Gyu Min Yeon

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.

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.

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 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.

Three children of meningoencephalitis with Kikuchi necrotizing lymphadenitis

BACKGROUND Kikuchi necrotizing lymphadenitis (KNL) is a rare and benign cause of lymphadenopathy, most often cervical. The etiology of KNL remains unknown. Central nervous system (CNS) involvement, such as in meningoencephalitis, is a very rare clinical manifestation of KNL, especially in children. CASE REPORTS A 12-year-old boy presented with unilateral cervical lymphadenopathy and fever. Histopathological findings led to the diagnosis of KNL. He revisited due to severe headache and vomiting one week later. Cerebrospinal fluid (CSF) analysis demonstrated pleocytosis (lymphocytic 57%), high protein (312 mg/dL) and low CSF/serum glucose ratio (52/121 mg/dL.) The next day, he had a seizure. Brain MRI revealed increased signal involving posterior area of both hemisphere. Another 17-year-old boy presented with headache for 7 days and behavioral and personality changes. He had a history of cervical lymphadenopathy two weeks ago. CSF analysis demonstrated lymphocytosis, high protein and low glucose ratio. MRI revealed the involvement of right cerebellum and posterior brainstem. A biopsy of one cervical lymphadenopathy demonstrated the findings of KNL. A 15-year-old girl presented with fever, headache, and cervical pain lasting 10 days. CSF analysis demonstrated pleocytosis (lymphocytic 95%), high protein and low CSF/serum glucose ratio. Histopathological findings of lymph node were suggestive of KNL. Above three patients were undertaken the steroid therapy and recovered fully without neurological dysfunction. CONCLUSIONS Recognition of CNS involvement in KNL may help evaluate the patients of acute meningitis/encephalitis with regional lymphadenopathy, thereby avoiding unnecessary treatment.

Relationship between initial electroencephalographic characteristics and seizure outcomes in children with non-lesional West syndrome

BACKGROUND To characterize the initial interictal electroencephalography (EEG) activities associated with seizure outcomes in children with non-lesional West syndrome (WS), and their worth in the prediction of seizure-free (SF) vs no seizure-free (No-SF) outcomes. METHODS We retrospectively reviewed the initial scalp EEGs for at least a 40-min duration, and the medical records of 66 children who were diagnosed as WS with normal MRI, and who were followed-up with for 4.5 ± 2.1 years. We assessed the following clinical and EEG findings: onset of seizures, development, underlying etiologies, initial interictal EEGs, and seizure evolution. These variables were compared between two groups: SF vs No-SF groups. RESULTS In total, 36 (54.5%) children had SF outcomes and 30 (45.5%) had No-SF outcomes during long-term follow-up (4.4 ± 2.3 vs 4.6 ± 2.0 years, p = 0.7644). The mean age at seizure onset was similar in the SF and No-SF groups (6.0 ± 3.0 vs 6.9 ± 3.2 months, p = 0.2443). Delayed development before the onset of spasms was similarly observed in both groups (13.9% vs 13.3%). Initial EEG findings significantly differed with typical hypsarrhythmia (41.7% vs 73.3%, p = 0.0098), multiple independent spike foci (MISF) (55.6% vs 83.3%, p = 0.0158), frontal-dominant MISF (0.0% vs 40.0%, p < 0.0001), and frontal-dominant generalized epileptiform discharges (EDs) (0.0% vs 16.7%, p=0.0108) being involved more infrequently in the SF group than in the No-SF group, respectively. Patients in the SF group showed no frontal-dominant MISF or frontal-dominant generalized EDs, and a more often normal to borderline sleep-spindle (83.3% vs 40.0%, p = 0.0002) than the No-SF group. CONCLUSION Patients with SF outcomes more frequently showed the posterior-dominant generalized EDs and normal to borderline sleep-spindle, and the No-SF group more often had typical hypsarrhythmia, frontal-dominant MISF, frontal-dominant generalized EDs, and no normal sleep-spindle. Initial interictal EEG findings may predict seizure outcomes in patients with non-lesional WS.