• Title/Summary/Keyword: white matter disease

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Wallerian Degeneration of Insufficiently Affected White Matters in Old Infarction: Tract of Interest Analysis of Diffusion Tensor Imaging

  • Choi, Chi-Hoon;Lee, Jong-Min;Koo, Bang-Bon;Park, Jun-Sung;Kwon, Jun-Soo;Kim, Sun-I.
    • Journal of Biomedical Engineering Research
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    • v.28 no.3
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    • pp.317-324
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    • 2007
  • The application of diffusion tensor imaging (DTI) and fiber tractography to Wallerian degeneration (WD) is important because this technique is a very potent tools for quantitatively evaluating fiber tracts in vivo brain. We analyzed a case and control using tracts of interest (TOI) analysis to quantify WD. We scanned a case of old infarction and an age-matched healthy volunteer. T1 magnetization prepared rapid acquisition gradient echo (MPRAGE), fluid attenuated inversion recovery (FLAIR) and 12-direction diffusion tensor imaging (DTI) were obtained and analyzed using TOI analysis. The value of mean diffusity ($D_{av}$) and fracional anisotrophy (FA) were analyzed statistically by MWU test. A p-value of less than 0.05 was considered to indicate statistical significance. A comparison of the global fiber diffusion characteristics shows WD of both the corpus callosum and the ipsilateral superior longitudinal fasciculus. The corpus callosum in particular showed trans-hemispherical degeneration. Local fiber characteristics along the geodesic paths show WD in the corpus callosum, ipsilateral superior longitudinal fasciculus, ipsilateral corticospinal tract, and ipsilateral corticothalamic tract. We have demonstrated changes in $D_{av}$ and FA values and a clear correspondence with the WD in various tracts. TOI analysis successfully revealed radial WD in white matter tracts from a region of encephalomalacia and primary gliosis, although they were only slightly affected.

Acute disseminated encephalomyelitis in children: differential diagnosis from multiple sclerosis on the basis of clinical course

  • Lee, Yun-Jin
    • Clinical and Experimental Pediatrics
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    • v.54 no.6
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    • pp.234-240
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    • 2011
  • Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

Biological Control of Soil-borne Diseases with Antagonistic Bacteria

  • Kim, Byung-Ryun;Hahm, Soo-Sang;Han, Kwang-Seop;Kim, Jong-Tae;Park, In-Hee
    • 한국균학회소식:학술대회논문집
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    • 2016.05a
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    • pp.25-25
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    • 2016
  • Biological control has many advantages as a disease control method, particularly when compared with pesticides. One of the most important benefits is that biological control is an environmental friendly method and does not introduce pollutants into the environment. Another great advantage of this method is its selectivity. Selectivity is the important factor regarding the balance of agricultural ecosystems because a great damage to non target species can lead to the restriction of natural enemies' populations. The objective of this research was to evaluate the effects of several different bacterial isolates on the efficacy of biological control of soil borne diseases. White rot caused by Sclerotium cepivorum was reported to be severe disease of garlic and chive. The antifungal bacteria Burkholderia pyrrocinia CAB08106-4 was tested in field bioassays for its ability to suppress white rot disease. In field tests, B. pyrrocinia CAB08106-4 isolates suppressed white rot in garlic and chive, with the average control efficacies of 69.6% and 58.9%, respectively. In addition, when a culture filtrate of B. pyrrocinia CAB08106-4 was sprayed onto wounded garlic bulbs after inoculation with a Penicillium hirstum spore suspension in a cold storage room ($-2^{\circ}C$), blue mold disease on garlic bulbs was suppressed, with a control efficacy of 79.2%. These results suggested that B. pyrrocinia CAB08106-4 isolates could be used as effective biological control agents against both soil-borne and post-harvest diseases of Liliaceae. Chinese cabbage clubroot caused by Plasmodiophora brassicae was found to be highly virulent in Chinese cabbage, turnips, and cabbage. In this study, the endophytic bacterium Flavobacterium hercynium EPB-C313, which was isolated from Chinese cabbage tissues, was investigated for its antimicrobial activity by inactivating resting spores and its control effects on clubroot disease using bioassays. The bacterial cells, culture solutions, and culture filtrates of F. hercynium EPB-C313 inactivated the resting spores of P. brassicae, with the control efficacies of 90.4%, 36.8%, and 26.0%, respectively. Complex treatments greatly enhanced the control efficacy by 63.7% in a field of 50% diseased plants by incorporating pellets containing organic matter and F. hercynium EPB-C313 in soil, drenching seedlings with a culture solution of F. hercynium EPB-C313, and drenching soil for 10 days after planting. Soft rot caused by Pectobacterium carotovorum subsp. carotovorum was reported to be severe disease to Chinese cabbage in spring seasons. The antifungal bacterium, Bacillus sp. CAB12243-2 suppresses the soft rot disease on Chinese cabbage with 73.0% control efficacy in greenhouse assay. This isolate will increase the utilization of rhizobacteria species as biocontrol agents against soft rot disease of vegetable crops. Sclerotinia rot caused by Sclerotinia sclerotiorum has been reported on lettuce during winter. An antifungal isolate of Pseudomonas corrugata CAB07024-3 was tested in field bioassays for its ability to suppress scleritinia rot. This antagonistic microorganism showed four-year average effects of 63.1% of the control in the same field. Furthermore, P. corrugata CAB07024-3 has a wide antifungal spectrum against plant pathogens, including Sclerotinia sclerotiorum, Sclerotium cepivorum, Botrytis cinerea, Colletotrichum gloeosporioides, Phytophotra capsici, and Pythium myriotylum.

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A case of X-linked Charcot-Marie-tooth disease type 1 manifesting as recurrent alternating hemiplegia with transient cerebral white matter lesions

  • Kang, Minsung;Hwang, Sun-Jae;Shin, Jin-Hong;Kim, Dae-Seong
    • Annals of Clinical Neurophysiology
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    • v.23 no.2
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    • pp.130-133
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    • 2021
  • X-linked Charcot Marie Tooth disease type 1 (CMTX1) is a clinically heterogenous X-linked hereditary neuropathy caused by mutation of the gene encoding gap junction beta 1 protein (GJB1). Typical clinical manifestations of CMTX1 are progressive weakness or sensory disturbance due to peripheral neuropathy. However, there have been some CMTX1 cases with accompanying central nervous system (CNS) manifestations. We report the case of a genetically confirmed CMTX1 patient who presented recurrent transient CNS symptoms without any symptom or sign of peripheral nervous system involvement.

Cognitive dysfunctions in individuals with diabetes mellitus

  • Kim, Hye-Geum
    • Journal of Yeungnam Medical Science
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    • v.36 no.3
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    • pp.183-191
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    • 2019
  • Some patients with type 1 and type 2 diabetes mellitus (DM) present with cognitive dysfunctions. The pathophysiology underlying this complication is not well understood. Type 1 DM has been associated with a decrease in the speed of information processing, psychomotor efficiency, attention, mental flexibility, and visual perception. Longitudinal epidemiological studies of type 1 DM have indicated that chronic hyperglycemia and microvascular disease, rather than repeated severe hypoglycemia, are associated with the pathogenesis of DM-related cognitive dysfunction. However, severe hypoglycemic episodes may contribute to cognitive dysfunction in high-risk patients with DM. Type 2 DM has been associated with memory deficits, decreased psychomotor speed, and reduced frontal lobe/executive function. In type 2 DM, chronic hyperglycemia, long duration of DM, presence of vascular risk factors (e.g., hypertension and obesity), and microvascular and macrovascular complications are associated with the increased risk of developing cognitive dysfunction. The pathophysiology of cognitive dysfunction in individuals with DM include the following: (1) role of hyperglycemia, (2) role of vascular disease, (3) role of hypoglycemia, and (4) role of insulin resistance and amyloid. Recently, some investigators have proposed that type 3 DM is correlated to sporadic Alzheimer's disease. The molecular and biochemical consequences of insulin and insulin-like growth factor resistance in the brain compromise neuronal survival, energy production, gene expression, plasticity, and white matter integrity. If patients claim that their performance is worsening or if they ask about the effects of DM on functioning, screening and assessment are recommended.

An adverse event following 2009 H1N1 influenza vaccination: a case of acute disseminated encephalomyelitis

  • Lee, Sang-Teak;Choe, Young-June;Moon, Won-Jin;Choi, Jin-Woo;Lee, Ran
    • Clinical and Experimental Pediatrics
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    • v.54 no.10
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    • pp.422-424
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    • 2011
  • Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that typically follows an infection or vaccination and has a favorable long-term prognosis. We describe the first reported case of ADEM after vaccination against novel influenza A (H1N1). A previously healthy 34-month-old boy who developed ADEM presented with a seizure and left-sided weakness 5 days after vaccination against novel influenza A (H1N1). Cerebrospinal fluid examination revealed elevated cell counts. T2-weighted images and fluid-attenuated inversion recovery images revealed multiple patchy hyperintense lesions in the frontal and parietal subcortical white matter and the left thalamus. After the administration of intravenous corticosteroid, the patient's clinical symptoms improved and he recovered completely without neurologic sequelae.

Dental Treatment of a Patient with Pelizaeus-Merzbacher Disease under Outpatient General Anesthesia -A Case Report- (Pelizaeus-Merzbacher 병 환자의 외래전신마취 하 치과치료 -증례보고-)

  • Kim, Tae-Kyung;Shin, Cha-Uk;Kim, Hyun-Jeong;Yum, Kwang-Won;Seo, Kwang-Suk
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.7 no.1
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    • pp.18-21
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    • 2007
  • Pelizaeus-Merzbacher disease (PMD) can be defined as an X-linked recessive leukodystrophy that is caused by a mutation in the proteolipid protein gene on chromosome Xq22. PMD is one of a group of progressive, degenerative disorders of the cerebral white matter known as the leukodystrophies. Due to the progressive nature of the disorders and their devastating effects on the central nervous system, these children frequently require anesthesia during imaging procedures such as MRI or during various surgical procedures. Anesthetic concerns in theses cases include high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, airway complications related to poor pharyngeal muscle control and copious oral secretions, and mental retardation. We report a successful anesthetic management in a patient with PMD for dental procedures.

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Association of Type 2 Diabetes Mellitus With Perivascular Spaces and Cerebral Amyloid Angiopathy in Alzheimer's Disease: Insights From MRI Imaging

  • Ozlem Bizpinar Munis
    • Dementia and Neurocognitive Disorders
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    • v.22 no.3
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    • pp.87-99
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    • 2023
  • Background and Purpose: According to the amyloid cascade hypothesis, fibrillary amyloid-beta load in the brain causes Alzheimer's disease (AD) with toxic effects. Recently, perivascular spaces (PVSs), fluid-filled cavities around small penetrating arterioles and venules in the brain, and the glymphatic system relationship with type 2 diabetes mellitus (DM2) and AD has been an important research topic from a physiopathological point of view. There are two types of PVSs that are associated with sporadic atherosclerosis and cerebral amyloid angiopathy. In this study, we evaluated the relationship between the number and localization of enlarged PVSs in AD. Methods: A total of 254 patients with AD and 125 healthy controls were included in this study All the patients were evaluated with neurological and cognitive examinations and magnetic resonance imaging (MRI). PVSs on MRI were graded by recording their number and location. The study was a retrospective study. Results: In our study, the number of white matter convexity-central semiovale localized PVSs was higher in patients than in the control group. In addition, the number of PVSs in this localization score was higher in patients with DM2. Cerebral PVS counts were higher in patients with AD than in the control group. Conclusions: These results suggest the important role of cerebral amyloid angiopathy, one of the vascular risk factors, and the glymphatic system in the pathogenesis of AD. In addition, the results of our study suggest that the evaluation of PVSs levels, especially at the (centrum semiovale), using imaging studies in AD is a potential diagnostic option.

A Bibliographical Study on Dementia (치매(痴?)에 대한 문헌적(文獻的) 고찰(考察))

  • Kim, Yeong-Gyun;Gwon, Jeong-Nam;Choi, Ran-Suk
    • The Journal of Internal Korean Medicine
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    • v.18 no.2
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    • pp.177-194
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    • 1997
  • This thesis, deduced from studying eastern and western medical records, deals with geriatric demedtia in modern society. The result were obtained as follows : 1. Dementia is a kind of chronic, progressive, degenerative disease. The chief expression and pathogenic change of the disease is organic: e.g., extensive change such as cerebrum - atrophy, and denaturalization result. in such a situation intellectual capacities and the ability to enjoy daily life deteriorate trenendously. 2. A basic internal cause of the disease is Defficiencies of the heart, liver and kidneys. An exterior cause is an Excessiveness of the 'Dam'(痰), 'Blood Stasis', 'Fung'(風) and 'Fire'. In a Western Medical view, the reason for dementia is due to the onset of Alzheimer's disease and Brain anemia resulting from Multi - infarction or some other reason. If the white - matter of the brain is injured, then dementia easily to results. 3. Disease symptoms result in troubles in intellectual functions : e.g., memory, orientation, intelligence, judgement, common sence and calculating abilities. 4. The proper therapeutic treatment depends on the causes. When the Deficiency is serious, Fortification (heart, liver. and kidney deficiency) is applied and Decrease is follow. When Excessiveness of wrong is serious, the Decrease is tried before the supplement measure is used depending on the deficiency, which generally is used together with 'Fortify Right - Decreace Wrong'. 5. If the disease wasn't caused by some mental reason, it's difficult to be cured of the disease. When the degree of the disease is light and it doesn't continue for a long time, the therapeutic treatment can block the disease's progress and improve the patient's symptoms.

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Congenital muscular dystrophy type 1A with residual merosin expression

  • Kim, Hyo Jeong;Choi, Young-Chul;Park, Hyung Jun;Lee, Young-Mock;Kim, Heung Dong;Lee, Joon Soo;Kang, Hoon-Chul
    • Clinical and Experimental Pediatrics
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    • v.57 no.3
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    • pp.149-152
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    • 2014
  • Congenital muscular dystrophy type 1A (MDC1A) is an autosomal recessive disorder characterized by hypotonia, elevated serum creatine kinase level, delayed motor milestones, white matter changes observed by brain magnetic resonance imaging, and normal intelligence. A mutation in the laminin ${\alpha}2$ (LAMA2) gene, located at 6q22-23, is a genetic cause of MDC1A. Patients have merosin (laminin ${\alpha}2$)-deficient skeletal muscles. However, the degree of merosin expression ranges from total absence to partial reduction. Patients with residual merosin expression have more variable and milder phenotypes than those with absolute merosin deficiency. We observed a Korean girl with MDC1A with residual merosin expression. Clinical presentation of this patient was typical except for late onset of the disease and external capsule involvement. Immunohistochemical staining of muscle fibers including merosin, is important to evaluate patients with hypotonia, delayed motor development, and abnormal white matter changes.