The efficacy of ketogenic diet in childhood intractable epilepsy with malformation of cortical development / 소아과

PURPOSE: Malformation of cortical development(MCD) constitutes an important etiology of intractable epilepsy and is considered an indication for surgical treatments, though their efficacy is limited and variable depending on MCD's location or distribution. Ketogenic diets are widely known to be effective, but as little study has been made concerning their efficacy on epilepsy with MCD, we evaluated the efficacy of ketogenic diets on MCD patients compared with that of epileptic surgery, which is more invasive. METHODS: We performed retrospective studies and analyse on 30 patients with MCD diagnosed by brain MRI and treated with ketogenic diets for intractable epilepsy since 1998, checking decreases in their seizure episodes after starting the diets. RESULTS: Cortical dysplasia was observed in 24(80.0 percent) patients as the most common type of MCD. Also, MCD was observed in unilateral hemisphere most commonly, in 23(76.7 percent) patients; it was observed in both hemispheres in 7(23.3 percent) patients. Nine(30.0 percent) out of 30 patients became seizure-free after starting ketogenic diets, and 14(46.7 percent) patients experienced 50 percent seizure reductions as well. Age of starting the diet or the duration of epilepsy period before starting showed no statistical relationship with the efficacy of the diet. Though the younger the patient and the longer the treatment the more effective the diet seemed to be, there was no statistical correlation between them. The location of MCD showed no significance neither. CONCLUSION: Considering various limits and invasiveness of surgical treatment, a ketogenic diet could be a good tool in treating children with intractable epilepsy with MCD.

Prognostic Factors of Status Epilepticus in Children

We retrospectively reviewed the medical records of 189 children who were admitted to the Pediatric Neurology Department at Yonsei University College of Medicine with status epilepticus (SE) between April, 1994 and April, 2003. The children were followed up for a mean duration of 17 months. We analyzed the clinical findings and the relationships between neurologic sequelae, recurrence, age of onset, presumptive causes, types of seizure, seizure duration and the presence of fever. Mean age at SE onset was 37 months. Incidences by seizure type classification were generalized convulsive SE in 73.5%, and non-convulsive SE in 26.5%. The incidences of presumptive causes of SE were idiopathic 40.7%, epilepsy 29.1%, remote 16.4% and acute symptomatic in 13.3%. Among all the patients, febrile episodes occurred in 35.4%, especially in patients under 3 year old, and 38.4% of these were associated with febrile illness regardless of presumptive cause. Neurologic sequelae occurred in 33% and the mortality rate was 3%. Neurologic sequelae were lower in patients that presented with an idiopathic etiology and higher in generalized convulsive SE patients. The recurrence of SE was higher in patients with a remote symptomatic epileptic etiology, and generalized convulsive SE showed higher rates of recurrence. Based on this retrospective study, the neurologic outcomes and recurrence of SE were found to be strongly associated with etiology and seizure type. Age, seizure duration and the presence of febrile illness were found to have no effect on outcome.

Clinical Progress and Treatment Result in Children with Acute Disseminated Encephalomyelitis

PURPOSE: Acute disseminated encephalomyelitis(ADEM) is a neurological disease that is commonly associated with previous history of infection or vaccination. It is mediated through immunological mechanisms, resulting in inflammatory demyelination of the central nervous system. The authors investigated the clinical, radiological features and disease progress of patients diagnosed with ADEM. METHODS: We have retrospectively reviewed 25 patients diagnosed with ADEM through neurological symptoms and brain MRI findings from July 1992 to July 2003. Patients were divided into three groups; patients treated with dexamethasone(group I), those with dexamethasone and immune globulin(group II), and those with methylprednisolone and immune globulin(group III). The neurological symptoms, time taken for recovery, recurrence rates and presence of neurologic residues were statistically analyzed. RESULTS: All 25 patients were treated with steroid, and 18 patients received immune globulin at the same time. Symptomatic improvements occurred 1 to 14 days with a mean of 6.9+/-4.0 days after administration of steroids. 6 patients displayed long-lasting sequelae, including 2 patients who relapsed. The average time taken for clinical improvements in various treatment groups were as follows; 7.5+/-2.1 days for group I(N=4), 7.3+/-5.1 days for group II(N=9), and 5.3+/-2.8 days for group III(N=4). Group III showed most rapid recovery without statistical significance. Differences in the rates of relapse and prevalence of neurological sequelae among those groups were statistically insignificant. CONCLUSION: The image detected on MRI and the clinical features of the patients did not differ from these of previously reported studies. The size of the sample was too small to acquire statistically significant results, but the patients who received methylprednisolone and immune globulin showed shortest recovery time, which might necessiate further studies.

Morphologic Changes of the Pulmonary Arteries after Stent Implantation on Branch Pulmonary Artery Stenosis: Impact of Pulmonary Insufficiency

PURPOSE: Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. METHODS: The subjects in our study were 23 children who had undergone stent implantaion from Jan. 1995 to Jul. 2002 in the Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. RESULTS: The CSAI of the RPA without stenosis changed from mean 238+/-17 mm2/BSA to mean 249+/-20 mm2/BSA(P=0.47), but didn't regress. The CSAI of the LPA with stenosis was increased effectively by stent implantation from the mean 102+/-12 mm2/BSA to mean 125+/-11 mm2/BSA(P< 0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. CONCLUSION: In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPA.

A Case of Linear and Whorled Nevoid Hypermelanosis with Intracranial Lipoma

Linear and whorled nevoid hypermelanosis(LWNH) is characterized by macules in streaks and whorls along Blaschko's lines, that are not preceded by inflammation. LWNH can present with various findings such as neurologic, musculoskeletal, ophthalmologic, facial and cardiac abnormalities. Intracranial lipoma is very rare condition, with an incidence of less than 1% of all intracranial tumors. We recently experienced a case of LWNH with intracranial lipoma of corpus callosum. We report this case with a brief review of the related literature.

Growth Status in Children with Type 1 and 2 Diabetes Mellitus / 대한소아내분비학회지

PURPOSE: It is well known that linear growth in diabetic children is closely related to metabolic control and age of onset. Some studies showed growth retardation in diabetic children regardless of the degree of metabolic control, whereas others reported no growth impairment. Until now, no study has been done comparing linear growth between type 1 and type 2 diabetic children. Therefore, we compared the growth and weight status between type 1 and type 2 diabetic children. METHODS: 145 children with type 1 DM and 27 patients with type 2 DM were studied. Their growth status in height standard deviation score(HTSDS), obesity according to standard weight for height and body mass index(BMI), and HbA1c level at onset, 6 months, 1 year, 3 years and 5 years after treatment were compared. RESULTS: There was no distinct correlation in HTSDS in type 1 and type 2 DM, but in type 2 DM obesity according to standard weight for height and BMI was higher than in type 1 DM. deltaHTSDS 5 years after treatment of diabetic children with poor glycemic control were significantly decreased(P<0.05), but there was no significant correlation between HbA1c and obesity according to standard weight for height and BMI. CONCLUSION: There is no significant correlation between the type of diabetes and growth status, but the poor glycemic controled group showed a significant decrease in deltaHTSDS. These data suggest that the growth of diabetic children could be impaired in the case of poor glycemic control and long duration of diabetes.

Linear Hyperechoic Lesions in the Thalami and Basal Ganglia of Neonates and Infants:A Sonographic Finding of Vasculopathy and Clinical Significance

PURPOSE: This study is to evaluate the clinical significance and possible etiologies of abnormal neurosonographic findings that revealed linear or branching linear hyperechoic vascular lesions in the thalami and basal ganglia of neonates and infants. METHODS: Analysis of 2,866 cranial sonograms (US) and doppler ultrasonography obtained from 1,150 patients identified 53 patients with linear hyperechoic vascular lesions in the thalami or basal ganglia. Patients with only punctate or coarse, nonlinear lesions of echogenicity in these lesions were excluded. Clinical records in 53 patients were reviewed for documentation of congenital cytomegalovirus (CMV) infection, rubella, syphilis, toxoplasmosis, chromosomal abnormality and other major diagnoses. Brain computed tomography (CT) had been performed in 23 of the 53 patients and these images were compared with the cranial sonograms. RESULTS: There were five CMV infection, two congenital syphilis, three Down syndrome, and one Patau syndrome patients. Other major diagnoses encountered included perinatal asphyxia, ventriculomegaly, intraventricular hemorrhage, periventricular leukomalacia, small-for-gestational age, prematurity with hyaline membrane disease, multiple congenital anomalies, neonatal seizure, meningitis, congenital choroid plexus cyst, neonatal hyperbilirubinemia, congenital heart disease, bronchopulmonary dysplasia and others. Brain CT found small calcification in basal ganglia from one patient. Resolution of lesions found in 5 cases with follow-up US studies. CONCLUSION: A broad etiologic basis for linear hyperechoic vascular lesions in the thalami and basal ganglia of neonates and infants indicates complete screening for possible various etiologies.