SUMMARYObjective: We aimed to compare the efficacy, safety, and tolerability of a modified Atkins diet (MAD) with the classic ketogenic diet (KD) for the treatment of intractable childhood epilepsy. Methods: From March 2011 to March 2014, 104 patients aged 1-18 years who had refractory epilepsy were randomly assigned to each diet group (ClinicalTrials.gov, number NCT2100501). A seizure diary record was used to compare seizure frequencies with the baseline prediet seizure frequency at the third and sixth months after diet therapy initiation. Results: Fifty-one patients were assigned to the KD and 53 patients to the MAD. The KD group had a lower mean percentage of baseline seizures compared with the MAD group at 3 months (38.6% for KD, 47.9% for MAD) and 6 months (33.8% for KD, 44.6% for MAD), but the differences were not statistically significant (95% confidence interval [CI] 24.1-50.8, p = 0.291 for 3 months; 95% CI 17.8-46.1, p = 0.255 for 6 months). Instead, for patients aged 1-2 years, seizure outcomes were consistently much more favorable in patients consuming the KD compared with those consuming the MAD. The rate of seizure freedom at 3 months after diet therapy initiation was significantly higher (53% for KD, 20% for MAD, p = 0.047) in these patients. The MAD had advantages with respect to better tolerability and fewer serious side effects. Significance: The MAD might be considered as the primary choice for the treatment of intractable epilepsy in children, but the classic KD is more suitable as the first line of diet therapy in patients <2 years of age.
PurposeThe aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea.MethodsWe retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric Neurology at Severance Children's Hospital from March 2005 to March 2011.ResultsFourteen (9 males and 5 females) subjects (82.3%) were diagnosed with epilepsy in AS. The most common seizure types were generalized tonic-clonic (n=9, 27%) and myoclonic (n=9, 27%), followed by atonic (n=8, 24%), atypical absence (n=4, 12%) and complex partial seizure (n=3, 9%). The most commonly prescribed antiepileptic drug (AED) was valproic acid (VPA, n=12, 86%), followed by lamotrigine (LTG, n=9, 64%), and topiramate (n=8, 57%). According to questionnaires that determined whether each AED was efficacious or not, VPA had the highest response rate and LTG was associated with the highest rate of seizure exacerbation. Complete control of seizures was achieved in 6 patients. Partial control was achieved in 7 patients, while one patient was not controlled.ConclusionEpilepsy is observed in the great majority of AS patients. It may have early onset and is often refractory to treatment. There are few reports about epilepsy in AS in Korea. This study will be helpful in understanding epilepsy in AS in Korea.
PurposeThe purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety.MethodsA retrospective chart review was conducted of 720 children aged 7-17 years who had visited a pediatric neurology clinic for headaches and were referred to a pediatric psychiatric clinic for psychiatric symptoms from January 2010 to December 2011. All patients completed the CDI and RCMAS. Among them, charts of patients with clinically significant total scores (cutoff>15) for psychiatric symptoms, as defined by the CDI and RCMAS scoring scales, were reviewed.ResultsNineteen patients had headaches and clinically significant total scores for psychiatric symptoms. The mean age at headache diagnosis was 11.7 years, and 57% were male. Mean duration of headache was 11.5 months. Two point eight percent of the patients were diagnosed with psychiatric disorders including major depression (1.7%), generalized anxiety disorder (1.1%), and bipolar disorder (0.1%). Four patients (0.6%) were diagnosed with attention deficit/hyperactivity disorder (ADHD). Total mean CDI and RCMAS scores of patients referred to the psychiatric clinic were 18.8 and 22.2, respectively. There was no correlation between CDI or RCMAS total scores and headache frequency, duration, or severity.ConclusionWe recommend that all patients with headache should be screened for depression and anxiety by CDI and RCMAS scores.
This study provides Class II evidence that in patients with pediatric focal epilepsy, LVT and CBZ exhibit equivalent effects on neuropsychological function.
The aim of this study was to evaluate the clinical usefulness of measurement of β 2 microglobulin (β 2 MG), N-acetyl-β-D-glucosaminidase (NAG) of spot urine samples as indices of renal tubular damage and microalbumin of spot urine samples as a parameter of glomerular damage in children with vesicoureteral reflux (VUR) or renal defects. Methods : We studied 91 children with previous UTI. The children were classified as 62 children without VUR and renal defects (group I), 10 children with VUR, without renal defects (group II), and 19 children with VUR and renal defects (group III). Patients having VUR were separated according to the degree of VUR (mild VUR: VUR grade I-III, severe VUR: VUR grade IV-V). Urinary excretion of β 2 microglobulin (β 2 MG), microalbumin, N-acetyl-β-D-glucosaminidase (NAG), creatinine were measured in samples of morning urine specimens. Children with VUR or renal defects detected by voiding cystourethrography (VCUG) and DMSA renal scan were investigated. Results : Microalbumin/Cr ratio of spot urine was significantly increased in group III compared group I (42.3±27.2 mg/gCr vs 25.2±10.9 mg/gCr, P<0.05). NAG/Cr ratio of spot urine was significantly increased in group II compared group I (3.70±23.4 mg/gCr vs 18.7±12.7 mg/gCr, P<0.05). There was no statistically significant difference of β 2 MG/Cr ratio among three groups. Conclusion : Urinary microalbumin excretion of morning urine sample may be a simple and reliable clinical indicators for early identification of renal damage in children with VUR and renal defects. Urinary microalbumin excretion may be useful marker to predict the the severity of VUR.
Purpose : Vitamin D deficiency rickets is a significant public health problem that results from insufficient exposure to sunlight and inadequate vitamin D supplementation. The purpose of this study is to identify the clinical characteristics of vitamin D deficiency rickets in infants. Methods : Data of 35 infants diagnosed as vitamin D deficiency rickets at Sanggye-Paik Hospital, Seoul, Korea, from March 2007 to May 2009 were reviewed. Children with plasma 25-hydroxyvitamin D levels&lt;15 ng/mL and 15-30 ng/mL were considered to have vitamin D deficiency and vitamin D insufficiency, respectively. Results : Thirty-five infants (22 boys, 13 girls) were diagnosed with rickets. Mean age at diagnosis was 7.4¡?#?.1 ;months (range: 0.1-29.8 months). Eighteen infants (51%) were vitamin D deficient and seventeen infants (49%) were insufficient. Twenty-eight of all (80%) diagnosed as subclinical rickets. Twenty-nine infants (83%) were below the age of 12months. Twenty infants (57%) had breastfed and ten infants (29%) had iron deficiency anemia. Nine of breastfed infants (45%) were vitamin D deficient and ten of their mothers were vitamin D insufficient. Overall, radiographic evidence of rickets was present in 93% of the cases. Radiographic sign of rickets was evident even in vitamin D insufficient state. Conclusion : It is important for the clinician to screen for subclinical vitamin D deficiency rickets in inadequately supplemented infants by pairing 25-hydroxyvitamin D levels with wrist radiographs. A nationwide epidemiological study of vitamin D deficiency rickets must be conducted and evidence-based national guidelines must be defined to prevent rickets
The ketogenic diet (KD) is an established, effective, nonpharmacologic treatment for children with pharmacoresistant epilepsy. Although the KD is the most well-established dietary therapy for epilepsy, it is too restrictive and is associated with serious complications; therefore, alternative lower-fat diets, including a modified Atkins diet and low-glycemic index diet, have been developed. Recent ongoing clinical evidence suggests that other dietary therapies have an efficacy almost comparable to that of the KD. In addition, a diet rich in polyunsaturated fatty acids appears to increase the efficacy of diet therapy and reduce the complications of a high-fat diet. Here, we review the systematic information about lower-fat diets and better-quality dietary therapies and the current clinical status of each of these dietary approaches.
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