Systemic lupus erythematosus (SLE) is an autoimmune disorder involving multiple organs. Neuropsychiatric symptoms are frequently associated in SLE, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contains both central and peripheral nervous systems, which includes transverse myelitis, and Guillain-Barre syndrome (GBS). We report our experience of concurrent manifestation of transverse myelitis and GBS as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 14-year-old boy with SLE who first presented with features of GBS. The patient developed ascending weakness starting from low extremities, experienced difficulty voiding, and had a facial rash. An initial diagnosis of GBS was made on the basis of clinical findings and nerve conduction studies. But he did not respond to intravenous immunoglobulin therapy and following spine MRI displayed T2 weighted high signal intensities from the cervical to thoracic region of the spinal cord, and serological analysis revealed the presence of anti-dsDNA, anti-smAb, anti nuclear antibody with decreased level of complements. The diagnosis was revised to GBS and acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. This was followed by oral prednisolone and cyclophosphamide pulse therapy. This is the first case of concurrent manifestation of GBS and transverse myelitis as initial presentation of SLE. The cross-reactivity of autoantibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Clinicians should consider a diagnosis of SLE as an etiology of GBS or transverse myelitis.
Adolescent ; Autoantibodies ; Complement System Proteins ; Cyclophosphamide ; Exanthema ; Extremities ; Guillain-Barre Syndrome ; Humans ; Immunization, Passive ; Lupus Erythematosus, Systemic ; Methylprednisolone ; Myelitis, Transverse ; Neural Conduction ; Peripheral Nervous System ; Prednisolone ; Spinal Cord ; Spine

Brainstem encephalitis caused by Epstein-Barr virus (EBV) is rare in childhood. We describe a 14-month-old-boy who presented with limitation of eye movements with sixth cranial nerve palsy. Brainstem encephalitis caused by EBV was diagnosed, based on the neuroimaging and cerebrospinal fluid (CSF), serological examination for EBV infection. Brain magnetic resonance imaging (MRI) showed high signal intensity in T2 and low signal intensity in T1 in both pons and medulla. The result of EBV polymerase chain reaction (PCR) of the CSF was positive, and a serological test showed a convalescent phase of primary infection: positive for viral capsid antigen (VCA)-IgM, VCA-IgG and EBV nuclear antigen (EBNA)-IgG, negative for heterophil antibody. We suggest that EBV infection should be considered a differential diagnosis cause of pediatric brainstem encephalitis and emphasizes the need for immediate diagnosis and initiation of symptomatic and specific therapy.
Abducens Nerve ; Abducens Nerve Diseases ; Brain ; Brain Stem ; Capsid ; Child ; Diagnosis, Differential ; Encephalitis ; Epstein-Barr Virus Infections ; Eye Movements ; Herpesvirus 4, Human ; Humans ; Magnetic Resonance Imaging ; Neuroimaging ; Polymerase Chain Reaction ; Pons ; Serologic Tests

Basilar migraine is a rare type of migraine with complex symptoms including aura such as dysarthria, vertigo, tinnitus, and decreased level of consciousness. A 13-year-old male patient was presented with severe headache and immobility of whole body for 20 minutes after vomiting, dizziness, and dysarthria. Similar episode of headache and unconsciousness after vomiting happened 2 weeks ago prior to the visit. Vital signs were stable and pupils showed positive light reflex. Brain MRI and MRA, EEG, and electrocardiography revealed no abnormality. One day after discharge from hospital, patient revisited emergency room because of similar episode of headache. Preventive medication was started with flunarizine 5 mg per day. However, similar episodes of headache and loss of consciousness repeated three times over the next four months. Topiramate was then added with dose of 100 mg divided into two doses. Since then, the patient has been symptom free over 1 year.
Adolescent ; Brain ; Consciousness ; Dizziness ; Dysarthria ; Electrocardiography ; Electroencephalography ; Emergencies ; Epilepsy ; Flunarizine ; Fructose ; Headache ; Humans ; Light ; Male ; Migraine Disorders ; Migraine with Aura ; Paralysis ; Pupil ; Reflex ; Tinnitus ; Unconsciousness ; Vertigo ; Vital Signs ; Vomiting

The term 'rhombencephalitis' refers to inflammatory diseases affecting the hindbrain. We experienced a case of rhombencephalitis in a 14 years old girl, who presented with fever, headache, and intractable hiccup for one week prior to admission. Brain MRI on admission showed bilateral high signal intensities in the dorsal portion of the medulla oblongata on T2WI and FLAIR. Examination of cerebrospinal fluid showed increased WBC count (33/mm3, Lymphocyte 81%), normal protein and glucose level. Immunoserologic study showed decreased C3, C4 level and increased Anti-ds DNA level. However, FANA was negative. The hiccup was controlled by administration of IVIG (1 g/kg/day for 2 days) and steroid (30 mg/kg/day for 3days) without any neurologic symptoms. We report a case with the brief review of related literatures.
Brain ; DNA ; Encephalitis ; Fever ; Glucose ; Headache ; Hiccup ; Immunoglobulins, Intravenous ; Lymphocytes ; Medulla Oblongata ; Neurologic Manifestations ; Rhombencephalon

PURPOSE: Seizure outcomes are more complicated in terms of repeated remission and relapse in the course of epilepsy. We aim to investigate the different patterns of evolution in new-onset pediatric epilepsy and the seizure outcome of different types of epilepsy syndromes. METHODS: We examined the evolution pattern of remission and relapse in the course of epilepsy in 326 children who were less than 15 years of age, with new-onset epilepsy. Different remission-relapse patterns were determined in each patient and according to epilepsy syndromes. The probability of repeated remission and relapse were analyzed with Markov process. RESULTS: During follow-up (mean+/-SD: 79+/-25 months) of 326 patients, early remission, defined as remission within the first year of treatment, was seen in 288 patients (88.4%), and late remission was achieved in 21 patients (6.4%). 17 patients (5.2%) never achieved remission. 94.8% of the cohort experienced at least one remission, with first relapse, second relapse, and third relapse occurring in 115 patients (35.3%), 61 patients (18.7%), and 28 patients (8.6%), respectively. At the end of follow-up period, 281 patients (86.2%) were in terminal remission. 194 patients (59.6%) showed a continuous remitting course, and 87 patients (26.7%) showed a remitting-relapse course. 45 patients (13.8%), including worsening courses in 28 patients (8.6%) and drug resistant courses in 17 patients (5.2%), did not show terminal remission. Markov process disclosed that children with epileptic encephalopathy and symptomatic partial epilepsy were less likely to show remission than children with idiopathic partial or generalized epilepsy (P<0.001). CONCLUSION: Only 13.8% of children with new-onset epilepsy have poor seizure outcome in terms of never achieving remission or persistent seizure after achieving at least one remission. The etiology of epilepsy syndrome is an important factor determining seizure outcome.
Child ; Cohort Studies ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Generalized ; Follow-Up Studies ; Humans ; Markov Chains ; Natural History ; Recurrence ; Seizures

PURPOSE: Tic disorder is a neurodevelopmental disorder which begins in early childhood and continues into adolescence and adults. Tic disorder affects 1-2% of the population. In order to make a correct diagnosis and treatment of tic disorder, the clinical psychological analysis is needed because children with tic disorder tend to have higher rates of emotional and behavioral difficulties than those of the general population. The aim of this study was to evaluate the clinical and psychological characteristics of child with tic disorder. METHODS: The sample consisted of 69 patients with tic disorder who visited to the pediatric neurology clinic of Kangnam sacred heart hospital from January 2007 to June 2010. Fifty patients who visited our clinic without tic disorder were included in the control group. Patients were classified as the DSM-IV, Tourette's disorder, chronic motor or vocal tic disorder, transient tic disorder and tic disorder not otherwise specified. Korean child behavior checklist (K-CBCL), K-WISC-III, ADS, K-ARS was conducted in the tic disorder patients and T score of K-CBCL was compared with the control group. RESULTS: Male to female ratio was 2.5:1 in the study group and the mean age was 9.5+/-3.2 years old. The symptom period was 16.7+/-17.2 months at their first visit. Twelve subjects (18%) presented with Tourette syndrome, 14 subjects (20%) had multiple chronic motor or phonic tic disorder, 20 subjects (29%) had transient tic disorder and 23 subjects (33%) had tic disorder not otherwise specified. The Medications were given in 26 patients and risperidone was the primary drug for most of them. The mean T scores of K-CBCL were 55.5+/-6.1 for attention scale, 56.9+/-6.9 for anxiety-depression scale, 56.5+/-7.4 for withdrawn scale, 52.0+/-3.4 for emotional lability scale. All of them were higher than control group (P<0.05). CONCLUSION: In order to make a correct diagnosis and treatment of tic disorder, the clinical psychological assessment is mandatory due to their higher rates of emotional and behavioral difficulties.
Adolescent ; Adult ; Checklist ; Child ; Child Behavior ; Diagnostic and Statistical Manual of Mental Disorders ; Female ; Heart ; Humans ; Male ; Neurology ; Risperidone ; Tic Disorders ; Tics ; Tourette Syndrome

PURPOSE: The aim of this study was to describe the clinical course and outcome of the patient with epilepsy after acute febrile encephalopathy. METHODS: Medical records of the twenty patients with acute febrile encephalopathy from Mar. 2003 to Dec. 2011, were reviewed. The outcome of epilepsy over 12 months after discharge from encephalopathy was investigated and compared with non-epilepsy group on several clinical and laboratory aspects. RESULTS: All revealed negative on viral study. Eight of 20 patients evolved to epilepsy eventually and 3 out of them had a status epilepticus as initial manifestation. Five of them remained medically intractable. Two showed ongoing violent behavior during follow-up period and 1 had motor weakness of lower limbs for a year. All had non-specific brain MRI findings, except the one who showed suspected cerebritis of right basal ganglia on follow-up study. Five of 8 epilepsy patients showed epileptic discharges on the first electroencephalogram. Two of them showed sustained frontal spikes and one who had abnormal brain MRI findings showed left temporal spike on follow-up EEG. Longer period of altered mentality and more frequent epileptiform discharges on initial EEG appeared to be related with progression to epilepsy during follow-up period. CONCLUSION: Acute febrile encephalopathy in children may be related to poor seizure outcome and resultant psychomotor problems. Further studies including laboratory exams to define its pathophysiology would be needed.
Basal Ganglia ; Brain ; Child ; Electroencephalography ; Epilepsy ; Fever ; Follow-Up Studies ; Humans ; Lower Extremity ; Medical Records ; Seizures ; Status Epilepticus

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) is known to have a good prognosis, and is easily controlled with antiepileptic drugs. In this study, we evaluate the efficacy of low-dose topiramate (TPM), once at night time in children with BECTS, and compare with that of oxcarbazepine (OXC). METHODS: OXC was used as a first drug in 58 patients with BECTS at the department of pediatrics, Chonnam National University hospital from Jan. 2003 to Nov. 2010. Their medical records were reviewed, focusing on the onset age of seizure, the dosage of drugs, response to treatment and other clinical features. Since Aug. 2008 through Apr. 2011, some newly diagnosed patients with BECTS were given a low-dose (25mg) of TPM, once at nighttime. The efficacy was reviewed in 14 children, who had been followed up in the outpatient clinic for longer than twelve months. RESULTS: Forty five out of 58 (77.6%) patients were well controlled with OXC. Thirteen out of 58 (22.4%), seizures recurred, on whom TPM (n=9) or lamotrigine (n=4) was added. Nine (64.3%) of the 14 patients who were started with low dose TPM became seizure-free for at least 12 months. However, Two out of 14, the dose of TPM had to be increased up to 100 mg/day and 75 mg/day, respectively. In three, seizure was controlled with OXC. CONCLUSION: A single low-dose TPM would be as effective as the conventional OXC without any adverse effects in children with BECTS.
Age of Onset ; Ambulatory Care Facilities ; Anticonvulsants ; Carbamazepine ; Child ; Epilepsy ; Epilepsy, Rolandic ; Fructose ; Humans ; Medical Records ; Pediatrics ; Prognosis ; Seizures ; Triazines

PURPOSE: This study was aimed to investigate the optimum dose of diazepam to reduce the recurrence of febrile seizures and side effects in children with febrile seizures. METHODS: The subjects of this study included 528 children with febrile seizures (3 months-5 years of age) who were admitted to Eulji University Hospital (Daejeon, Korea) from January 2008 to December 2011. The children divided into four groups according to the dose of diazepam; Group I, 121 patients, received no diazepam therapy, group II, 129 patients, received oral diazepam in a single dose of 0.1 mg/kg after the febrile seizures, group III, 127 patients, 0.2 mg/kg, and group IV, 151 patients, 0.3 mg/kg, respectively. RESULTS: Seizures recurred in 6 of 129 children (4.7%) in group II, 1of 127 children (0.8%) in group III, and none of 151 children in group IV recurred. For the 121 untreated patients, febrile seizures recurred in 20 (16.5%) children. This study revealed a significant difference in the rate of recurrence of febrile seizures between children treated with diazepam and those who were not. And the recurrence rate was decreased by the increment of the dosage of diazepam, but there was no significant difference between groups. The side effects were observed in 19.9% of children treated with diazepam, 3.9% in group II, 12.6% in group III, and 39.7% in group IV, The rate of side effect was also increased with the increment of the dosage. CONCLUSION: An oral diazepam therapy will reduce the incidence of recurrent febrile seizures during the same febrile illnesses. We think the optimum dose of diazepam is 0.1 mg/kg or 0.2 mg/kg rather than 0.3 mg/kg. However, the use of oral diazepam after a febrile seizure should be carefully considered with weighing the benefits and potential adverse effects.
Child ; Diazepam ; Humans ; Incidence ; Psychotherapy, Group ; Recurrence ; Seizures ; Seizures, Febrile

PURPOSE: Paroxysmal kinesigenic dyskinesia (PKD) is one of the movement disorders in which dyskinesia occurs in a part of the body by a sudden movement after a rest under a tension or a stress. This study was aimed to evaluate the clinical features of children and adolescents with PKD in Korea via analysing the patients who have treated in Department of Pediatrics, Kyungpook National University Hospital. METHODS: A total of seven children with PKD was involved in the study and their medial records were retrospectively evaluated. RESULTS: The mean age of the 7 subjects was 15.7 years (10.0-21.4 years old). The male to female ratio was 6:1. They presented with dystonia with the average duration of 10.5 seconds (3.5-17.5 seconds), which triggered by various sudden movements. No accompanying cormorbidities were noted. Their laboratory findings were unremarkable. Six of the patients, except one who refused treatment with medicine, responded well to medication and remained symptom free. The average time response to medication was 3.4 weeks (0.95-7.81 weeks). They were of treated with either oxcarbazepine (n=4, 14.9+/-5.8 mg/kg/day) or lamotrigine (n=2, 1.5+/-0.9 mg/kg/day). There was no significant difference between two groups in terms of age, response, adverse events, and so on. CONCLUSION: This study showed that clinical features of Korean children with PKD are quite similar to those of other countries. They responded well to the medication. In addition, lamotrigine can be an alternative choice for the treatment.
Adolescent ; Carbamazepine ; Child ; Chorea ; Dyskinesias ; Dystonia ; Female ; Humans ; Korea ; Male ; Movement Disorders ; Pediatrics ; Retrospective Studies ; Triazines