• Title/Summary/Keyword: seizures

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Long-term outcomes of infantile spasms (영아 연축 환아의 장기적 예후에 관한 고찰)

  • Oh, Seak Hee;Lee, Eun-Hye;Joung, Min-Hee;Yum, Mi-Sun;Ko, Tae-Sung
    • Clinical and Experimental Pediatrics
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    • v.53 no.1
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    • pp.80-84
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    • 2010
  • Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

The characteristics of neurocardiogenic syncope in children (소아 신경-심인성 실신의 특징)

  • Lee, Kyung Yeon;Lee, Jin Young;Kim, Myoung Hyun;Lee, Jeong Eun;Kim, Yong Dae;Lee, Eun Ju;Lim, Young Su;Kim, Won Seop
    • Clinical and Experimental Pediatrics
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    • v.51 no.5
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    • pp.512-517
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    • 2008
  • Purpose : Syncope is relatively common in children and adolescents. Among the etiologies of syncope, neurocardiogenical syncope is the most common, but it is often confounded with seizure. We investigated the clinical features of patients under the age of 15 with neurocardiogenical syncope, compared to patients with neurologic disorders including epilepsy. Methods : Among the children who visited the Department of Pediatrics at Cheongju St. Mary's Hospital and Chungbuk National University Hospital from March 2005 to February 2007, we retrospectively analyzed 69 patients whose chief complaint was syncope. We classified the patients by syncope etiology and made comparisons between the neurocardiogenical syncope (NCS) group and the neurological disorders (ND) group regarding to age; location; time and season in which syncope occurred; associated symptoms, including seizures, provocation factors; prodromes; duration of syncope; frequency of previous syncope; birth history; associated disease; past medical history; family history; neurological exam; physical exam; laboratory findings; electrocardiography; electroencephalography (EEG); head-up tilt test; brain CT; and MRI. Results : Among 69 syncope patients, 53 (76.8%) were in the NCS group and 11 (15.9%) were in the ND group. There were no statistically significant differences between the two study groups except for the presence of prodromes and EEG abnormalities. The presence of prodromes in the NCS group was more common than in the ND group [46.9% (23/49) vs. 9.1% (1/11), (P=0.038)]. The EEG abnormality in the ND group was more common than in the NCS group [90% (9/10) vs. 5.8% (3/52), (P<0.01)]. Conclusion : Our study suggests that detailed history-taking, including that concerning prodromes, is important for the accurate diagnosis of neurocardiogenical syncope, and EEG should be obtained if neurological disorders are suggested.

A Case of Adult-onset Type II Citrullinemia Confirmed by Mutation of SLC25A13 (SLC25A13 유전자 돌연변이로 확진된 성인형 제 2형 시트룰린혈증 1례)

  • Jeung, Min Sub;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Lee, Heon Ju;Jin, Dong-Kyu;Cho, Sung Yoon
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.1
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    • pp.34-41
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    • 2016
  • Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

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Clinical Features of Cricopharyngeal Incoordination in Newborns and Infants (신생아 및 영아기 윤상인두 협조불능의 임상적 고찰)

  • Han, Young-Mi;Park, Jae-Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.116-121
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    • 2008
  • Purpose: Cricopharyngeal incoordination is a rare cause of swallowing difficulties in newborns and infants; it is characterized by delayed pharyngeal contractions related to cricopharyngeal relaxation. Dysphagia and repeated aspiration are common findings despite normal sucking. We conducted this study to assess the clinical features of cricopharyngeal incoordination in newborns and infants. Methods: An analysis of the clinical data from 17 patients with cricopharyngeal incoordination who were admitted to the Department of Pediatrics, Pusan National University Hospital, between 2000 and 2006 was conducted retrospectively. The diagnosis of cricopharyngeal incoordination was established by the clinical characteristics and the videofluoroscopic swallowing studies. Results: The male to female ratio was 1:1.1 (males 8, females 9) the age range 1 to 60 days. The body weight of 11 patients (64.7%) was less than the $10^{th}$ percentile at diagnosis. Six patients (35.3%) were born prematurely. The associated anomalies or diseases were chromosomal anomaly (2 cases), congenital heart disease (3 cases), and laryngomalacia, hypoxic brain damage or neonatal seizures (1 case each). The chief complaints of patients were recurrent aspiration pneumonia (10 cases), feeding difficulty (9 cases), dyspnea (4 cases), and chocking (4 cases). The severity of aspiration on the videoesophagogram or esophagogram was mild in 12 cases. The correlation between the severity of aspiration and the duration of tube feeding after the diagnosis was significant (p<0.05). Conclusion: Cricopharyngeal incoordination should be considered in the differential diagnosis of newborns and infants, without known risk factors associated with swallowing dysfunction, when they present with unexplained respiratory problems. Although the prognosis of cricopharyngeal incoordination is good, early diagnosis and tube feeding are recommended to prevent the complications associated with this disorder.

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Human Parechovirus as an Important Cause of Central Nervous System Infection in Childhood (소아청소년기 중추신경 감염의 주요 원인으로서 Human Parechovirus의 의의)

  • Jung, Hyun Joo;Choi, Eun Hwa;Lee, Hoan Jong
    • Pediatric Infection and Vaccine
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    • v.23 no.3
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    • pp.165-171
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    • 2016
  • Purpose: Human parechovirus (HPeV) is an increasingly recognized pathogenic cause of central nervous system (CNS) infection in neonates. However, HPeV infections have not been studied in older children. This study determined the prevalence and clinical features of HPeV CNS infection in children in Korea. Methods: Reverse transcription polymerase chain reaction assays were performed using HPeV-specific, 5' untranslated, region-targeted primers to detect HPeV in cerebrospinal fluid (CSF) samples from children presenting with fever or neurologic symptoms from January 1, 2013, to July 31, 2014. HPeV genotyping was performed by sequencing the viral protein 3/1 region. Clinical and laboratory data were retrospectively abstracted from medical records and compared with those of enterovirus (EV)-positive patients from the same period. Results: Of 102 CSF samples, six (5.9%) were positive for HPeV; two of 21 EV-positive samples were co-infected with HPeV. All samples were genotype HPeV3. Two HPeV-positive patients were <3 months of age and four others were over 1 year old. While HPeV-positive infants under 1 year of age presented with sepsis-like illness without definite neurologic abnormalities, HPeV-positive children over 1 year of age presented with fever and neurologic symptoms such as seizures, loss of consciousness, and gait disturbance. The CSF findings of HPeV-positive patients were mostly within the normal range, whereas most (73.7%) EV-positive patients had pleocytosis. Conclusions: Although HPeV is typically associated with disease in young infants, the results of this study suggest that HPeV is an emerging pathogen of CNS infection with neurologic symptoms in older childhood.

The Findings and Significances of Brain SPECT in Acute Mealses Encephalitis (급성 홍역 뇌증 환아들의 뇌 SPECT 소견과 유용성)

  • Kim, Jung Chul;Choung, Ju Mi;Eun, So Hee;Lee, Dae-Yeol;Kim, Jung Soo
    • Clinical and Experimental Pediatrics
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    • v.45 no.11
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    • pp.1373-1380
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    • 2002
  • Purpose : Acute measles encephalitis(ME) is characterized by an abrupt onset of fever and obtundation, frequently accompanied by seizures and multifocal neurological signs. The aim of this study was to clarify the clinical manifestation, progression and the brain SPECT patterns in patients with acute ME. Methods : This study included 11 children with acute ME admitted to Chonbuk National University Hospital. Ten patients received a first dose of measles vaccine, one patient did not receive a first dose, and no patients received a second dose. ME was diagnosed based on characteristic clinical pictures, measles antibodies by ELISA and abnormal CSF findings. Brain MRI and brain SPECT were performed in 11 patients with acute ME. Results : There were four males and seven females whose ages at onset ranged between 18 months and 14 years(mean : 10.5 years). The main clinical neurologic pictures were loss of consciousness( 10) and seizure(five). The titer of IgG and IgM antimeasles antibodies in serum were positive in 10 patients. In CSF, nine patients had IgG antibodies and one patient had IgM antibodies. The concentration of protein(mean : $124{\pm}60mg/dL$) and WBC counts(mean : $158{\pm}157/{\mu}L$) in CSF were elevated in all patients. In electroencephalographic examination, nine patients showed increased slow waves. Seven of 11 patients(63.6%) revealed high signal intensity on the brain MRI. In contrast, all patients showed hypoperfusion in brain SPECT examination. According to brain SPECT, the perfusion deficits were frequently observed in the frontal lobe(nine), temporal (nine), parietal(eight) and thalamus(eight). Conclusion : Brain SPECT is more sensitive than MRI for the evaluation of brain damage in early stages of acute ME.

Lumbar Puncture in Patients Who Experienced First Seizure with Fever (열이 동반된 첫 경련시의 뇌척수액검사)

  • Kim, Jung;Lee, Jun Su;Lee, Sang Eun;Choi, Wan Suk;Han, Sung Ryoung;Cho, Kyunglae
    • Clinical and Experimental Pediatrics
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    • v.46 no.10
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    • pp.1008-1012
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    • 2003
  • Purpose : We aimed to ascertain whether lumbar punctures should be done to prove febrile seizure, and to study what conditions can delay or abolish lumbar puncture. Methods : This retrospective study was done for four years, from January, 1996 to December, 1999. The subjects were the patients who visited Masan Samsung Hospital for the first convulsion with fever, and whose age ranged from 1-month to 5-years old. The total number of patients was 197. We investigated the rate of meningitis, which was classified by age, season, convulsion type, associated symptoms, and results of blood test. All data were tested chi-square. Results : The diagnosis rate of meningitis among all 197 patients was 7.6%, and of these only one patient was proved to have bacterial meningitis. The rate was high in the young(below 6 month), in males and in summer and autumn. Although the general appearance was good after resolution of the fever, the rate of meningitis was 6.5%. Patients with nausea and vomiting totalled 11%; 9.1% of them were diagnosed with meningitis. The diagnosis rate of meningitis in cases in which additional seizures took place were 33.3%. Conclusion : The ultimate goal of lumbar punctures in seizure patients with accompanying fever is to rule out bacterial meningitis, which, has been demonstrated to be rare. So we think that lumbar puncture can be postponed by close observation of pediatrics specialists and skilled nurses.

Change of interictal epileptiform discharges after antiepiletic drug treatment in childhood epilepsy (소아 간질 환자에서 항경련제 치료 후 간질파의 변화)

  • Kim, Mun-Ju;Nam, Sang-Ook
    • Clinical and Experimental Pediatrics
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    • v.53 no.4
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    • pp.560-564
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    • 2010
  • 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, 6.79 (3.40) years; duration of therapy, 2.48 (1.85) years] diagnosed with epilepsy at the Department of Pediatrics, Pusan National University Hospital between January 2001 and December 2007, who received AEDs for more than 6 months. EEG was performed at the intervals of 6-12 months. We divided patients into 4 groups according to IED detection before and after AEDs treatment. Related clinical factors, including gender, age at the start of treatment, seizure type, cause of seizure, AED frequency, seizure control, duration of AED therapy, and background activity were investigated in the 4 groups. Results : Generalized epilepsy was relatively frequen in patients who did not show IEDs in last follow-up EEG. There were no clinically significant differences according to gender, age at the start of treatment, cause of seizure, AED frequency, seizure control, duration of AED medication, and background activity in the 4 groups ($P$>0.05). Conclusion : IEDs changed after AED treatment in one-third of the patients. Generalized epilepsy is positive factor for negative IEDs in last follow-up EEG.

Outcome of LINAC Radiosurgery for a Cavernous Angioma (해면상혈관종에 대한 선형가속기를 이용한 고선량 정위 방사선수술의 임상경험)

  • Hong Semie;Chie Eui Kyu;Park Suk Won;Kim Il Han;Ha Sung Hwan;Park Charn Il
    • Radiation Oncology Journal
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    • v.21 no.2
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    • pp.107-111
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    • 2003
  • Purpose: To establish the role of stereoactic radiosurgery using a linear accelerator for the treatment of patients with cavernous angloma. Materials and Methods: Between February 1995 and May 1997, 11 patients with cavernous angioma were treated with stereotactic radiosurgery using a linear accelerator. Diagnoses were based on the magnetic resonance imaging in 8 patients, and the histological in 3. The vascular lesions were located on the brainstem (5 cases), cerebellum (2 cases) thalamus (1 case) and cerebrum (3 cases). The clinical presentation at onset included previous intracerebral hemorrhages (9 cases) and seizures (2 cases). All patients were treated with a a linac-based radiosurgery. The median dose of radiation delivered was 16 Gy ranging from 14 to 24 Gy, which was typically proscribed to the 80$\%$ isodose surface (range 50 $\~$ 80$\%$), corresponding to the periphery of the lesion with a single isocenter. Ten patients were followed-up. Results: The median follow-up was 49 months ranging from 8 to 73 months, during which time two patients developed an intracerebral hemorrhage, 1 at 8 months, with the other at 64 months post radiosurgery. One patient developed neurological deficit after radiosurgery, and two developed an edema on the T2 weighted images of the MRI surrounding the radiosurgical target. Conclusion: The use of stereotactic radiosurgery in the treatment of a cavernous angioma may be effective in the prevention of rebleedlng, and can be safely delivered. However, a longer follow-up period will be required.

Supraclavicular Brachial Plexus block with Arm-Hyperabduction (상지(上肢) 외전위(外轉位)에서 시행(施行)한 쇄골상(鎖骨上) 상완신경총차단(上腕神經叢遮斷))

  • Lim, Keoun;Lim, Hwa-Taek;Kim, Dong-Keoun;Park, Wook;Kim, Sung-Yell;Oh, Hung-Kun
    • The Korean Journal of Pain
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    • v.1 no.2
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    • pp.214-222
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    • 1988
  • With the arm in hyperabduction, we have carried out 525 procedures of supraclavicular brachial plexus block from Aug. 1976 to June 1980, whereas block with the arm in adduction has been customarily performed by other authors. The anesthetic procedure is as follows: 1) The patient lies in the dorsal recumbent position without a pillow under his head or shoulder. His arm is hyperabducted more than a 90 degree angle from his side, and his head is turned to the side opposite from that to be blocked. 2) An "X" is marked at a point 1 cm above the mid clavicle, immediately lateral to the edge of the anterior scalene muscle, and on the palpable portion of the subclavian artery. The area is aseptically prepared and draped. 3) A 22 gauge 3.5cm needle attached to a syringe filled with 2% lidocaine (7~8mg/kg of body weight) and epineprine(1 : 200,000) is inserted caudally toward the second portion of the artery where it crosses the first rib and parallel with the lateral border of the muscle until a paresthesia is obtained. 4) Paresthesia is usually elicited while inserting the needle tip about 1~2 em in depth. If so, the local anesthetic solution is injected after careful aspiration. 5) If no paresthesia is elicited, the needle is withdrawn and redirected in an attempt to elicit paresthesia. 6) If, after several attempts, no paresthesia is obtained, the local anesthetic solution is injected into the perivascular sheath after confirming that the artery is not punctured. 7) Immediately after starting surgery, Valium is injected for sedation by the intravenous route in almost all cases. The age distribution of the cases was from 11 to 80 years. Sex distribution was 476 males and 49 females (Table 1). Operative procedures consisted of 103 open reductions, 114 skin grafts combined with spinal anesthesia in 14, 87 debridements, 75 repairs, i.e. tendon (41), nerve(32), and artery (2), 58 corrections of abnormalities, 27 amputations above the elbow (5), below the elbow (3) and fingers (17), 20 primary closures, 18 incisions and curettages, 2 replantations of cut fingers. respectively (Table 2). Paresthesia was obtained in all cases. Onset of analgesia occured within 5 minutes, starting in the deltoid region in almost all cases. Complete anesthesia of the entire arm appeared within 10 minutes but was delayed 15 to 20 minutes in 5 cases and failed in one case. Thus, our success rate was nearly 100%. The duration of anesthesia after a single injection ranged from $3\frac{1}{2}$ to $4\frac{1}{2}$, hours in 94% of the cases. The operative time ranged from 0.5 to 4 hours in 92.4% of the cases(Table 3). Repeat blocks were carried out in 33 cases when operative times which were more than 4 hours in 22 cases and the others were completed within 4 hours (Table 4). Two patients of the 33 cases, who received microvasular surgery were injected twice with 2% lidocaine 20 ml for a total of $13\frac{1}{2}$ hours. The 157 patients who received surgery on the forearms or hands had pneumatic tourniquets (250 torrs) applied without tourniquet pain. There was no pneumothorax, hematoma or phrenic nerve paralysis in any of the unilateral and 27 bilateral blocks, but there was hoarseness in two, Horner's syndrome in 11 and shivering in 7 cases. No general seizures or other side effects were observed. By 20ml of 60% urcgratin study, we confirm ed the position of the needle tip to be in a safer position when the arm is in hyperabduction than when it is in adduction. And also that the humoral head caused some obstraction of the distal flow of the dye, indicating that less local anesthetic solution would be needed for satisfactory anesthesia. (Fig. 3,4).

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