Headache associated with moyamoya disease (HAMD) has been reported in about 20-30% of pediatric cases with moyamoya disease. However, the characteristic patterns of HAMD during disease progression are not completely known, although much is known on the incidence of HAMD and on the treatment effects of bypass surgery. In a child who presented with HAMD and had no infarction or hemorrhage till bypass surgery, we described the characteristic patterns of headache progression. At first presentation, the patient complained of severe bilateral headache despite mild stenosis only in the left internal carotid artery on brain magnetic resonance angiography. During all stages of progression, headache was severe and pressing in nature rather than throbbing. Nausea and vomiting were present even in the early stage, but photophobia, phonophobia and neck stiffness developed in the late stage. Headache was aggravated by menstruation and was relieved by analgesics and topiramate in the early stage, although the medication failed to provide relief when the headache increased in frequency and duration.
Analgesics ; Brain ; Carotid Artery, Internal ; Child* ; Constriction, Pathologic ; Disease Progression ; Female ; Headache* ; Hemorrhage ; Humans ; Hyperacusis ; Incidence ; Infarction ; Magnetic Resonance Angiography ; Menstruation ; Moyamoya Disease* ; Nausea ; Neck ; Photophobia ; Vomiting

Ophthalmoplegia without ataxia has various etiologies. An atypical Miller Fisher syndrome implies an ophthalmoplegia without ataxia, areflexia or both. The presence of anti-GQ1b antibody supports the diagnosis of an atypical Miller Fisher syndrome. A 4-year-old Russian girl visited our hospital because of acute bilateral abducens nerve palsy and mydriasis. Although the muscle power of extremities was normal and she didn't show an ataxia, the deep tendon reflex of both knees and ankles was absent. The results of nerve conduction study and cerebrospinal fluid analysis were normal. Magnetic resonance imaging (MRI) showed an enhancement of the bilateral abducens nerve. The anti-Gq1b antibody titer was elevated. The diagnosis of atypical Miller Fisher syndrome was made and a therapy with intravenous immunoglobulins led to the clinical recovery. We report a girl with atypical Miller Fisher syndrome with acute bilateral abducens nerve palsy and mydriasis, diagnosed by of anti-GQ1b antibody positivity.
Abducens Nerve ; Abducens Nerve Diseases* ; Ankle ; Ataxia ; Cerebrospinal Fluid ; Child, Preschool ; Diagnosis ; Extremities ; Female ; Humans ; Immunoglobulins, Intravenous ; Knee ; Magnetic Resonance Imaging ; Miller Fisher Syndrome ; Mydriasis* ; Neural Conduction ; Ophthalmoplegia ; Reflex, Stretch

PURPOSE: Cytokines play important roles on the expression of various neuronal inflammatory disease and insults. The purpose of this study was to evaluate the levels of interleukine (IL)-6, IL-8, IL-10 in cerebrospinal fluid (CSF) in children with aseptic meningitis and compare them with those of the patients having other acute neurological symptoms. METHODS: We retrospectively reviewed the medical records of the children who admitted in the pediatric department of Hanyang University Guri Hospital for acute neurological symptoms and had CSF examinations from September 2012 to July 2013. We classified them into six groups as acute encephalopathy, epilepsy, febrile convulsion, headache, infantile fever, and meningitis. We analyzed the clinical and laboratory data from them. RESULTS: A total of 87 CSFs of the patients were available. The levels of CSF IL-6, IL-8, and IL-10 were significantly increased in the group with aseptic meningitis group as compared to the other groups (P<0.05). CSF IL-6 (r=0.576, P=0.000), IL-8 (r=0.329, P=0.003), and IL-10 (r=0.523, P=0.000) were all significantly correlated with CSF White bood cell (WBC) count. Among the patients with aseptic meningitis, CSF enterovirus positive patients (CSF entero+) showed significantly increased IL-6, IL-8, IL-10 levels than CSF enterovirus negative patients (CSF entero-) (P<0.05). In addition, the CSF entero+ and the increase of IL-10 were significantly correlated (x2=6.827, P=0.033). CONCLUSION: In patients with aseptic meningitis, the CSF IL-6, IL-8 and IL-10 were more expressed than in other neurological disease group. Among them, the enteroviral meningitis may be more related with IL-6, IL-8 and IL-10 expression than in other causes of aseptic meningitis.
Cerebrospinal Fluid* ; Child* ; Cytokines ; Enterovirus ; Epilepsy ; Fever ; Headache ; Humans ; Interleukin-10 ; Interleukin-6 ; Interleukin-8 ; Interleukins ; Medical Records ; Meningitis ; Meningitis, Aseptic* ; Neurons ; Retrospective Studies ; Seizures, Febrile

PURPOSE: The benefit of electroencephalography after sleep deprivation (SDEEG) in the diagnosis of epilepsy is controversial, and it is difficult to apply SDEEG to all pediatric patients in the clinical setting. This study is to figure out the usefulness of SDEEG for the detection of epileptiform discharges (EDs). METHODS: Medical records were reviewed retrospectively. Routine electroencephalography (REEG) was performed in 150 patients, and SDEEG was taken in 69 patients among the study population(n=219). The detection rates of EDs were compared between two groups, and also compared according to the presence of sleep in each group. RESULTS: The detection rate of EDs was higher in SDEEG group than in REEG group [n=69 (59.4%) vs n=150 (43.3%), P=0.03). Whereas there was no significant differences of the detection rate of EDs between REEG with and without sleep recording groups (43.8% vs 38.5%, P=0.777), there was significantly higher detection rate of EDs in SDEEG with sleep than SDEEG without sleep recording groups [n=63 (61.9%) vs n=6 (33.3%), P=0.022]. The detection rate of EDs was not significantly different between each group with and without sleep recording in overall EEGs (49.5% vs 36.8%, P=0.342). However, in overall EEGs with sleep recording, the detection rate of EDs was statistically significantly higher in SDEEG group than REEG group [n=63 (61.9%) vs n=137 (43.8%), P=0.022]. CONCLUSION: SDEEG revealed higher detection rate of EDs than REEG. It is not because of the effect of sleep recording, but of sleep deprivation itself. SDEEG is useful tool to apply for the diagnosis of epilepsy in daily clinical practice in children.
Child* ; Diagnosis* ; Electroencephalography* ; Epilepsy* ; Humans ; Medical Records ; Retrospective Studies ; Sleep Deprivation*

Human herpesvirus-6 (HHV-6) often causes mild illnesses, but is rarely associated with encephalitis or other fatal neurological conditions. We report a girl who died of a intractable status epilepticus from HHV-6. A 14-month-old girl presented with focal motor seizures in right extremities evolving to generalized convulsive status epilepticus. She had a history of mild diarrhea for 4 days and high fever for 2 days. Although she was treated with lorazepam, phenytoin, phenobarbital, and continuous midazolam infusion, generalized seizures continued for 3 hours after arrival. She became seizure free, but remained unconscious and ended up to death at the 44 days of hospitalization. The CSF HHV-6 DNA PCR turned out to be positive.
Brain Death ; Diarrhea ; DNA ; Encephalitis ; Extremities ; Fever ; Herpesvirus 6, Human ; Hospitalization ; Humans ; Infant ; Lorazepam ; Midazolam ; Phenobarbital ; Phenytoin ; Polymerase Chain Reaction ; Seizures ; Status Epilepticus ; Unconscious (Psychology)

PURPOSE: Neonatal seizures are the most prominent feature of neonatal neurologic dysfunction, and the lifespan risk for seizures is highest in the neonatal period, especially in preterm infants. This study was aimed to find and analyze the risk factors and clinical profiles of seizures in preterm infants. METHODS: Sixteen cases of preterms with seizures were retrospectively reviewed between Mar. 2009 and Feb. 2012. RESULTS: The average gestational age was 30.3+/-4.1 weeks and 11 patients (68.7%) experienced seizures within 1 week after the birth. Eight cases (50%) had a perinatal asphyxia during the delivery and 7 cases (43.8%) had a history of perinatal maternal illness. Various types of seizures were observed of which subtle seizures were the most common (50%). Electroecephalographies were performed in 12 cases, which showed abnormal findings in 9 cases (75%). Neuroimaging studies were performed in 16 cases and showed abnormal findings in 10 cases (62.5%). Thirteen patients were discharged in the improved state and 3 patients expired. Eight cases had normal outcome, while 3 cases showed developmental delay. Prognosis was unknown in 2 cases due to follow up loss. CONCLUSION: Neonatal asphyxia is the most important risk factor. Furthermore, infants with a history of perinatal maternal illness appear to be at risk for neonatal seizures. EEG is a sensitive method for assessing seizure activity and prognosis. Monitoring high risk infants with asphyxia and a history of perinatal maternal illness, and early postnatal tracing with EEG is required to detect and manage high risk preterms.
Asphyxia ; Electroencephalography ; Follow-Up Studies ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Neuroimaging ; Neurologic Manifestations ; Parturition ; Premature Birth ; Prognosis ; Retrospective Studies ; Risk Factors ; Seizures

PURPOSE: This study was aimed to evaluate the clinical features of seizures in breastfed children with vitamin D deficient rickets. METHODS: Seventeen children, breastfed and diagnosed as vitamin D deficient rickets at Inha University Hospital from January 2000 to July 2010, were retrospectively investigated. Subjects were divided into two groups according to the presence/absence of seizures. Demographic and biochemical results were compared and statistically analyzed between the two groups, and the relative risk for seizure occurrence was estimated. Clinical features of seizures were also analyzed. RESULTS: Out of the 17 subjects, nine patients (53.0%) had seizures, while eight patients (47.0%) did not. The mean age for the two groups were 4.1+/-2.0 months and 9.3+/-2.7 months, respectively, which was statistically different between the two groups (P<0.0001). Serum calcium (Ca) and 25-hydroxyvitamin D3 (25-OHD3) levels were significantly lower in the 'seizure' group (5.7+/-1.0 vs. 9.5+/-0.9 mg/dL, P<0.0001; 5.7+/-0.8 vs. 15.3+/-4.2 IU/L, P<0.0001). The relative risk for seizure occurrence was 8 times higher in hypocalcemia and 17 times higher in 25-OHD3<8 ng/mL. Seizures occurred several times as generalized or focal types, but none of them developed epilepsy nor showed developmental abnormalities later on. CONCLUSION: Seizures in breastfed children with vitamin D deficiency rickets are mainly due to hypocalcemia, which is affected by 25-OHD3 levels. Seizures may also occur more frequently in children in the stages of rapid growth. Although seizures occurred multiple times, future outcomes were favorable. Further large-scaled prospective studies are required in the future.
Breast Feeding ; Calcifediol ; Calcium ; Child ; Epilepsy ; Humans ; Hypocalcemia ; Retrospective Studies ; Rickets ; Seizures ; Vitamin D ; Vitamin D Deficiency

PURPOSE: Proliferation, differentiation, and survival of hippocampal dentate granule cells have been reported to be influenced by epileptic seizures in rodent epilepsy models. However, most studies have been done in adult rat models. This study was designed to investigate hippocampal dentate granule cell neurogenesis after pilocarpine-induced seizures in young mice. METHODS: Fifteen male ICR mice at postnatal day 21 were divided into pilocarpine-treated (n=7) and control (n=8) groups. Seizures were chemically induced by intraperitoneal injection of pilocarpine (300 mg/kg). Bromodeoxyuridine (BrdU, 50 mg/kg) was subsequently administered once a day for 6 consecutive days, starting at 24 hours after pilocarpine or saline treatment. We then examined BrdU-positive cells in the hippocampal dentate gyrus by immunohistochemistry and by double-labeled immunofluorescence with confocal microscopy. RESULTS: After pilocarpine administration, every seizure behavior was grade 3 or more. Quantitative analysis revealed that BrdU-positive cells were significantly increased in the pilocarpine-treated group compared to control (230.5+/-59.5 vs. 148.6+/-40.0, P<0.001). The majority of these mitotic cells were differentiated into neurons. CONCLUSION: Our results indicated that mitotic activity in the hippocampal dentate gyrus was enhanced after pilocarpine-induced seizures in young mice, and the majority of BrdU-positive cells showed the phenotypic differentiation to neuronal cells.
Adult ; Animals ; Bromodeoxyuridine ; Dentate Gyrus ; Epilepsy ; Fluorescent Antibody Technique ; Humans ; Immunohistochemistry ; Injections, Intraperitoneal ; Male ; Mice ; Mice, Inbred ICR ; Neurogenesis ; Neurons ; Pilocarpine ; Rats ; Rodentia ; Seizures

PURPOSE: The purpose of the study was to evaluate the efficacy and safety of rufinamide for intractable generalized epilepsies. METHODS: Eighteen patients with intractable generalized epilepsies were included in the study. Their medical records were retrospectively reviewed. Rufinamide was administered as an add-on treatment for intractable epilepsies. The initial administered dose was 10 mg/kg/day, which was subsequently titrated up to 30-50 mg/kg/day. The effectiveness was assessed by comparing the frequency of seizures after the treatment. The difference in number of seizures during 4 weeks was compared before and after reaching the final dose. RESULTS: The study population consisted of 13 males and 5 females (mean age 13.6+/-6.2 years, range 3.3-29.2 years). The responder rate (> or =50% in seizure frequency) was 39% and the seizure free rate was 11%. Retention rate was 44% and the reasons for withdrawal was adverse events (6/18 patients, 33%), aggravation of seizures (4/18 patients, 22%), and ineffectiveness (2/18 patients, 11%). Adverse events included hyperactivity, somnolence, ataxia and polyhidrosis. Adverse events and seizure aggravation occurred even at the starting dose of rufinamide treatment. CONCLUSION: Rufinamide can be used as an efficacious and safe adjunctive anticonvulsant for patients with intractable generalized epilepsy.
Ataxia ; Epilepsy ; Epilepsy, Generalized ; Female ; Humans ; Male ; Medical Records ; Retention (Psychology) ; Retrospective Studies ; Seizures ; Triazoles

PURPOSE: The developmental history is an important element in the evaluation of children with epilepsy. However, obtaining accurate information from history is often very difficult especially in older children because of their parents' incomplete recollection. This study was aimed to investigate if the timing of independent walking has a valuable clinical significance in children with epilepsy. METHODS: We classified 262 patients with epilepsy into normal and delayed groups from a history of the timing of independent walking at 18 months as a cutoff point. We compared their clinical and laboratory characteristics between two groups. RESULTS: Out of 262 patients, twenty one (8%) were delayed. There was no difference in seizure types between two groups. The Age of seizure onset and diagnosis of epilepsy was earlier in delayed group (5.6:2.2 years, P<0.001; 7.0:3.2 years, P<0.001). The proportion of Cesarean section and preterm delivery were also higher in delayed group (25%:52%, P= 0.010; 6%:29%, P<0.001). Abnormal MRI findings were more frequent in the delayed group (19%:86%, P<0.001). EEG at diagnosis showed no difference in epileptiform discharges, but background abnormality was more common in the delayed group (15%:81%, P<0.001). EEG at 1 year after the diagnosis showed both epileptiform discharges and background abnormality were more frequent in the delayed group (60%:90%, P=0.004; 14%:67%, P<0.001). CONCLUSION: Based on the result of this study, we strongly recommended that we should take a history of the timing of independent walking in children and adolescents with epilepsy.
Adolescent ; Cesarean Section ; Child ; Electroencephalography ; Epilepsy ; Female ; Humans ; Medical History Taking ; Pregnancy ; Seizures ; Walking