• Journal of The Korean Society of Inherited Metabolic disease
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• v.2 no.1
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• pp.15-19
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• 2002

Disorders resulting from defects in peroxisomal biogenesis include Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. The three diseases are now considered as a continuum of clinical features. Neonatal adrenoleukodystrophy is intermediate between Zellweger syndrome and infantile Refsum disease in severity, and is characterized by profound hypotonia, intractable seizures and premature death. We report a cases of neonatal adrenoleukodystrophy presenting with neonatal seizure and hypotonia. At the age of 43 months, she had clinical evidence of adrenal insufficiency with skin hyperpigmentation and electrolyte imbalance. She was diagnosed having neonatal adrenoleukodystrophy based on abnormally high levels of plasma very long-chain fatty acids, pipecolic acid and phytanic acid.

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.957-963
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• 2009

Neonatal seizures are the most common and distinctive clinical sign of prenatal and/or neonatal brain disorders. Newborn infants with seizures are at risk of mortality and survivors at risk for neurologic impairment, developmental delay, and subsequent epilepsy. Fifteen reports on neonatal seizures in Korea from 1983 to 2009 were analyzed. A total of 731 neonatal seizure cases were reported. Day of seizure onset, etiology, type of seizures, electroencephalogram findings, and outcomes were analyzed. It is necessary to establish a basic report for a future nationwide study of neonatal seizures.

• Neonatal Medicine
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• v.16 no.1
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• pp.10-17
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• 2009

The immature neonatal brain is susceptible to the development of seizures. Seizures occur in 1% to 5% of infants during the neonatal period. Neonatal seizures are most commonly associated with serious acute illnesses, such as hypoxic-ischemic encephalopathy, birth trauma, metabolic disturbances, or infections. Thus, newborn infants with seizures are at risk for neonatal death and survivors are at risk for neurologic impairment, developmental delay, and subsequent epilepsy. Experimental data have also raised concerns about the potential adverse effects of the currently used anticonvulsants in neonates on brain development. Therefore, in the management of neonatal seizures, confirmatory diagnosis and optimal, but shorter, duration of anticonvulsant therapy is essential. Nevertheless, there has been substantial progress in understanding the developmental mechanisms that influence seizure generation and responsiveness to anticonvulsants. The currently used therapies have limited efficacy and the treatment of neonatal seizures has not significantly changed in the past several decades, This review includes an overview of current approaches to the treatment of neonatal seizures.

• Clinical and Experimental Pediatrics
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• v.60 no.2
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• pp.50-54
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• 2017

Purpose: The aims of this study were to evaluate the safety and pharmacokinetics of levetiracetam (LEV) in neonates with seizures and to establish a population pharmacokinetics (PPK) model by using the software NONMEM. Methods: A retrospective analysis of 18 neonatal patients with seizures, who were treated with LEV, including 151 serum samples, was performed. The mean loading dose was 20 mg/kg, followed by a mean maintenance dose of 29 mg/kg/day. Results: Seventeen neonates (94%) had seizure cessation within 1 week and 16 (84%) remained seizure-free at 30 days under the LEV therapy. The mean serum concentration of LEV was $8.7{\mu}g/mL$. Eight samples (5%) were found above the therapeutic range. No serious adverse effects were detected. In the PPK analysis for Korean neonates, the half-life was 9.6 hours; clearance, 0.357 L/hr; and volume of distribution, 4.947 L, showing differences from those in adults. Conclusion: LEV is a safe and effective option for the treatment of neonatal seizures with careful therapeutic drug monitoring.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.535-539
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• 2002

Incontinentia pigmenti is a rare neurocutaneous syndrome characterized by vesiculobullous skin disease in neonates and infants, a noninfectious disease that should be distinguished from infectious diseases with the neonatal seizure or encephalopathy. This disease is X-linked dominant with Xq28 region abnormalities and often associated with developmental defects of the ocular, skeletal, dental, and central nervous system. Central nervous system involvement in the neonatal period, or complicated by encephalopathy, may cause severe neurologic impairment, retardation or even death. We experienced a case of incontinentia pigmenti in a three-day-old female patient who had characteristic papulovesicular skin lesions and partial seizures with secondary generalization. Histopathological examination favored the diagnosis of incontinentia pigmenti and a brain MRI showed undifferentiated white matters with periventricular nodular lesions.

• Neonatal Medicine
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• v.16 no.1
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• pp.18-24
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• 2009

Seizures are the most common clinical symptom of a neurologic insult and have long been recognized as an obvious marker of brain dysfunction in newborns. Presence of seizures in newborn infants may signify substantial risk for subsequent neurodevelopmental impairment including postneonatal epilepsy and death. The outcomes of seizures in neonates are determined mainly by the etiology of the seizures. Despite the decreasing trend of mortality of neonatal seizures, the prevalence of long-term neurodevelopmental sequelae in survivors has remained unchanged over time. Clinical studies have contributed to identifying significant prognostic factors for neurodevelopmental outcome. The underlying etiology of the seizures and electroencepaphalography background pattern are considered as most reliable early predictors of later neurologic sequelae. However, clinicians managing neonatal seizures are still challenged by difficult therapeutic and prognostic questions because of many unresolved issues in seizure recognition, terminology, relationships to the underlying brain lesion, effect of current management, particularly antiepileptic drugs on long-term outcomes. This review presents the prognosis of neonatal seizures, especially about mortality and neurodevelopmental deficit, and predictors of outcomes.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.192-194
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• 2003

A neonate born at 38 gestational weeks was admitted due to generalized tonic-clonic seizure and cyanosis. The neonate was born six days previously at home through normal delivery and the umbilical cord was cut using scissors sterilized in boiling water. The neonate weighed 3,180 g at admission. Physical examination revealed cyanosis, opisthotonus, trismus and reactive muscle spasms. Laboratory exam, brain sonogram and EEG showed no significant abnormal findings. Based on her history and physical examination, the neonate was diagnosed with tetanus and put in an incubator isolated in a quiet, dark room. Treatment with tetanus human immunoglobulin along with antibiotics (penicillin G) were started immediately, and mechanical ventilation, administration of neuromuscular blocking agent and muscle relaxant were also started off. The frequency of seizure episode decreased gradually, and on the 32nd hospital day, mechanical ventilatory support was stopped along with extubation two days later. Thereafter, the neonate was in continuous generalized hypertonic state and showed feeding difficulty, but there was gradual improvement. She was dismissed on the 49th hospital day and is currently under OPD follow-up, doing well with no special problems. Respiratory management is critical to neonatal tetanus. We report here a case of tetanus treated with inhibition of self-respiration, neuromuscular blocker and application of ventilator, and present this method as a useful direction for future treatment of neonatal tetanus.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.10-18
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• 2014

Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis of many of these diseases, if diagnosed early enough before irreversible damage occurs. This makes it essential that the practicing pediatrician, especially neonatologists be familliar with the clinical presentations and systematic approaches of these disorders. Characteristic clinical presentations, methods of systematic approach and typing of various disorders is discussed in this review. The signs of neurological dysfunctions of many IEMs manifesting in the neonatal period is very nonspecific, such as poor feeding, poor sucking, apnea or tachypnea, vomiting, hypertonia, hypotonia, seizure, letharginess, consciousness change and coma. Many other non-metabolic severe disorders of neonatal period such as neonatal sepsis and intracerebral hemorrhage share these nonspecific symptoms. Hyperammonemia, metabolic acidosis, ketosis and hyperlatic acidemia are observed in many of these conditions but there are exceptions in which conditions all basal laboratory tests are normal, such as NKH, sulfite oxidase deficiency and peroxisomal disorders. According to the results of basal laboratory tests, IEMs in the neonatal period can be categorized in to 6 types. Grouping of IEMs into 6 types will make confirmatory tests and early emergency treatment more efficient.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1253-1259
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• 2003

Backgroud : Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. Methods : A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. Results : The average age at onset of seizures was $6.1{\pm}4.6days$ and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. Conclusion : Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.

• Neonatal Medicine
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• v.25 no.2
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• pp.85-89
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• 2018

Rotavirus is the major cause of gastroenteritis in children under the age of 5. Rotavirus infection may lead to several neurological complications as meningitis, encephalitis, convulsion, encephalopathy, hemorrhagic shock, central pontine myelinolysis, Guillain-Barre syndrome, and Reye's syndrome. Further, some reports have described diffuse cerebral white matter lesions on diffusion-weighted magnetic resonance imaging (MRI) in neonates with rotavirus induced seizures. Here, we report on three neonates with rotavirus induced seizures with cerebral white matter abnormalities on MRI.