5.5 ± 3.2 years, P = 0.016). Significant differences between CSF pleocytosis and nonpleocytosis in EVM appear to be associated with distinct levels of CSF cytokines.

Longitudinal Change in Thyroid Hormone Levels in Children with Epilepsy on a Ketogenic Diet: Prevalence and Risk Factors

Background and Purpose The aim of this study is to evaluate the prevalence of hypothyroidism and the change of thyroid hormone level in the children with epilepsy on a ketogenic diet (KD). Methods The levels of serum free thyroxine (fT4) and thyroid-stimulation hormone (TSH) were measured at the start of the KD and at 6- to 12-month intervals in children with intractable epilepsy. Hypothyroidism was defined as fT4 level < 0.8 ng/dL and TSH level > 6.0 μIU/mL. Results A total of 28 children (17 boys and 11 girls) were enrolled in the study. The mean age of onset of seizure was 1.4 ± 1.6 years, the mean age of the start of the KD was 3.2 ± 2.4 years, and the mean duration of KD was 1.9 ± 1.5 years. Overall, there was no significant longitudinal change in the mean fT4 (0.99 ± 0.25 vs. 0.94 ± 0.71 ng/dL, p = 0.28) and TSH (2.94 ± 1.32 vs. 3.18 ± 1.21 μIU/mL, p = 0.44) levels from the start of the KD to last follow-up. The patients with a younger age of seizure onset, earlier initiation of KD, and higher serum levels of cholesterol and triglyceride had a significant decrease in fT4 levels and increase in TSH levels during the KD. Sex, duration of the seizure or KD therapy, seizure types, seizure frequency, seizure outcomes, brain lesion, ratio of KD, and being overweight did not affect the longitudinal change of fT4 and TSH levels during KD. Conclusion Thyroid function had no significant longitudinal decrease in pediatric epilepsy during KD therapy. However, careful monitoring of the serum levels of fT4/TSH should be recommended in children on KDs, especially in those with earlier seizure onset, earlier start of KD, and higher levels of lipid profiles.