• Journal of Korean Neurosurgical Society
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• v.55 no.6
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• pp.375-378
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• 2014

Hydranencephaly is a rare congenital disease defined as an absence of cerebral hemispheres with an intact thalamus, brain stem, and cerebellum. Generally, patients with hydranencephaly require cerebro-spinal fluid diversion due to progressive hydrocephalus. An excellent view of the choroid plexus and advancement of the neurosurgical endoscope were possible, leading to easy coagulation of the choroid plexus to balance cerebro-spinal fluid production without the use of a shunt device. We present this rare case and good treatment outcome from endoscopic coagulation of the choroid plexus.

• Korean Journal of Veterinary Research
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• v.20 no.1
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• pp.65-78
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• 1980

From November 1978 to April 1979, there occurred among cows many cases of abortion, premature birth, stillbirth and congenital arthrogryposis or hydranencephaly in Kyongki-do province in Korea. Epizootics recurred between February and May, 1980 in the same area. Six calves born at 8 or 10 months of gestation were examined by means of pathology and one of the calves was examined by serological test. And a survey on local occurrence of the disease in other than Kyongki-do province, was conducted. 1. At necropsy, some calves showed distinct lesions such deformities of the limbs and the vertebrae as arthrogryposis or scoliosis. Other calves revealed arthrogryposis and partial or total deficience of the cerebral hemispheres with hydranencephaly. 2. In the calves with deformities the skeletal muscle showed histologically severe hypoplasia of the muscle fibers with cellular infiltration. In the central nervous system of the calves with hydranencephaly there were minute cystic cavitation and the decrease of ventral horn cells of the spinal cord in the number. 3. Similar epizootics were also observed in the same season in the other several provinces such as Chungoheongnam-do, Kangwon-do and Jeonrabuk-do. 4. Anti-Akabane virus antibody was detected in precolostral blood from the calf with arthroglposis and hydranencephaly syndrome. 5. On these findings of the disease it was diagnosed as Akabane disease presenting the first report on the epizootics in Korea.

• Korean Journal of Veterinary Service
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• v.14 no.1
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• pp.19-26
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• 1991

A considerably high rate of abnormal deliveries of unknown etiology was observed among dairy cattles from November 1988 to February 1989 and Korean native tattles from January to April 1990. The abnormal deliveries consisted of abortions, stillbirths and calf deformities refers to as congenital arthrogryposis hydranencephaly (AH )syndrome. In order to know the level of Akabane antibody of dairy cattle raised in Kyungbuk province, serum neutralization test was conducted with Akabane virus(OBE-1 strain) and HmLu(Hamster lung) cell line. The results were summarized as follows. 1. During 4 months(Nov. 1988-Feb. 1989), abortion (3 heads), stillbirth(1 head) and congenital abnormalities(13 heads) of newborn were occurred in 17 dairy cattles raised in Kyungbuk province. 2. During 4 months(Jan.-Apr.1990), stillbirth(2 heads) and congenital deformities (13 heads) of newborn were occurred in 15 Korean native tattles raised in Kyungbuk province. 3. In Fev, and Apr. 1990, 1,005 dairy cattles at 99 farms were investigated on the actual condition of possessing Akabane antibody. The result was that 1,000 heads (99.9%) in 1,005 dairy cattles reacted as positive condition in Akahane antibody. The antibody titer was from 4 to over 256. 4. 189 heads (18.8%) of 1,005 dairy cattles werw below antibody titer 8 and 816 heads (81.2%) were over 16. 5. Akabane antibody titer of east coast legions(Pohang Yeongil etc) was all over 16, that of internal legions (Yeongiu, Andong. etc) was relatively low, The result suggest that the vaccination for Akabane disease will be unnecessary for the time being because of possessing higher antibody titer reaction except the newly introduced cattle and Akabane virus was widely disseminated in kyungbuk province during the summer months in 1987 or 1988.

• Korean Journal of Veterinary Service
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• v.14 no.2
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• pp.154-158
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• 1991

To investigate the Akabane antibody in the cattle with the serological test in Chung Chung Buk Do from May to Nov 1191. The result are summarized as follows. 1. Breed in cattle reacted as positive condition in Akabane antibody 76 heads(42%) in 180 cattles reacted as positive condition in Akabane antibody, 23 heads(51%) in 45 Korea native cattle reacted as positive condition in Akabane antibody. 2. During 5, 9, 10, 11 month, Akabane antibody in cattle is over 45%. 3. Less of 2 years old and over 4 years old cattle are Akabane antibody in cattle is over 40%. 4. The relation of titer of 2 folds of dilution HA and 10 folds of dilution TCID$_{50}$ was same relation.n.

• Korean Journal of Veterinary Service
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• v.23 no.1
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• pp.93-102
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• 2000

In this experiment, we studied the sero-positive rate of akabane virus in cattle from Jeju-do and analyzed the seroepidemiological features. In an analysis of 1,051 samples, the positive rate for neutralizing antibody in sera collected in nine regions on Jeju-do was 56.7%. The rate varied with the region. The positive rate was 69.6% in Aewol, 63.1% in Jeju city, 54.4% in Anduck, 51.0% in Hallim, 69.8% in Jocheun, 47.6% in Pyosun, 40% in Daejeong, 30.0% in Harkyung, 71.6% in Namwon, 24.5% in Sungsan, 133.,3% in Seokypo and 44.5% in Gujwa, respectively The rate also depended on the age of the cattle. The positive rate was 67.2% in calves 0- to 12-month old, 48.3% in cattle 13- to 24-month old, 65.4% in cattle 25- to 36-month old, and 65.4% in cattle more than ,B7 months old. To isolate the virus from calves with malformations including arthrogryposis and hydranencephaly, cerebral homogenates were inoculated into Vero cells, which were determined for cytopathic effect (CPE). Vero cells with CPE were examined for Akabane virus using an electron microscope (EM) and indirect immunofluorescent antibody test (EM). Typical virus particles with a width of 90-130nm and specific immunofluorescence in the cytoplasm of infected cells were sought for identification.

• Clinical and Experimental Pediatrics
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• v.60 no.9
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• pp.282-289
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• 2017

Purpose: Recent advancements in molecular techniques have greatly contributed to the discovery of genetic causes of unexplained developmental delay. Here, we describe the results of array comparative genomic hybridization (CGH) and the clinical features of 27 patients with global developmental delay. Methods: We included 27 children who fulfilled the following criteria: Korean children under 6 years with global developmental delay; children who had at least one or more physical or neurological problem other than global developmental delay; and patients in whom both array CGH and G-banded karyotyping tests were performed. Results: Fifteen male and 12 female patients with a mean age of $29.3{\pm}17.6months$ were included. The most common physical and neurological abnormalities were facial dysmorphism (n=16), epilepsy (n=7), and hypotonia (n=7). Pathogenic copy number variation results were observed in 4 patients (14.8%): 18.73 Mb dup(2)(p24.2p25.3) and 1.62 Mb del(20p13) (patient 1); 22.31 Mb dup(2) (p22.3p25.1) and 4.01 Mb dup(2)(p21p22.1) (patient 2); 12.08 Mb del(4)(q22.1q24) (patient 3); and 1.19 Mb del(1)(q21.1) (patient 4). One patient (3.7%) displayed a variant of uncertain significance. Four patients (14.8%) displayed discordance between G-banded karyotyping and array CGH results. Among patients with normal array CGH results, 4 (16%) revealed brain anomalies such as schizencephaly and hydranencephaly. One patient was diagnosed with Rett syndrome and one with $M{\ddot{o}}bius$ syndrome. Conclusion: As chromosomal microarray can elucidate the cause of previously unexplained developmental delay, it should be considered as a first-tier cytogenetic diagnostic test for children with unexplained developmental delay.

• Korean Journal of Veterinary Research
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• v.40 no.1
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• pp.42-48
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• 2000

Akabane disease is transmitted through mosquitoes in cattle, sheep and goats. It shows congenital abnormalities including encephalomyetitis, hydranencephaly, neurogenic arthrogryposis, and deformed neonatal calves. Akabane viruses, 93FMX and K-9 strain, were isolated from fetal matrix of aborted cow and blood of healthy cow, respectively. S gene sequences of 93FMX and K-9 showed 100% homology with that of OBE-1 strain isolated in Japan. Based upon our sequencing data, we synthesized specific primers for PCR diagnosis. Using these primers, we were able to amplify the S gene of Akabane virus not only from the culture fluid of Vero cells but also from the brain tissue of suckling mouse inoculated with, Akabane virus. These PCR products were confirmed by Southern blot hybridization. Not only the sensitivity of PCR test was high enough to detect the viruses of $10^{1.0}TCID_{50}/ml$, but also the time for diagnosis was significantly shorter than that of the virus isolation by tissue culture method. This method was also effective for the detection of Akabane virus in the cerebrum of fetus. RT-PCR method may be used for a useful diagnostic test of the clinical cases of Akabane disease.