• Title/Summary/Keyword: White matter degeneration

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Changes of Electroencephalography & Cognitive Function in Subjects with White Matter Degeneration (대뇌 백질 변성을 보인 환자에서의 뇌파와 인지기능의 변화)

  • Kwon, Do-Hyoung;Yu, Sung-Dong;Lee, Ae-Young
    • Annals of Clinical Neurophysiology
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    • v.4 no.1
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    • pp.21-27
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    • 2002
  • Background : Spatial analysis of EEG is a phenomenal assessment and not so informative for phase space and dynamic aspect of EEG data. In contrast, nonlinear EEG analysis attempts to characterize the dynamics of neural networks in the brain. We have analyzed the features of EEG nonlinearly in subjects with white matter change on brain MRI and compared the results with cognitive function in each. Methods : Digital EEG data were taken for 30 seconds in 9 subjects with white matter degeneration and in 5 healthy normal controls without white matter change on MRI. Then we analyzed them nonlinearly to calculate the correlation dimension(D2) using the MATLAB software. The cognitive function was assessed by 3MS(modified mini-mental state examination). The severity of white matter change was assessed by Scheltens scale. Results : The mean D2 value of normal control was greater than that of white matter degeneration group. The D2s of some channels were correlative with 3MS and degree of white matter degeneration significantly. Conclusions : nonlinear analysis of EEG can be used as one of adjuvant functional studies for prediction of cognitive impairment in subjects with white matter degeneration and subcortical white matter change can be influential on cognitive function and correlation dimension of EEG.

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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.

Association between Global Cortical Atrophy, Medial Temporal Atrophy, White Matter Hyperintensities and Cognitive Functions in Korean Alzheimer's Disease Patients (알츠하이머병 환자의 전반적 피질 위축, 내측두엽 위축, 백질 고강도 신호와 인지기능의 연관성)

  • Choi, Leen;Joo, Soo-Hyun;Lee, Chang-Uk;Paik, In-Ho
    • Korean Journal of Biological Psychiatry
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    • v.22 no.3
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    • pp.140-148
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    • 2015
  • Objectives The aim of this study is to investigate the correlation between degenerative changes in brain [i.e., global cortical atrophy (GCA), medial temporal atrophy (MTA), white matter hyperintensities (WMH)] and neurocognitive dysfunction in Korean patients with Alzheimer's disease. Methods A total of 62 elderly subjects diagnosed with Alzheimer's disease were included in this study. The degenerative changes in brain MRI were rated with standardized visual rating scales (GCA or global cortical atrophy, MTA or medial temporal atrophy, and Fazekas scales) and the subjects were divided into two groups according to the degree of degeneration for each scale. Cognitive function was evaluated with Korean version of the Consortium to Establish a Registry for Alzheimer's Disease (CERAD-K) and several clinical features, including apolipoprotein E ${\varepsilon}4$ status, lipid profile and thyroid hormones, were also examined. Chi-square test and Fisher's exact test were performed to analyze the relationship between the degree of cerebral degeneration and neurocognitive functions. Results Demographic and clinical features, except for the age, did not show any significant difference between the two groups divided according to the degree of cerebral degenerative changes. However, higher degree of GCA was shown to be associated with poorer performance in verbal fluency test, word list recall test, and word list recognition test. Higher degree of MTA was shown to be associated with poorer performance in Mini-Mental State Examination in the Korean Version of CERAD Assessment Packet (MMSE-KC), word list recognition test and construction praxis recall test. Higher degree of white matter hyperintensities was shown to be associated with poorer performance in MMSE-KC. Conclusions Our results suggest that severe brain degeneration shown in MRI is associated with significantly poorer performance in neurocognitive tests in patients with Alzheimer's disease. Moreover, the degree of GCA, MTA and white matter hyperintensities, represented by scores from different visual rating scales, seems to affect certain neurocognitive domains each, which would provide useful information in clinical settings.

A Case of Canavan Disease (Canavan Disease 1례)

  • Son, Young Ho;Hwang, Tae Gyu;Sinn, Jong Beom
    • Clinical and Experimental Pediatrics
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    • v.46 no.9
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    • pp.934-938
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    • 2003
  • Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups. Patients have severe mental retardation, poor head control, macrocephaly and seizures. Canavan disease is caused by the accumulation of N-acetylaspartic acid(NAA) in the brain as the result of a deficiency of aspartoacylase(ASPA) activity. Most children are reported to have the infantile form, becoming symptomatic between three and six month of age, after unremarkable prenatal and perinatal course. We experienced a case of Canavan disease in a six day old female newborn baby, associated with seizure, degeneration of brain white matter and markedly elevated urine N-acetylaspartic acid(NAA) level. So, we report the case with a brief review of the related literature.

Clinical Implications of Focal Mineral Deposition in the Globus Pallidus on CT and Quantitative Susceptibility Mapping of MRI

  • Hyojin Kim;Jinhee Jang;Junghwa Kang;Seungun Jang;Yoonho Nam;Yangsean Choi;Na-young Shin;Kook-Jin Ahn;Bum-soo Kim
    • Korean Journal of Radiology
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    • v.23 no.7
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    • pp.742-751
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    • 2022
  • Objective: To assess focal mineral deposition in the globus pallidus (GP) by CT and quantitative susceptibility mapping (QSM) of MRI scans and evaluate its clinical significance, particularly cerebrovascular degeneration. Materials and Methods: This study included 105 patients (66.1 ± 13.7 years; 40 male and 65 female) who underwent both CT and MRI with available QSM data between January 2017 and December 2019. The presence of focal mineral deposition in the GP on QSM (GPQSM) and CT (GPCT) was assessed visually using a three-point scale. Cerebrovascular risk factors and small vessel disease (SVD) imaging markers were also assessed. The clinical and radiological findings were compared between the different grades of GPQSM and GPCT. The relationship between GP grades and cerebrovascular risk factors and SVD imaging markers was assessed using univariable and multivariable linear regression analyses. Results: GPCT and GPQSM were significantly associated (p < 0.001) but were not identical. Higher GPCT and GPQSM grades showed smaller gray matter (p = 0.030 and p = 0.025, respectively) and white matter (p = 0.013 and p = 0.019, respectively) volumes, as well as larger GP volumes (p < 0.001 for both). Among SVD markers, white matter hyperintensity was significantly associated with GPCT (p = 0.006) and brain atrophy was significantly associated with GPQSM (p = 0.032) in at univariable analysis. In multivariable analysis, the normalized volume of the GP was independently positively associated with GPCT (p < 0.001) and GPQSM (p = 0.002), while the normalized volume of the GM was independently negatively associated with GPCT (p = 0.040) and GPQSM (p = 0.035). Conclusion: Focal mineral deposition in the GP on CT and QSM might be a potential imaging marker of cerebral vascular degeneration. Both were associated with increased GP volume.

Bilateral Pallidotomy for Dystonia with Glutaric Aciduria Type 1

  • Hwang, Hyung-Sik;Salles, Antonio De
    • Journal of Korean Neurosurgical Society
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    • v.38 no.5
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    • pp.380-383
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    • 2005
  • Glutaric aciduria type 1 is an inborn error of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-coenzyme A dehydrogenase. The disease often appears in infancy with encephalopathy episode that results in acute basal ganglia and white matter degeneration. The majority of patients develop a dystonic-dyskinetic syndrome. This reports 6year-old boy who had been done previous gastrostomy due to swallowing difficulty underwent bilateral pallidotomy with intraoperative electromyography[EMG] monitoring for disabling dystonia. Intraoperative EMG was used to assess stimulation thresholds required for capsular responses and muscle tone. Surface EMG electrodes were placed on the face and cricopharyngeal muscles. Exact target were directly modified according to MRI-visualized anatomy. EMG response was consistently seen prior to visual observation of muscle activity. The surgery improved dystonic symptoms without swallowing difficulty.

A Case of Canine Hydrocephalus with Nonsuppurative Encephalitis (비화농성 뇌염을 동반한 개 수두증 1례)

  • 한정희;서강문
    • Journal of Veterinary Clinics
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    • v.17 no.2
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    • pp.470-474
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    • 2000
  • A Shih Tzu Puppy had clinical onset of anotexia crying and progression of neurological sings when enlargement of the cranial vault at 1 month old and died after showing clinical signs during 2 months period. Radiological and pathological examinations were performed. Radiological findings were homogeneous appearance of the calvaria with cortical thinning, loss of the normal convolutional skull markings and persistent fontanelles. Grossly enlargement of the cranial vault thinning of the bone and defective closure of the fontanelles were also observed. The entire subcortical area of the cerebral hemispheres with severe, dilatation of ventricles and cerebrospinal fluid(CSF) wits absent. There was parenchyma atrophy affecting chiefly in the white mater and the cerebral cortices, axon degeneration and necrosis and gitter cell infiltration in the whiter matter and the subependymal area. Mononuclear perivacular cuffing in the cerebrum and the pons was shown. Based on the radiological, gross and histopathological findings, this case was believed to have congenital hydrocephalus with nonsuppurative encephahitis. Possible etiology on the case is also discussed.

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Demyelination in natural canine distemper encephalomyelitis : An immunohistochemical study of myelin basic protein, myelin associated glycoprotein and glial fibrillary acidic protein in the lesion of demyelination (홍역이환개에서 발생한 수초탈락성 뇌척수염 : 수초탈락부위에서 MBP, MAG 및 GFAP의 면역조직학적 관찰)

  • Shin, Tae-kyun;Kwon, Oh-deog;Lee, Du-sik;Lee, Cha-soo
    • Korean Journal of Veterinary Research
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    • v.33 no.2
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    • pp.295-300
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    • 1993
  • Central nervous system of two dogs with natural canine distemper was investigated histopathologically and immunocytochemically with antisera to MBP, MAG and GFAP. Histopathologically, there were neuronal degeneration and diffuse gliosis in the cerebrum, vacuolar degeneration, hypertrophy of astrocytes and demyelination in cerebellar white matter adjacent to the 4th ventricle and optic tracts showing non-inflammatory demyelinating encephalomyelitis (Summers and Appel, 1987). Immunohistochemically, there was a concurrent disappearance of MBP and MAG in the well developed demyelinating lesion in the cerebellar white matter. At the margin of demyelination, Loss of both MBP and MAG varied on the stage of demyelinating process. GFAP-positive astrocytes were hypertrophied and contained canine distemper virus intranuclear inclusions. GFAP-positive fibers were increased at the early stage of demyelination, and then were not immunoreaeted at the well developed demyelination. Hypertrophic astrocytes with intranuclear inclusions were commonly identified in the interfascular layer without myelin vacuolation and demyelination. This is the first study of primary demyelination and astroglial reactions in natural CDE investigated using immunocytochemistry of two myelin proteins and GFAP. Concurrent loss of MBP and MAG suggest that the myelin sheath is the target in the demyelinating process in CDE.

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Neuroprotective Effects of 6-Shogaol and Its Metabolite, 6-Paradol, in a Mouse Model of Multiple Sclerosis

  • Sapkota, Arjun;Park, Se Jin;Choi, Ji Woong
    • Biomolecules & Therapeutics
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    • v.27 no.2
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    • pp.152-159
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    • 2019
  • Multiple sclerosis (MS) is an autoimmune disease characterized by progressive neuronal loss, neuroinflammation, axonal degeneration, and demyelination. Previous studies have reported that 6-shogaol, a major constituent of ginger (Zingiber officinale rhizome), and its biological metabolite, 6-paradol, have anti-inflammatory and anti-oxidative properties in the central nervous system (CNS). In the present study, we investigated whether 6-shogaol and 6-paradol could ameliorate against experimental autoimmune encephalomyelitis (EAE), a mouse model of MS elicited by myelin oligodendrocyte glycoprotein ($MOG_{35-55}$) peptide immunization with injection of pertussis toxin. Once-daily administration of 6-shogaol and 6-paradol (5 mg/kg/day, p.o.) to symptomatic EAE mice significantly alleviated clinical signs of the disease along with remyelination and reduced cell accumulation in the white matter of spinal cord. Administration of 6-shogaol and 6-paradol into EAE mice markedly reduced astrogliosis and microglial activation as key features of immune responses inside the CNS. Furthermore, administration of these two molecules significantly suppressed expression level of tumor necrosis $factor-{\alpha}$, a major proinflammatory cytokine, in EAE spinal cord. Collectively, these results demonstrate therapeutic efficacy of 6-shogaol or 6-paradol for EAE by reducing neuroinflammatory responses, further indicating the therapeutic potential of these two active ingredients of ginger for MS.

The Effects of Sustained Release Growth Hormone in the Repair of Neurological Deficits in Rats with the Spinal Cord Injury (척수손상 백서에서 서방형 성장호르몬의 투여가 신경회복에 미치는 영향)

  • Kim, Min Su;Heo, Jung;Kwon, Yong Seok;Lee, Keun Cheol;Kim, Seok Kwun
    • Archives of Plastic Surgery
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    • v.35 no.3
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    • pp.235-242
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    • 2008
  • Purpose: Due to increasing interest in the treatment of spinal cord injuries, many histopathological studies have been conducted to prove that many neurotrophic factors including growth hormone are important for regeneration of the injured spinal cord. Growth hormone has to be given everyday, however, and this negatively affects compliance in clinical trials. Recently, the invention of sustained release growth hormone (SRGH) that can be given just once a week may both help the regeneration of injured spinal cord and, at the same time, be more compliant and convenient for clinical patients. Methods: In this study, thirty 7-week-old female Spraque-Dawley rats were subjected to a weight-driven impact spinal cord injury. They were divided into 3 groups and Group I and II were injected with SRGH once a week for 4 weeks; Group I were injected into the injured spinal cord area, while Group II were injected into the peritoneal cavity. Meanwhile, Group III were injected with normal saline solution. The functional outcome was evaluated using the Basso-Beattie-Bresnahan motor rating score and the inclined plane test was done 4 weeks after the first injection. Histopathological examination was performed at the same time and the amount of residual white matter was measured in all groups. Results: After 4 weeks, Groups I and II showed greater improvement than Group III(the control group) in the functional test. In the control group, invasion of atypical phagocytes, axonal degeneration, edema and cavity formation in the posterior site of spinal cord gray matter was observed in histopatholgical examination. The rate of residual white matter in Group III was less than in the other groups. Conclusion: Data showed significant functional and histopathological improvement in the groups treated with SRGH into the spinal and peritoneal cavity compared with the control group. SRGH is therefore beneficial because it helps with regeneration of the injured spinal cord and improves the compliance and convenience of patients.