MOG antibody-associated disorder exhibits different pathophysiological and phenotypic findings than both aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder and typical MS. MOG-antibody is of particular interest in pediatric patients with clinical or radiological non-MS typical findings. MOG-antibody was included in a diagnostic algorithm for children recommending for the first time a standardized use in clinical practice except in cases of typical MS. |
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... |
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... |
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. 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... |
Complementary and alternative medicine (CAM) is a phrase used to describe additional health care methods such as mind/body practices and natural products not regarded as treatments by conventional medicine. The use of CAM in children with common neurologic diseases is more frequent than its use in healthy children (24%–78% vs. 12%). However, less than half of patients report such use... |
We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left... |
Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while... |
Purpose : Electroencephalography (EEG) findings can play a critical role in a variety of decisions, including initiation and withdrawal of antiepileptic drugs (AEDs) therapy. Interictal epileptiform discharges (IEDs) are predictor of recurrent seizures. We investigated IEDs in EEG after AED therapy and related factors in epileptic children. Methods : The subjects were 257 children [151 males and 106 females; age,... |
Purpose : In adults, valproate (VPA) has been reported to be associated with thrombocytopenia. However, few studies have investigated this association in children, and the factors affecting platelet counts remain controversial. This study was undertaken to investigate changes in platelet counts following VPA therapy and related factors in children with epilepsy. Methods : Our subjects comprised 75 epileptic children who... |
Purpose : To assess the height growth of children with epilepsy receiving antiepileptic drugs (AEDs) and the related factors. Methods : The subjects were 148 children diagnosed with epilepsy at Pusan National University Hospital between January 1996 and December 2003, who received AEDs for more than 3 y. We measured height at the initiation of AED medication and at the... |
Purpose : We carried out this study to determine if there is any difference in the occurrence rate of the epileptiform discharge between awake EEG and sleep EEG and if there are any factors influencing on the occurrence rate of EEG. Methods : This study included 178 epileptic children who had visited neurology clinic of the department of pediatrics, Pusan National... |
Epilepsy is one of the most common and disabling neurologic disorders in childhood. The primary goal of epilepsy treatment is to choose the treatment modality that provides the best chance of improving the patient's quality of life. In addition to classic antiepileptic drugs, newly developed antiepileptic drugs ketogenic diet, epilepsy surgery, and vagal nerve stimulation have improved the ability to... |
Hyperekplexia or startle disease is a hereditary neurological disorder characterized by an abnormally exaggerated startle response to tactile, auditory and visual stimuli, together with a global muscular hypertonia and hyperactive tendon reflexes. This disease is a rare, genetically determined disorder, with an autosomal dominant inheritance with variable expression, first described by Suhren, et al. We report two cases of familial hyperekplexia, who developed hypertonia... |
Tyrosinemia type I is an autosomal recessive disorder of amino acid metabolism and is caused by a deficiency of fumarylacetoacetate hydrolase(FAH), the last enzyme in the catabolic pathway of tyrosine. The disease is characterized by hepatic dysfunction, hepatocellular carcinomas, renal tubular dysfunction, rickets, and neurologic crises. We experienced 2 cases(a 4-day-old girl, a 7- month-old girl) of acute form of tyrosinemia type I. Case... |
Childhood onset nemaline myopathy, first described in 1963 by Shy, et al and Conen, et al, is rare congenital myopathy, characterized by nonprogressive or slowly progressive muscle weakness associated with rod-like structures in muscle fibers, often with dysmorphic features. This myopathy was confirmed by muscle biopsy. The light microscopic features noted generally small round fibers that showed variation in size... |
Purpose : We'd like to know the relationship between the changes of cardiac function and systemic O2 consumption according to the increasing dose of dopamine. Methods : Ten rabbits(from 2kg to 2.8kg) were used in this experiment. Anesthesia was induced with intraperitoneal pentobarbital sodium(35mg/kg) and tracheostomy was done. It was maintained by ventilation with a mixture of 1-3% halothane and 67-69%... |
Purpose : Periventricular leukomalacia(PVL) is associated with various neurologic sequelae such as cerebral palsy and cortical blindness. The aim of this study was to analyse the correlation between the degree of PVL on MRI and clinical features or its severity. Methods : Thirty-eight children with PVL on MRI among children brought to Pusan National University Hospital between January 1996 and August... |
Fibromuscular dysplasia is the single leading etiology of renovascular hypertension in children. We report an eight-year-old girl who was admitted for generalized tonic seizure with fibromuscular dysplasia of right renal artery. On admission, she presented hypertension and altered mentality. She had suffered from intermittent severe headache for the past year. Renal angiography of right renal artery showed stenosis, beaded pattern... |
Leigh Disease, or subacute necrotizing encephalopathy(SNE), is a degenerative disorder characterized by lesions of the gray and white matter in the bran and spinal cord. The pathogenesis was known as mitochondrial enzyme defect of the respiratory chain system. We experienced 2 cases of Leigh disease. The first case, a seven-month old girl who was presented with weak respiration and failure... |
Hemobilia caused by pancreatic disease is very rare. The most common cause is a splenic artery pseudoaneurysm caused by acute and chronic inflammation of the pancreas. We experienced a case regarding as hemobilia as a complication of chronic relapsing pancreatitis in a 14 year-old boy. He was admitted with chief complaints of abdominal pain and hematemesis. Two years prior to admission,... |