The objective of this retrospective study was to investigate the prevalence of retinal diseases in dogs presented to the Veterinary Medical Teaching Hospital, Seoul National University from April 2004 to December 2005. Sixty-five dogs (120 eyes) with retinal diseases involving blindness were included in this study. Age, breed, and gender data for all breeds were collected from medical records documenting vision loss. Generalized progressive retinal atrophy (gPRA) was the most common manifestation. Other prevalent findings included sudden acquired retinal degeneration (SARD) and retinal detachment (RD). Bilateral gPRA was found in 32 dogs with a female-to-male ratio of 1 : 1. The mean $age{\pm}SD$ of all dogs in this group was $4.66{\pm}2.30$ years with a range of 3 to 12 years. Breeds with highest prevalence of gPRA were Miniature Schnauzer (24/32, $mean{\pm}SD$ age: $3.79{\pm}0.78$ yr) and Poodle (2/32, $6.50{\pm}0.71$ yr). Twelve dogs (24 eyes) were diagnosed with SARD bilateraly, ranging from 3 to 13 years of age ($mean{\pm}SD:\;6.91{\pm}2.61$ yr). There were no abnormalities in fundus of 11 dogs at presentation. Electroretinography (ERG) without anesthesia was performed in 7 dogs, and all response was totally extinguished. Retinal detachment was identified in 21 dogs (32 eyes): 11 bilateral and 10 unilateral (7 right eye, 3 left eye). The most common breed was Shih Tzu (15/21, $mean{\pm}SD$ age: $4.39{\pm}3.24$ yr). Four other breeds comprised the remaining 6 cases (8 eyes). The everall mean age of the group was: $4.18{\pm}2.89$ years (range 0.8 to 14 years) with a female-to-male ratio of 1 : 1.1 (10 females, and 11 males).
The present case study highlights the effects of a novel Comprehensive Hand Repetitive Intensive Strengthening Training (CHRIST) on morphological changes and associated upper extremity (UE) muscle strength and motor performance in a child with spastic quadriplegic cerebral palsy (CP). The Child, a 10-year-old girl with spastic quadriplegic CP, was treated with CHRIST for 60 minutes a day, five times a week, for 5 weeks. The CHRIST was designed to improve motor function and strength. Clinical tests including the modified Wolf Test, Jebsen-Taylor Hand Function Test, and Pediatric Motor Activity Log questionnaire were used to determine motor function. Ultrasound imaging was performed to determine the changes in the cross-section area (CSA) of the extensor carpi radialis (ECR) and triceps brachii (TRI). Muscle strength was measured with a dynamometer at pretest, and post-test, and 3-month follow-up. Ultrasound imaging data showed that the CSAs of both ECR and TRI muscles were enhanced as a function of the intervention. These changes were associated with muscle strength and motor performance and their effects remained even at a 3-month follow-up test. Our results suggest that the CHRIST was effective at treating muscle atrophy, weakness and motor dysfunction in a child with spastic quadriplegic CP.
Hippocampal atrophy is a well-established imaging biomarker of Alzheimer disease (AD). However, hippocampus is a non-homogenous structure with cytoarchitecturally and functionally distinct sub-regions or subfield, with each region performing distinct functions. Certain regions of the subfield have shown selective vulnerability to AD. Here, we are interested in studying the effects of normal aging and mild cognitive impairment on these sub-regional volumes. With a reliable automated segmentation technique, we segmented these subregions of the hippocampus in 101 cognitively normal (CN), 135 early mild cognitive impairment (EMCI), 67 late mild cognitive impairment (LMCI) and 48 AD subjects. Thereby, dividing the hippocampus into hippocampal tail (tail), subiculum (SUB), cornu ammonis 1 (CA1), hippocampal fissure (fissure), presubiculum (PSUB), parasubiculum (ParaSUB), molecular layer (ML), granule cells/molecular layer/dentate gyrus (GCMLDG), cornu ammonis 3(CA3), cornu ammonis 4(CA4), fimbria and hippocampal-amygdala transition area (HATA). In this cross sectional study of 351 ADNI subjects, no differences in terms of age, gender, and years of education were observed among the groups. Though, the groups had statistically significant differences (p < 0.05 after the multiple comparison correction) in the Mini-Mental State Examination (MMSE) scores. There was asymmetrical volume loss in the early stages of AD with the left hemisphere showing volume loss in regions that were unaffected in the right hemisphere. Bilateral parasubiculum, right cornu ammonis 1, 3 and 4, right fimbria and right HATA regions did not show any volume loss till the late MCI stages. Our findings suggest that the hippocampal subfield regions are selectively vulnerable to AD and also that these vulnerabilities are asymmetrical especially during the early stages of AD.
Kim, Ja Kyoung;Kim, Dal Hyun;Kang, Bo Young;Kwon, Young Se;Hong, Young Jin;Son, Byong Kwan;Yoon, Hye Ran
Clinical and Experimental Pediatrics
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v.46
no.1
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pp.95-99
/
2003
Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe disease is galactocerebroside beta-galactosidase. Patients with Krabbe disease can be subdivided into the early-onset type and late-onset type, according to the onset of clinical manifestations. Most patients with early-onset type die before their second birthday. We describe a girl with Krabbe disease associated with uncontrolled seizures, which was confirmed with biochemical study and MRI. The clinical findings of this patient included hyperirritability, scissoring of the legs, flexion of arm, and clenching of the fists, and generalized tonic seizures. EEG showed hypsarrhythmia, and MRI demonstrated degenerative white matter changes in bilateral periventricular white matter, posterior rim of internal capsule, basal ganglia and brain stem on T2W1 and FLAIR image. The diagnosis was based on clinical features of progressive neurologic deterioration in conjunction with low galactocerebroside beta-galactosidase activity.
Carpal Tunnel Syndrome(CTS) is a common entrapment neuropathy of the median nerve at the wrist. An Electrophysiologic study has been widely used for the diagnosis of carpal tunnel syndrome. The subjects of this study were 48 cases (88 hands) with clinically suspected carpal tunnel syndrome who underwent electrodiagnostic examination from Jan 1, 2001 to Sep 30, 2001, The results were as follows: 1. Among 48 persons with a clinically suspected carpal tunnel syndrome, 40 patients were female 83.33$\%$ and the patients who are above 60 years old were 37.50$\%$. 2. Electrodiagnostic results were 22 cases (45.84$\%$) with bilateral carpal tunnel syndrome and 10 cases (20.83$\%$) with normal. 3. Physical findings consisted of tingling sensation in 48.86$\%$ of the involved hands, positive Phalen's Sign in 20.46$\%$ of them, thenar atrophy in 15.91$\%$ of them, and weakness in 14.77$\%$ of them. 4. Electrophysiologic studies showed a decreased sensory conduction velocity in 20 cases (22.73$\%$) of total hands, a prolonged latency in 3 cases (3.41$\%$) of them, abnormal sensory and motor fiber in 33 cases (37.50$\%$) of them, and normal in 27 cases (30.68$\%$) of them. Considering above results, we had better make a diagnosis precisely the patients with clinically suspected carpal tunnel syndrome through subjective symtoms, physical examinations, and electrophysiologic studies.
Gross and histological findings were obtained on cysts formed in the reproductive organs, particularly in the mesosalpinx, mesovarium, and fimbria, of 144 gilts and 37 sows. Of the 181 gilts and sows, 63 (34.8%) were found to have cysts which totaled 89. The number of cysts totaled 38 for the gilts and 51 for the sows, with relative frequencies of 18.1% and 48.6%, respectively. The total number of cysts and the incidence were greater in the sows than in the gilts. With regard to the location of cysts, the highest incidence was found in the mesosalpinx (59.6%). The incidence of cysts in the mesovarium and fimbria was relatively low, with 16.9% and 23.6%, respectively. Most cysts were spherical or oval in shape, and below 5.9mm in diameter. A pedunculated cyst was also seen. The bilateral, incidence of the cysts was lower than unilateral, although the incidence was approxmately the same between the gilts and the sows. With regard to the sexual cycle, the high incidence of cysts was found in the follicular phase than in the luteal phase. The cysts putting pressure upon the oviduct were classified into 3 types according to the shape of epithelium and lamina propria. In type I the epithelium had ciliated cells and the lamina propria formed folds. I type 2 ciliated cells existed, but no folds were formed. In type 3, neither ciliated cells nor folds were seen, the histological change, including partial atrophy and lack of folds in the tubal mucosa were seen.
Transient ischemic attacks (TIAs) is a syndrome resulting from brain ischemia lasting less than 24 hours. The mechanisms of TIAs may be similar to those of cerebral embolism and thrombosis, and thus TIAs may be followed by cerebral infarction. Despite the availability of CT scanning, the diagnosis and management of TIAs continue to be difficult. Recently SPECT has been advocated as a diagnostic imaging modality. We performed Tc-99m-HMPAO regional cerebral blood flow (rCBF) SPECT in 24 patients with the clinical diagnosis of TIAs to assess its ability to detect early changes of rCBF, and determine the diagnostic value. Ten men and fourteen women with an average of 51 years (range; 27-74 years) were included. All but 8 patients had normal brain CT prior to SPECT. The two patients had moderate degree of brain atrophy and the 6 patients nonspecific calcifications. Eighteen of the 24 patients had abnormal Tc-99m-HMPAO rCBF SPECT. Fifteen had unilateral involvement and the other three had bilateral involvements. Seventy-five percents of the defects were found in the left cerebral hemisphere. According to the distribution of the lesions (total number: 34 lesions), fourteen were in the parietal, eight in the temporal, and the remainders were elsewhere. Tc-99m-HMPAO rCBF SPECT is sensitive in detecting rCBF abnormalities in patients with TIAs, and represent the most accurate diagnostic tool available in the diagnosis of TIAs.
Kim, Seung Ju;Nam, Soo Hyun;Kanwal, Sumaira;Nam, Da Eun;Yoo, Da Hye;Chae, Jong?Hee;Suh, Yeon?Lim;Chung, Ki Wha;Choi, Byung?Ok
Genes and Genomics
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v.40
no.12
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pp.1269-1277
/
2018
Bcl2-associated athanogene 3 (BAG3) mutations have been reported to cause the myofibrillar myopathy (MFM) which shows progressive limb muscle weakness, respiratory failure, and cardiomyopathy. Myopathy patients with BAG3 mutation are very rare. We described a patient showing atypical phenotypes. We aimed to find the genetic cause of Korean patients with sensory motor polyneuropathy, myopathy and rigid spine. We performed whole exome sequencing (WES) with 423 patients with sensory motor polyneuropathy. We found BAG3 mutation in one patient with neuropathy, myopathy and rigid spine syndrome, and performed electrophysiological study, whole body MRI and muscle biopsy on the patient. A de novo heterozygous p.Pro209Leu (c.626C>T) mutation in BAG3 was identified in a female myopathy. She first noticed a gait disturbance and spinal rigidity at the age of 11, and serum creatine kinase levels were elevated ninefolds than normal. She showed an axonal sensory-motor polyneuropathy like Charcot-Marie-Tooth disease (CMT), myopathy, rigid spine and respiratory dysfunction; however, she did not show any cardiomyopathy, which is a common symptom in BAG3 mutation. Lower limb MRI and whole spine MRI showed bilateral symmetric fatty atrophy of muscles at the lower limb and paraspinal muscles. When we track traceable MRI 1 year later, the muscle damage progressed slowly. As far as our knowledge, this is the first Korean patient with BAG3 mutation. We described a BAG3 mutation patient with atypical phenotype of CMT and myopathy, and those are expected to broaden the clinical spectrum of the disease and help to diagnose it.
Purpose: The purpose of this study was to identify the effect of ghrelin on memory impairment in a rat model of vascular dementia induced by chronic cerebral hypoperfusion. Methods: Randomized controlled groups and the posttest design were used. We established the representative animal model of vascular dementia caused by bilateral common carotid artery occlusion and administered $80{\mu}g/kg$ ghrelin intraperitoneally for 4 weeks. First, behavioral studies were performed to evaluate spatial memory. Second, we used molecular biology techniques to determine whether ghrelin ameliorates the damage to the structure and function of the white matter and hippocampus, which are crucial to learning and memory. Results: Ghrelin improved the spatial memory impairment in the Y-maze and Morris water maze test. In the white matter, demyelination and atrophy of the corpus callosum were significantly decreased in the ghrelin-treated group. In the hippocampus, ghrelin increased the length of hippocampal microvessels and reduced the microvessels pathology. Further, we confirmed angiogenesis enhancement through the fact that ghrelin treatment increased vascular endothelial growth factor (VEGF)-related protein levels, which are the most powerful mediators of angiogenesis in the hippocampus. Conclusion: We found that ghrelin affected the damaged myelin sheaths and microvessels by increasing angiogenesis, which then led to neuroprotection and improved memory function. We suggest that further studies continue to accumulate evidence of the effect of ghrelin. Further, we believe that the development of therapeutic interventions that increase ghrelin may contribute to memory improvement in patients with vascular dementia.
Recent evidence has revealed that the intratesticular injection of hypertonic saline(20%) resulted in a chemically castrated state such as nadir testosterone levels in rats. To confirm the efficacy of this simple saline-injection method further, we investigated the changes in the gross and microscopic anatomy of testis. Our study comprised three groups; intact(control) group, orchidectomy group and saline-injection (experimental) group. Single dose of hypertonic saline (sterilized, $750{\mu}{\ell}/testis$) were directly administered into both testis of adult rats (about 300 g BW). Bilateral orchidectomy was performed at the same day of saline injection. Following 30 days post-injection, reproductive tissues were surgically removed, weighed and fixed for histological examination. The body weights were not changed in both orchidectomy group and saline-injection group when compared to those in intact group. The wet weights of testis were significantly decreased in saline-injection group when compared to those in intact group. The wet weights of epididymis and seminal vesicle and prostate were significantly decreased in orchidectomy group and saline-injection group when compared to those in intact group. Macroscopically, the testes exerted slight atrophy and the tunica albuginea seemed to be intact in saline injection group. Histologically, however, larger parts of testicular tissue underwent necrosis and were barely recognizable after hematoxylin-eosin staining. In the same section, only the opposite part of the injection site was stained showing abnormal state of cell layers mostly fibrosis and infiltrated leukocytes. Sloughing of immature germ cells from the basement membrane along with shedding cells in the intraluminal space was notable in most seminiferous tubules from the saline injected testis. The present study confirmed that the direct injection of hypertonic saline into testis can induce a castration-like, testosterone-depriving effects on accessory sex organs. Our findings suggest that the efficacy of this less expensive and minimally invasive method seems to be almost even with that of conventional orchidectomy and chemical castration, though more in-depth evaluation should be supported.
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