Meningitis is a rare and potentially serious complication in children with temporal bone fractures. We present an unusual case of a 7-year-old girl with pneumococcal meningitis complicated by cerebrospinal fluid (CSF) leakage following transverse fracture in the left temporal bone. She had an otorrhea resolved spontaneously in the early stage of hospitalization. At 4 days post-discharge, the patient returned with headache, fever, neck stiffness, voiding difficulty, and bilateral abducens nerve palsy. Magnetic resonance images demonstrated an intense uniform contrast enhancement in the cerebral cisterns and the sacral nerve roots. Laboratory analysis and culture diagnosed meningitis caused by Streptococcus pneumonia. She was discharged home after getting intravenous ceftriaxone for 5 weeks. Follow-up for the patient required constant vigilance and included a multidisciplinary approach. At 7 months after head trauma, the child was well with no neurological and auditory deficits. This case illustrates a previously unreported complication in pediatric patient of temporal bone fracture associated with CSF otorrhea.

An isolated ventricular abscess is a rare event, and its treatment is a real challenge. We report such case in a 52-year-old man that was successfully managed with aid of occipital approach and ventricular access device. This patient presented with the chief complaint of headache and fever of 3-day duration. Magnetic resonance imaging showed fluid-filled layering within the posterior horns of the lateral ventricle without contrast enhancement. Blood test and cerebrospinal fluid analysis was consistent with acute bacterial ventriculitis. Using stereotactic technique guided by electromagnetic navigation, the occipital horns were bilaterally targeted and catheterized, and then the abscess and debris was evacuated. The Ommaya reservoir implanted at the left entry was intermittently punctured for preventing the recollection. The pus culture was positive for Streptococcus pneumoniae. He received an antibiotic therapy and the reservoir aspiration leading to rapid recovery and remission of clinical manifestations. There was no evidence of the recurrence within the ventricles on follow-up scans after discharging. This modification, occipital approach and reservoir placement, is effective treatment for improving the cure rate in the selected cases with intraventricular cerebral empyema.

No abstract available.
Humans ; Peritoneal Dialysis* ; Spinal Cord*

PURPOSE: This is a clinical study to evaluate the efficacy and adverse reactions of deflazacort as adjunctive therapy in childhood intractable atonic seizure including Lennox- Gastaut syndrome. METHODS: This is a clinical prospective, add-on, and open-label study performed for 6 months from Jun. 2000 to Dec. 2000 at the pediatric neurology clinic of Severance Hospital. Subjects were selected according to the following criteria, 1) Patients were diagosed as refractory atonic seizure disorder including Lennox-Gastaut syndrome during more than 6 months, 2) Patients had been on maximal doses of at least 2 anticonvulants including sodium valproate and clonazepam or clobazam. We observed seizure frequency of 4 weeks and 24 week medication period as well as adverse reactions every 4 weeks. Those data were analysed primarily for median seizure frequency reduction rate and other efficacy variables such as responder rate with frequency reduction more than 50% and seizure free rate. We also compared the clinical aspects between responder and non responder group. RESULTS: 48 patients were evaluated for efficacy and adverse reactions. Median seizure frequency reduction rate was 42.7%, responders were 22 patients(45.8%) and seizure free patients were 4(8.3%). In Lennox-Gastaut syndrome, median seizure frequency reduction rate was 48.9% and in atonic seizure only 39.3%. However, there were no statistically significant differences in efficacy. We compared clinical aspects between respoder and non responder groups, but couldn't find any difference. The number of patients manifesting adverse reactions was 20(41.6%) in an descending order of frequency, weight gain in 16 patients(33.3%), and irritability in 4 patients(8.3%). CONCLUSION: Deflazacort is believed to be an effective and safe anticonvulsant when used as adjunctive therapy for atonic seizure including Lennox-Gastaut syndrome. However, long term follow up is required to evaluate relapse rate and its adverse reactions.
Clonazepam ; Epilepsy ; Follow-Up Studies ; Humans ; Neurology ; Prospective Studies ; Recurrence ; Seizures* ; Valproic Acid ; Weight Gain

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder. Over 75 pathogenic mutations have been identified in both the mitochondrial and nuclear genomes. Leigh syndrome can be diagnosed based on clinical manifestations, physical and biochemical examinations, and brain magnetic resonance imaging results. Patients with Leigh syndrome classically present in early childhood with developmental regression, ataxia, and hypotonia with subsequent respiratory and brainstem dysfunction. However, the clinical course of Leigh syndrome is heterogeneous with significant differences in age of onset, symptom severity and prognosis. Here, we report a case of Leigh syndrome with cardiac arrest as initial presentation, and identified the novel mutation of NDUFS1 gene.

Purpose: We report a diagnosis of Moyamoya disease based on brain magnetic resonance imaging (MRI) and brain magnetic resonance angiography (MRA) performed in a child diagnosed with morning glory syndrome.Case summary: A 6-year-old boy visited the hospital with a chief complaint of exodeviation of his left eye that started at 1 year of age. The corrected visual acuity was 20/30 in the right eye and 20/400 in the left. The Krimsky test showed exotropia of 35 prism diopters (PD) for near vision and 30 PD for distance. Fundus photography showed an enlarged optic disc with funnel-shaped excavation in the left eye. With a diagnosis of morning glory syndrome accompanied by exotropia, the patient underwent brain MRI and brain MRA. The patient had no neurological symptoms such as headache and muscle weakness. Moyamoya disease was diagnosed, as brain MRI and MRA showed tiny tortuous vessels forming collateral vessels from the lenticulostriatal arteries, left posterior cerebral artery, and left middle meningeal artery, which originated from the external carotid artery. Conclusions Moyamoya disease can occur in children with morning glory disease. Brain MRI and MRA can help to diagnose Moyamoya disease. Caution is needed because transient ischemic attack, cerebral infarction, and cerebral hemorrhage may occur in patients with Moyamoya disease.

Purpose: Temporal lobe resection can be categorized as either temporal lobe epilepsy (TLE), which involves cortical resection confined to the temporal lobe, or temporal plus epilepsy (TPE), which entails temporal resection along with involvement of additional extratemporal regions. We compared these forms within a pediatric population. Methods: We identified 136 patients who underwent temporal resection over a 17-year period and investigated the differences in the clinical profiles and seizure outcomes between TLE and TPE. Results: Of the total sample, 110 patients (80.9%) presented with TLE and 26 (19.1%) with TPE. Significant differences were observed between the groups in age at seizure onset (TLE: 6.3 years, TPE: 0.9 years; P=0.001), age at epilepsy surgery (TLE: 14.2 years, TPE: 9.2 years; P=0.002), the proportion of patients with a history of infantile epileptic spasm syndrome (IESS) (TLE: 6 [5.5%], TPE: 8 [30.3%]; P<0.001), electroclinical presentation with IESS or Lennox-Gastaut syndrome (LGS) (TLE: 11 [10.0%], TPE: 13 [50.0%]; P<0.001), the presence of focal temporal hypometabolism on positron emission tomography (TLE: 74 [68.5%], TPE: 11 [44.0%], P=0.021), and the use of intracranial electroencephalogram monitoring (TLE: 58 [52.7%], TPE: 21 [80.8%]; P=0.009). Furthermore, multivariate analysis identified the epileptic presentation of IESS or LGS as a significant predictor of TPE (P=0.049). The rates of seizure outcomes of International League Against Epilepsy class 1–3 at 1 year of follow-up were 83.8% for the entire cohort, 89.1% for TLE, and 61.5% for TPE (P=0.002). Conclusion TPE appears to represent a substantial subset of pediatric temporal resections. The variation in seizure outcomes between groups underscores the importance of predicting TPE in advance, with implications for effective treatment planning.

OBJECTIVES: The purpose of this study was to develop the Korean version of the Social Adjustment Scale II-Revised Version (KSAS II-RV) in the schizophrenics. METHODS: KSAS II-RV was administered to 67 schizophrenic patients and 78 matched normal controls for examining the reliability and validity. RESULTS: Data analysis showed statistically significant reliabilities and validities of KSAS II-RV. The test-retest reliability, inter-rater reliability, and internal consistency for total scores of KSAS II-RV were 0.85, 0.89. and 0.87, respectively. Evidence for discriminant validity of KSAS II-RV comes from the results that the mean scores of schizophrenic patients were significantly high than those of normal controls. Construct validity was assessed by calculating the 10 inter-subareas correlations of the KSAS II-RV, and all except the sexual adjustment area were statistically significant. Significant correlations between the global area scores of KSAS II-RV and those of Global Assessment of Functioning Scale lend support for the concurrent validity of this instrument indirectly. CONCLUSION: KSAS II-RV was found to be a valid and reliable instrument which can be used to evaluate the degree of social adjustment in the schizophrenic patients.
Humans ; Reproducibility of Results* ; Schizophrenia ; Social Adjustment* ; Statistics as Topic

It has been suggested that cortisol deficiency may be responsible for the cholestasis. Although congenital hypopituitarism was reported as a possible cause of neonatal cholestasis, congenital adrenal hyperplasia with neonatal cholestasis is a very rare report in the literature. We experienced a case presenting with neonatal cholestasis associated with congenital adrenal hyperplasia. This case provides a clinical evidence supporting the notion that cortisol has an important physiological role in bile acid synthesis and transport.
Adrenal Hyperplasia, Congenital* ; Bile ; Cholestasis* ; Hydrocortisone ; Hypopituitarism

PURPOSE: Neonatal strokes are common and may be associated with various complications. However, few studies have been conducted on the clinical spectrum in Korea. This study aimed at investigating the clinical presentation and neurological outcome of neonatal strokes. METHODS: Twenty-seven neonates with neonatal stroke were enrolled in the neonatal intensive care unit at Kyungpook National University Hospital from January 2000 to December 2009. Their medical records and neuroradiological findings were retrospectively reviewed. RESULTS: The mean age of the subjects was 4+/-5.6 days. Sixteen patients were full term, nine were prematurite and six had low birth weights. The onset of symptoms was mostly within first week (85.2%) of life, especially in the first day of life (51.9%). The most common symptom was seizure (40.7%), which were focal clonic (38.5%) or multifocal clonic (38.5%). Nine patients showed abnormal EEG findings. Thirteen patients had subdural hemorrhage, seven showed intraventricular hemorrhage, and three revealed cerebral infarction. Among 12 patients who followed-up for one year, four had mild neurologic dysfunction and two had severe impairment. CONCLUSION: We found that the onset of symptom in neonatal strokes was mostly within the first day of life, and the most common symptom was focal seizure. We, therefore recommend that neuroimaging be done when newborns have seizures within their first week of life. However, further studies are needed to elucidate this further.
Cerebral Infarction ; Electroencephalography ; Hematoma, Subdural ; Hemorrhage ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Infarction ; Intensive Care, Neonatal ; Korea ; Medical Records ; Neuroimaging ; Neurologic Manifestations ; Retrospective Studies ; Seizures ; Stroke