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2.
Korean Journal of Pediatrics ; : 259-260, 2019.
Article in English | WPRIM | ID: wpr-760221

ABSTRACT

No abstract available.


Subject(s)
Child , Humans , Epilepsy
3.
Journal of the Korean Child Neurology Society ; (4): 27-33, 2017.
Article in English | WPRIM | ID: wpr-139273

ABSTRACT

PURPOSE: Headache is a frequent neurological symptom in school aged individuals and recurrent headache has significant disabling effects among children and adolescents that manifest as school absenteeism, decreased extracurricular activities, and poor academic performance, as shown in previous studies. In Korea, there has not yet been a population-based study of headache-related disability in children and adolescents. We sought to estimate headache-related disability and investigate relevant predictors of disability due to headaches among schoolchildren in South Korea. METHODS: This was a cross-sectional school-based study. We surveyed 5,039 (boys 2,405, girls 2,634) students aged 6-18 years. Among 1,465 students with headache, six hundred sixty-six schoolchildren (225 boys, 441 girls) completed all questionnaires. The questionnaires collected demographic data, in addition to headache specific questions consistent with International Classification of Headache Disorder criteria, 2nd edition. Disability was evaluated using the 6-question Pediatric Migraine Disability Assessment (PedMIDAS). RESULTS: Six hundred sixty six school children and adolescents (225 boys, 441 girls) among 1,465 students with headaches completed all questionnaires. The percentage of headache sufferers with grade I disability was 88.6%. The mean (±standard deviation) PedMIDAS score was 5.11±11.17. There was a trend towards more severe disability in the older age groups, particularly among the 16 to18 year-olds. Students with migraine had the highest PedMIDAS scores (6.69±10.66) whereas students reporting other types of headache had the lowest scores (3.81±7.52). The predictors of headache-related disability were intensity (P=0.028), frequent headache (P=0.003), and longer duration of symptoms prior to presentation (P=0.008). CONCLUSION: A trend towards a more severe disability was observed in the older age group. Schoolchildren with migraine had the most headache-related disability. The predictors for headache-related disability were intensity, frequent headache, and longer duration of symptoms prior to presentation.


Subject(s)
Adolescent , Child , Female , Humans , Absenteeism , Classification , Headache , Headache Disorders , Korea , Migraine Disorders
4.
Journal of the Korean Child Neurology Society ; (4): 27-33, 2017.
Article in English | WPRIM | ID: wpr-139268

ABSTRACT

PURPOSE: Headache is a frequent neurological symptom in school aged individuals and recurrent headache has significant disabling effects among children and adolescents that manifest as school absenteeism, decreased extracurricular activities, and poor academic performance, as shown in previous studies. In Korea, there has not yet been a population-based study of headache-related disability in children and adolescents. We sought to estimate headache-related disability and investigate relevant predictors of disability due to headaches among schoolchildren in South Korea. METHODS: This was a cross-sectional school-based study. We surveyed 5,039 (boys 2,405, girls 2,634) students aged 6-18 years. Among 1,465 students with headache, six hundred sixty-six schoolchildren (225 boys, 441 girls) completed all questionnaires. The questionnaires collected demographic data, in addition to headache specific questions consistent with International Classification of Headache Disorder criteria, 2nd edition. Disability was evaluated using the 6-question Pediatric Migraine Disability Assessment (PedMIDAS). RESULTS: Six hundred sixty six school children and adolescents (225 boys, 441 girls) among 1,465 students with headaches completed all questionnaires. The percentage of headache sufferers with grade I disability was 88.6%. The mean (±standard deviation) PedMIDAS score was 5.11±11.17. There was a trend towards more severe disability in the older age groups, particularly among the 16 to18 year-olds. Students with migraine had the highest PedMIDAS scores (6.69±10.66) whereas students reporting other types of headache had the lowest scores (3.81±7.52). The predictors of headache-related disability were intensity (P=0.028), frequent headache (P=0.003), and longer duration of symptoms prior to presentation (P=0.008). CONCLUSION: A trend towards a more severe disability was observed in the older age group. Schoolchildren with migraine had the most headache-related disability. The predictors for headache-related disability were intensity, frequent headache, and longer duration of symptoms prior to presentation.


Subject(s)
Adolescent , Child , Female , Humans , Absenteeism , Classification , Headache , Headache Disorders , Korea , Migraine Disorders
5.
Korean Journal of Anesthesiology ; : S62-S63, 2014.
Article in English | WPRIM | ID: wpr-144893

ABSTRACT

No abstract available.


Subject(s)
Humans , Abdominal Pain , Celiac Plexus , Gastroparesis
6.
Korean Journal of Anesthesiology ; : S62-S63, 2014.
Article in English | WPRIM | ID: wpr-144880

ABSTRACT

No abstract available.


Subject(s)
Humans , Abdominal Pain , Celiac Plexus , Gastroparesis
7.
Journal of Biomedical Research ; : 141-145, 2014.
Article in Korean | WPRIM | ID: wpr-70877

ABSTRACT

Guillain-Barre syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy most commonly characterized by rapidly progressive, essentially symmetric weakness and areflexia. This study examined clinical symptoms of clinical variants of GBS through a cerebrospinal fluid (CSF) study, nerve conduction (NCV) study, treatment, and prognosis. There were 16 children with GBS who visited our hospital from January 2011 to December 2013. Guillen-Barre-like syndromes with transient synovitis were noted in three children. Clinical variants of GBS with acute demyelinating encephalomyelitis were observed in one child. Previous infections were noted in 16 children with Guillen-Barre-like syndrome. There were ascending infections in 16 cases. Fifteen children showed symmetric infections, and one showed asymmetric infection. In NCV, slow waves were noted in two cases. We treated using intravenous immunoglobulin (IVIG) in four cases, IVIG with steroid in two, cases and supportive care in 10 (62.5%) cases. Five children treated with IVIG and 10 with supportive care management were completely improved.Our study suggests that supportive care is effective as a treatment for clinical variants of GBS. Further study is necessary for more patients.


Subject(s)
Child , Humans , Cerebrospinal Fluid , Encephalomyelitis , Guillain-Barre Syndrome , Immunoglobulins , Immunoglobulins, Intravenous , Neural Conduction , Prognosis , Synovitis
8.
Journal of the Korean Child Neurology Society ; (4): 39-45, 2013.
Article in Korean | WPRIM | ID: wpr-65492

ABSTRACT

PURPOSE: Despite the high prevalence of headaches in students, their lifestyles have not been well examined. We examined correlation between lifestyle and headache in Korean students. METHODS: We conducted a cross-sectional, school-based study from a randomized and proportional sample of 5,360 male and female students. The questionnaires include demographic data, lifestyle, in addition to specific questions about headache according to International Classification of Headache Disorder II (ICHD-II) criteria. Valid questionnaires were returned by 94.1% (5,039, boys 2,405, girls 2,634) of the sample. Modified criteria changed the item 'duration' in migraine (>1 hour instead of 4 hours). We analyzed the data in terms of exercise, regular eating, sleep time, fluid intake, and caffeine intake. RESULTS: Students on regular exercise have less headache (P=0.03). Students who eat regularly have less recurrent headache (P<0.0001, odds ratio=0.5). Students sleep more have less headache (P<0.0001). Students take more fluid have less headache (P=0.0002). Students take more caffeine have more headache (P=0.0001). CONCLUSION: There seem to be a significant relationship between life style and headache in school children in South Korea. We suggest that changing the lifestyles or habits should be considered to manage and prevent headaches in school children.


Subject(s)
Child , Female , Humans , Male , Caffeine , Eating , Headache , Headache Disorders , Life Style , Migraine Disorders , Prevalence , Surveys and Questionnaires , Republic of Korea
9.
Journal of the Korean Child Neurology Society ; (4): 241-249, 2013.
Article in Korean | WPRIM | ID: wpr-199734

ABSTRACT

PURPOSE: The aim of this study was to investigate the causative organisms, clinical manifestations, and prognosis of pediatric patients with bacterial meningitis in Daejeon and Chungcheong area, occurred from 2006 to 2010. METHODS: We retrospectively reviewed medical records of patients aged between 1 month and 15 years, diagnosed with bacterial meningitis at 8 university or general hospitals in Daejeon and Chungcheong area. The bacterial meningitis was defined by isolation of organism from cerebrospinal fluid(CSF). The data was collected from January 2006 to December 2010, and analyzed including patient's demographics, causative organisms, clinical presentation, laboratory findings and complications. RESULTS: During the 5-year study period, 24 patients were diagnosed with CSF culture-proven bacterial meningitis. The most common causative organism was Streptococcus pneumoniae (S. pneumoniae, 37.5%), and the others were group B streptococcus (GBS, 20.8%), Escherichia coli (E. coli, 16.7%), Neisseria meningitidis (N. meningitidis, 8.3%), Haemophilus influenzae (H. influenzae, 4.2%), respectively. They initially complained of fever (95.8%), vomiting (83.3%), anorexia (45.8%), seizure (29.2%), headache (20.8%). The leukocyte counts exceeding 1,000/mm3 in CSF was observed in 14 patients (58.3%). In 15 patients (62.5%), the glucose concentration in CSF was less than 50 mg/dL, 18 patients showed that the protein concentration in CSF was more than 100 mg/dL. Long-term neurologic sequelae were observed in 4 patients (16.7%) and described as hearing disturbance (2 patients), hemiparesis (1 patient) and endocrine dysfunction (1 patient). Ten patients (41.7%) showed abnormal neuroradiologic findings and the most common abnormalities was subdural effusion (25.0%). CONCLUSION: Compared to the previous study performed between 2001 and 2005, S. pneumonia continued to be the leading cause of the pediatric bacterial meningitis in Daejeon and Chungcheong area. The frequency of pneumococcal meningitis was not decreased, despite of the introduction of conjugated pneumococcal vaccination. On the other hand, H. influenzae meningitis was notably decreased.


Subject(s)
Humans , Anorexia , Demography , Escherichia coli , Fever , Glucose , Haemophilus influenzae , Hand , Headache , Hearing , Hospitals, General , Influenza, Human , Leukocyte Count , Medical Records , Meningitis , Meningitis, Bacterial , Meningitis, Pneumococcal , Neisseria meningitidis , Paresis , Pneumonia , Prognosis , Retrospective Studies , Seizures , Streptococcus , Streptococcus pneumoniae , Subdural Effusion , Vaccination , Vomiting
10.
Korean Journal of Pediatrics ; : 269-269, 2013.
Article in English | WPRIM | ID: wpr-22359

ABSTRACT

No abstract available.

11.
Journal of the Korean Child Neurology Society ; (4): 152-161, 2013.
Article in Korean | WPRIM | ID: wpr-27425

ABSTRACT

PURPOSE: To avoid a unnecessary lumbar puncture, we tried to find a useful tool for prediction of meningitis in infants. METHODS: One hundred and twenty four patients underwent a lumbar puncture at Chungbuk National University Hospital from Jan 1, 2011 to Dec 31, 2011. We divided them into 2 groups by the evidence of meningitis, and compared laboratory findings and clinical manifestations. RESULTS: There were 30 patients for meningitis group and 94 patients for control group. Meningitis group revealed lower serum monocyte percentage (meningitis group 10.1+/-4.7%, control group 12.2+/-5.0%, P=0.049) than control group. Other laboratory findings and clinical manifestations showed no statistically significant differences between two groups. CONCLUSION: Serum monocyte percentage could help to predict meningitis in young infants, but it is not enough to replace a lumbar puncture. We therefore think that it requires a more accurate non-invasive diagnostic tool to detect meningitis.


Subject(s)
Humans , Infant , Meningitis , Monocytes , Spinal Puncture
12.
Journal of the Korean Child Neurology Society ; (4): 129-136, 2012.
Article in Korean | WPRIM | ID: wpr-37027

ABSTRACT

PURPOSE: Attention Deficit Hyperactivity Disorder (ADHD) is known to be more common in children with epilepsy than in the general population. Thirty one to forty percent of ADHD is accompanied with epilepsy. Few studies regarding this matter have been reported in Korea. This study was aimed to evaluate the comorbidity of ADHD in children with epilepsy. METHODS: This is a two center based, retrospective and controlled study. Thirty four ADHD children with epilepsy from Chungbuk National University hospital and 38 ADHD children without epilepsy from Cheonju St. Mary's hospital were recruited from January 2005 to June 2010. RESULTS: In ADHD children with epilepsy, twelve (35.2%) had partial seizures, 11 (32.2%) did generalized seizures and 11 (32.2%) were unclassified. EEG abnormalities were found in the frontal lobe (15 cases), in the central lobe (7 cases), in the temporal lobe (6 cases), and in the occipital lobe (3 cases). In ADHD children with epilepsy, the combined type was major (76.4%) and in ADHD children without epilepsy, the inattentive type was major (50.5%) (P=0.004). Learning disability was mor common in ADHD with epilepsy than in ADHD without epilepsy (P=0.01). CONCLUSION: This study showed that ADHD children with epilepsy are more likely to have combined type (76.4%) and learning disability as compared with ADHD without epilepsy.


Subject(s)
Child , Humans , Attention Deficit Disorder with Hyperactivity , Comorbidity , Electroencephalography , Epilepsy , Frontal Lobe , Korea , Learning Disabilities , Occipital Lobe , Retrospective Studies , Seizures , Temporal Lobe
13.
Journal of the Korean Child Neurology Society ; (4): 26-32, 2011.
Article in Korean | WPRIM | ID: wpr-171522

ABSTRACT

PURPOSE: Levetiracetam hasa been used in adjuvant therapy. It has also been used in monotherapy in other countries and therefore, we also studied the effect and efficacy of Levetiracetam monotherapy. METHODS: We retrospectively studied the types of epilepsy, EEG, and drug dosage. We studied 101 epilepsy children treated by Levitiracetam monotherapy who had visited our hospital from August 2007 to July 2009. RESULTS: Participants were aged one month to 20 years. The mean age of Levetiacetam therapy was initially 11+/-4 years (from 3 years to 21 years), and the type of epilepsy was partial in 57.4% and generalized in 42.6%. The mean dose I initially began at 6+/-4 mg/kg/day (from 2 mg/kg/day to 30 mg/kg/day) with a mean final dose of 30+/-8 mg/kg/day (from 6 mg/kg/day to 60 mg/kg/day), a mean duration of therapy of 21+/-11months, and a duration of therapy ranging from one to 38months. Further, 60.3% of patients became seizure free and 96.9% exhibited at least a 50% reduction in seizure over a 12 month period. Side effects included behavioral change (8), asthenia (2), cognitive change (1), rash (2), headache (5), inadequate seizure control (2), and increased seizure (5). Levetiracetam was discontinued due to inadequate seizure control (2), increased seizure (5) and side effects (2). CONCLUSION: We studied the efficacy and tolerability of monotherapy of Levetiracetam and found that it was effective and tolerable in monotherapy for epilepsy.


Subject(s)
Aged , Child , Humans , Anticonvulsants , Asthenia , Electroencephalography , Epilepsy , Exanthema , Headache , Piracetam , Retrospective Studies , Seizures
14.
Korean Journal of Pediatrics ; : 241-245, 2011.
Article in English | WPRIM | ID: wpr-31620

ABSTRACT

Tuberous sclerosis complex (TSC) is a genetic multisystem disorder that results from mutations in the TSC1 or TSC2 genes, and is associated with hamartomas in several organs, including subependymal giant cell tumors. The neurological manifestations of TSC are particularly challenging and include infantile spasms, intractable epilepsy, cognitive disabilities, and autism. The TSC1- and TSC2-encoded proteins modulate cell function via the mammalian target of rapamycin (mTOR) signaling cascade, and are key factors in the regulation of cell growth and proliferation. The mTOR pathway provides an intersection for an intricate network of protein cascades that respond to cellular nutrition, energy levels, and growth factor stimulation. In the brain, TSC1 and TSC2 have been implicated in cell body size, dendritic arborization, axonal outgrowth and targeting, neuronal migration, cortical lamination, and spine formation. The mTOR pathway represents a logical candidate for drug targeting, because mTOR regulates multiple cellular functions that may contribute to epileptogenesis, including protein synthesis, cell growth and proliferation, and synaptic plasticity. Antagonism of the mTOR pathway with rapamycin and related compounds may provide new therapeutic options for TSC patients.


Subject(s)
Humans , Infant , Infant, Newborn , Autistic Disorder , Axons , Body Size , Brain , Drug Delivery Systems , Epilepsy , Giant Cell Tumors , Hamartoma , Logic , Neurologic Manifestations , Neurons , Plastics , Proteins , Sirolimus , Spasms, Infantile , Spine , Tuberous Sclerosis
15.
Journal of the Korean Child Neurology Society ; (4): 177-183, 2011.
Article in Korean | WPRIM | ID: wpr-80166

ABSTRACT

PURPOSE: To assess the usefulness of amplitude integrated electroencephalography (aEEG), we compared aEEG with electroencephalography (EEG), which is one of the most useful tools for assessment of neonatal seizures in the neonatal intensive care unit. METHODS: We retrospectively studied 24 neonates who had been suspected of seizure activity in the neonatal intensive care unit from January 2009 to February 2010. The 24 neonates had seizure or apnea that did not respond to aminophylline or continuous positive airway pressure. We compared the correlation and efficacy of diagnosis among aEEG, EEG, and neurosonography. RESULTS: We analyzed 24 aEEGs in the neonatal intensive care unit. The aEEGs showed a seizure pattern in 15 (62.6%), burst suppression pattern in 2 (8.3%), flat pattern in 4 (8.3%), and normal in 5 (20.8%). The results of EEGs showed 15 neonatal seizures (62.5%), 2 cerebral dysfunctions (8.3%), and 7 normal findings (29.2%). A correlation between aEEG and EEG was noted (P<0.05). However, there was no correlation between the results of aEEG and echoencephalography. CONCLUSION: There is correlation between aEEG and EEG for seizure detection in the neonatal intensive care unit. We think that aEEG is an useful screening tool for seizure detection in neonates.


Subject(s)
Humans , Infant, Newborn , Aminophylline , Apnea , Continuous Positive Airway Pressure , Electroencephalography , Intensive Care, Neonatal , Mass Screening , Retrospective Studies , Seizures
16.
The Korean Journal of Physiology and Pharmacology ; : 317-324, 2010.
Article in English | WPRIM | ID: wpr-728365

ABSTRACT

We elucidated the distribution of interstitial cells of Cajal (ICC) in human stomach, using cryosection and c-Kit immunohistochemistry to identify c-Kit positive ICC. Before c-Kit staining, we routinely used hematoxylin and eosin (HE) staining to identify every structure of human stomach, from mucosa to longitudinal muscle. HE staining revealed that the fundus greater curvature (GC) had prominent oblique muscle layer, and c-Kit immunostaining c-Kit positive ICC cells were found to have typical morphology of dense fusiform cell body with multiple processes protruding from the central cell body. In particular, we could observe dense processes and ramifications of ICC in myenteric area and longitudinal muscle layer of corpus GC. Interestingly, c-Kit positive ICC-like cells which had morphology very similar to ICC were found in gastric mucosa. We could not find any significant difference in the distribution of ICC between fundus and corpus, except for submucosa where the density of ICC was much higher in gastric fundus than corpus. Furthermore, there was no significant difference in the density of ICC between each area of fundus and corpus, except for muscularis mucosa. Finally, we also found similar distribution of ICC in normal and cancerous tissue obtained from a patient who underwent pancreotomy and gastrectomy. In conclusion, ICC was found ubiquitously in human stomach and the density of ICC was significantly lower in the muscularis mucosa of both fundus/corpus and higher in the submucosa of gastric fundus than corpus.


Subject(s)
Humans , Eosine Yellowish-(YS) , Gastrectomy , Gastric Fundus , Gastric Mucosa , Hematoxylin , Immunohistochemistry , Interstitial Cells of Cajal , Mucous Membrane , Muscles , Stomach
17.
Journal of the Korean Child Neurology Society ; (4): 284-291, 2010.
Article in Korean | WPRIM | ID: wpr-106681

ABSTRACT

PURPOSE: Autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD) are neurodevelopmental disorders. previous report have examined the neurologic problems both in ASD and ADHD. METHODS: We studied 49 children with ASD and 51 with ADHD who had visited our hospital's neuropsychiatric clinic from January 1999 to December 2008. We examined age, EEG abnormalities, developmental delays, and seizures in both groups. RESULTS: 1) The mean age of children diagnosed with ASD was 4 years while that for ADHD was 9 years (P=0.000). 2) The percentage of abnormal EEG in children with ASD was 54.5% and that for those with ADHD was 25.4% (P=0.027). 3) The percentage of developmental delays in children with ASD was 89.8% and that for those with ADHD was 39.2% (P=0.000). 4) The mean age of first seizure in children with ASD was 13 years and that for those with ADHD was 6 years (P=0.005). CONCLUSION: The percentage of abnormal EEG and developmental delay in children with ASD was more than in those with ADHD. The mean age of diagnosis in children with ASD was younger than in those with ADHD and the mean age of first seizure in children with ASD was older than in those with ADHD. The percentage children with ASD who exhibited normalized EEGs upon follow-up was greater than that for children with ADHD.


Subject(s)
Child , Humans , Attention Deficit Disorder with Hyperactivity , Autistic Disorder , Autism Spectrum Disorder , Developmental Disabilities , Electroencephalography , Epilepsy , Follow-Up Studies , Seizures
18.
Journal of the Korean Child Neurology Society ; (4): 123-130, 2009.
Article in Korean | WPRIM | ID: wpr-121635

ABSTRACT

PURPOSE: Alternating hemiplegia of childhood(AHC) is a rare neurologic syndrome of unknown pathophysiology. The typical clinical features are characterized by recurrent hemiplegic attacks shifting from one side to the other, beginning before 18 months, dystonia, strabismus, nystagmus, epilepsy, and immediate resolution of all symptoms during sleep. This study is to access clinical features and outcome of treatment of AHC in Korea. METHODS: We sent standardized questionnaires by e-mail to 118 council members of the Korean Society of Child Neurology. We received it through e-mail from 7 centers and analyzed clinical characteristics, treatment, and outcomes. RESULTS: We collected 11 AHC patients(male 6, female 5). The mean age was 6.13+/-5.23 years. The mean age of onset of hemiplegia was 9.80+/-6.67 months. The mean frequency of episode was 10.73+/-11.44 per month and mean duration was 3.10+/-2.50 days. The manifestations were hemiplegia in 100%, dystonia in 81.8%, abnormal eye movements in 81.8%, developmental delay in 81.8%, seizure in 72.7%, autonomic phenomena in 54.5%, ataxia 45.5%, and episodes of quadriparesis in 45.5%. The onset age of seizure was 3-84 months. Psychomotor retardation was more severe in patients with seizures than in those without seizures. Magnetic resonance imaging of brain and cerebral angiography were normal in all patients. Interictal SPECT shows hypoperfusion in the frontal in 2 of 5 patients. Nine patients received flunarizine and antiepileptic drugs but two patients received antiepileptic drugs only. A favorable response was reported in 45.5%, which included excellent response to the combination therapy of flunarizine and high dose topiramate. CONCLUSIONS: Patients with seizures faired the poorer psychomotor retardation. The combination of flunarizine and high dose topiramate was very effective in AHC.


Subject(s)
Child , Female , Humans , Age of Onset , Anticonvulsants , Ataxia , Brain , Cerebral Angiography , Dystonia , Electronic Mail , Epilepsy , Eye Movements , Flunarizine , Fructose , Hemiplegia , Korea , Magnetic Resonance Imaging , Neurology , Quadriplegia , Surveys and Questionnaires , Seizures , Strabismus , Tomography, Emission-Computed, Single-Photon
19.
Journal of the Korean Child Neurology Society ; (4): 167-173, 2009.
Article in Korean | WPRIM | ID: wpr-121630

ABSTRACT

PURPOSE: Reversible posterior leukoencephalopathy syndrome(RPLS) is a complex condition affecting gray/white matter of parieto-occipital lobes from multiple and different etiologies. This study was aimed to analyze clinical characteristics of this condition in Korean children. METHODS: A total of 36 patients from tertiary care medical centers were involved in the study. They were diagnosed as RPLS mainly by clinical and radiological manifestations. Their medical records and radiological features of brain MRI were retrospectively analyzed. RESULTS: Thirty six patients were involved in the study(21 males and 15 females, mean age: 9.8+/-4.1 years of age). Main underlying causes included drug induced(47%), renal diseases(28%), neurogenic tumors(8%) and so on. Initial manifestations were seizures, headache, visual disturbance, mental change, and nausea/vomiting. 13 patients(36%) had two or more symptoms. The brain MRI showed typical pattern of fairly symmetric, high T2 signal intensity in both parieto-occipital regions in 23 patients(64%) and various, atypical pattern in 13 patients(36%). 26 patients had the lesions affecting predominantly white matter, but 10 patients had the lesions affecting predominantly gray matter. 34 patients(94 %) had bilateral lesions, but 2 patients(6%) had unilateral lesion. This complex syndrome was associated with acute rise of blood pressure from a variety of conditions. A patient ended up a visual deficit despite the good prognosis. CONCLUSION: RPLS in Korean children seems to be complex, but a better understanding of this complex syndrome will lead to better clinical outcome by avoiding unnecessary investigations and appropriate management.


Subject(s)
Child , Female , Humans , Male , Blood Pressure , Brain , Headache , Leukoencephalopathies , Medical Records , Posterior Leukoencephalopathy Syndrome , Retrospective Studies , Seizures , Tertiary Healthcare
20.
Journal of the Korean Child Neurology Society ; (4): 221-225, 2009.
Article in Korean | WPRIM | ID: wpr-121623

ABSTRACT

Neurofibromatosis type I is one of the most common neurocutaneous syndrome which is inherited by autosomal dominant manner, characterized by cafe au-lait spots, axillary freckling, Lisch nodules in iris, multiple neurofibromas and bone involvement with pseudoarthrosis, bowing of the long bone. And Moyamoya disease is a specific disease characterized by progressive idiopathic stenosis and eventual occlusion of the large cerebral arteries at the circle of Willis. In response to the stenosis, an abnormal network of small collateral vessels develops, creating the "puff of smoke". Intracranial lesions associated with Neurofibromatosis type I include optic glioma, sphenoid wing dysplasia, "unidentified bright objects" and cerebrovascular lesions such as Moyamoya syndrome and aneurysm. Moyamoya syndrome is an uncommon association of neurofibromatosis type 1 and lung mass has not frequently been found with neurofibromatosis and moyamoya syndrome. We report a case with Neurofibromatosis type I with Moyamoya syndrome and ganglioneuroma in lung with reviewing literatures.


Subject(s)
Aneurysm , Cerebral Arteries , Circle of Willis , Constriction, Pathologic , Ganglioneuroma , Iris , Lung , Moyamoya Disease , Neurocutaneous Syndromes , Neurofibromatoses , Neurofibromatosis 1 , Optic Nerve Glioma , Pseudarthrosis
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