• Title/Summary/Keyword: Leukoencephalopathy

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Leukoencephalopathy and Disseminated Necrotizing Leukoencephalopathy Following Intrathecal Methotrexate Chemotherapy and Radiation Therapy for Central Nerve System Lymphoma or Leukemia

  • Kim, Ji-Yeon;Kim, Sung-Tae;Nam, Do-Hyun;Lee, Jung-Il;Park, Kwan;Kong, Doo-Sik
    • Journal of Korean Neurosurgical Society
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    • v.50 no.4
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    • pp.304-310
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    • 2011
  • Objective : Intrathecal methotrexate (MTX) therapy combined with whole brain radiotherapy (WBRT) is one of the major treatment modalities for leukemia and lymphoma involving the central nervous system (CNS). The purpose of this study was to retrospectively determine the incidences of leukoencephalopathy and disseminated necrotizing leukoencephalopathy (DNL) following intrathecal MTX therapy for CNS lymphoma or leukemia and to assess the potential risk factors. Methods : Between January 2000 and August 2009, 143 patients with CNS lymphoma or leukemia received intrathecal MTX therapy alone or in combination with WBRT at a single institution. Patients were followed up clinically and radiologically at regular two- or three-month intervals. Medical records were reviewed to obtain information regarding the patients' demographics, medical histories, radiologic characteristics, treatments, and clinical courses. Results : On follow-up MR images, leukoencephalopathy was found in 95 of 143 patients (66.4%). The median time to develop leukoencephalopathy was 6.6 months. Among those with leukoencephalopathy, four patients showed seven extensive white-matter changes with strongly enhancing lesions demonstrating DNL. Histological confirmation was done in six lesions of three patients and radiological diagnosis alone in one patient. Four lesions spontaneously disappeared on MR images without any treatment, with a mean duration of 14 months before disappearance of DNL. Conclusion : Leukoencephalopathy is a common phenomenon that occurs following intrathecal MTX therapy; however, DNL occurs at a very low incidence. For newly developed enhancing lesions, consideration for the occurrence of DNL should be taken to avoid unnecessary invasive procedures or therapies.

A Case of Posterior Reversible Leukoencephalopathy Syndrome Following Poststreptococcal Glomerulonephritis (A군 사슬알균 감염 후 사구체신염 환아에서 발생한 가역적 후두부 뇌병증 증후군 1례)

  • Lee, Eun Kyoung;Kang, Jin Han;Ma, Sang Hyuk
    • Pediatric Infection and Vaccine
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    • v.22 no.2
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    • pp.113-116
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    • 2015
  • Posterior reversible leukoencephalopathy syndrome is a clinical radiographic syndrome of many causative factors. Sudden onset headache, vomiting, altered mental status, blurred vision and seizures are main symptoms shown in posterior reversible leukoencephalopathy syndrome. In addition, it typically shows radiological findings of edema in the white matter of posterior cerebrum, being in commonly bilateral but asymmetric. We report a case of poststreptococcal glomerulonephritis (PSGN) presenting as posterior reversible leukoencephalopathy syndrome. Immediate control of hypertension resulted in rapid and complete neurological recovery.

A case of Reversible Posterior Leukoencephalopathy Syndrome in Lupus Nephritis Treated with Cyclosporine (루프스 신염 치료로 Cyclosporine 투여 후 발생한 가역성후백질뇌병증 증후군)

  • Na, Sang-Jun;Lee, Kee-Ook
    • Annals of Clinical Neurophysiology
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    • v.11 no.1
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    • pp.24-27
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    • 2009
  • Reversible posterior leukoencephalopathy syndrome (RPLS) is an acute encephalopathy, usually characterized by seizures, visual disorder, mental change and reversible posterior white and gray matter lesions on brain magnetic resonance imaging (MRI). Conditions commonly associated with RPLS include acute renal failure, eclampsia, hypertension, immunosuppresion state, and use of intravenous immunoglobulin or chemotherapy, and additional contributing factors. We report the case of RPLS associated with cyclosporine use in lupus nephritis. In this case, it was not clearly elucidated which was main cause of RPLS. We could think RPLS developed maybe due to combined effect of cyclosporine and lupus nephritis.

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A Case of Acute Leukoencephalopathy Patient with Akinetic Mutism and Cognitive Dysfunctions (무동무언증과 인지기능장애를 주소로 하는 급성 백질뇌증 환자 치험 1례)

  • Kim, Bo-Eun;Chung, Dae-Kyoo;Kim, Sang-Ho;Lee, Kyung-Suk
    • Journal of Oriental Neuropsychiatry
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    • v.24 no.4
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    • pp.385-392
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    • 2013
  • This case report presents a 34-year-old female patient. Her chief complaints are akinetic mutism and cognitive dysfunctions. We have diagnosed her with leukoencephalopathy. She was treated with Korean traditional herbal medicines (Joojackwhan) for 33 days continuously. The effects of treatment were measured by the Korean Mini - Mental State Examination and Modified Bathel Index. After treatment, her chief complaints have been improved. Furthermore, the K-MMSE, MBI scores increased. From this case, we deduced that the Korean traditional herbal medicines (Joojackwhan) could be effective for cognitive dysfunctions and akinetic mutism caused by leukoencephalopathy.

Acute Leukoencephalopathy During Cyclosporin A Therapy in a Pediatric Patient with Nephrotic Syndrome (신 증후군 환아에서 Cyclosporin A 치료 중 발생한 급성 백색질 뇌증 1례)

  • Jung Suk-Won;Rhee Kyung-Hwa;Kwon Young-Se;Kim, Soon-Ki;Son Byong-Kwan;Lee Ji-Eun
    • Childhood Kidney Diseases
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    • v.9 no.1
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    • pp.91-96
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    • 2005
  • Cyclosporin A-induced central neurotoxicity has been rarely reported in patients with nephrotic syndrome. We report a pediatric patient who developed acute leukoencephalopathy diagnosed by MRI during CsA therapy for nephrotic syndrome.

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Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children (소아에서 가역성 후두부 백질 뇌증 증후군의 임상적, 방사선학적 특성에 대한 고찰)

  • Lim, Hae-Ri;Seo, Hye-Eun;Kwon, Sun-Hak
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.901-904
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    • 2007
  • Purpose : Reversible posterior leukoencephalopathy syndrome is a complex disorder with characteristic clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. Methods : A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were retrospectively reviewed and analyzed. Results : Fifteen pateints were involved. (9 males and 5 females). The patients' ages ranged from 2-20 years (mean age : 10 years). The brain MRI revealed fairly symmetric areas of increased T2 signal involving both white and gray matter of parieto-occipital regions. The condition seemed to be associated with cyclosporin A and steroid therapy or a variety of other conditions in which blood pressure rises acutely. Conclusion : Reversible posterior leukoencephalopathy syndrome is a complicated neurological condition, but a better understanding of this complex syndrome may obviate unnecessary investigations and lead to prompt and appropriate management of the associated problems.

A Case of Reversible Posterior Leukoencephalopathy Syndrome during Methylprednisolone Pulse and Cyclophosphamide Therapy in a Child with Nephrotic Syndrome (신증후군 환자에서 스테로이드 충격요법과 경구용 면역억제제 치료 중 발생한 가역성 후백질뇌병증 1례)

  • Seo Joo Hee;Shin Jung Wook;Kim Ji Hong;Yoon Choon Sik
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.245-250
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    • 2005
  • The syndrome of reversible posterior leukoencephalopathy syndrome(RPLS) is characterized clinically by acute neurologic signs such as headache, vomiting, confusion, seizures, and visual abnormalities. Radiologically, abnormalities consistent with reversible white matter edema in the occipital and parietal lobes are characteristic. RPLS has often been associated with various systemic disorders, such as hypertensive encefhalopathy, eclampsia, and the use of intravenous or intrathecal immunosuppressive drugs. We report a case of RPLS that occurred after intravenous steroid pulse therapy and treatment with oral cyclophosphamide in a child with nephrotic syndrome, and we emphasize the importance of early recognition of RPLS in the treatment of nephrotic syndrome and appropriate management tn prevent Permanent neurologic disability. (J Korean Soc Pediatr Nephrol 2005;9:245-250)

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Progressive Multifocal Leukoencephalopathy in a Patient with T Cell Lymphoma of Head and Neck - A Case Report - (두경부 T 세포 림프종 환자에서 발생한 진행성 다초점성 백질뇌병증 - 증 례 보 고 -)

  • Shin, Dong Ah;Chang, Jong Hee;Chang, Jin Woo;Park, Yong Gou;Kim, Tai Seung;Chung, Sang Sup
    • Journal of Korean Neurosurgical Society
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    • v.29 no.12
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    • pp.1682-1687
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    • 2000
  • Progressive multifocal leukoencephalopathy(PML) is a fatal demyelinating disease that occurs in immunocompromised hosts. We report a case of PML that developed in patient with T cell lymphoma of head and neck. During chemotherapy for lymphoma, she was confused and had memory impairment. A magnetic resonance imaging of the brain revealed confluent signal change at white matter of the frontal lobe, insula, and anterior limb and genu of internal capsule. The lesion was confirmed with brain biopsy and the histopathological finding was compatible with PML.

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Glial Cell-specific Regulation of the JC virus Early Promoter by Silencer and DNA Methylation (Silencer 및 DNA methylation에 의한 JC virus early promoter의 뇌교세포 특이적인 조절)

  • 김희선;우문숙
    • YAKHAK HOEJI
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    • v.46 no.2
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    • pp.143-148
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    • 2002
  • The human polyomavirus JC virus is the etiologic agent of progressive multifocal leukoencephalopathy (PML). The JC virus early promoter directs cell-specific expression of the viral replication factor large T antigen, thus transcriptional regulation constitutes a major mechanism of glial tropism in PML. Here we found that pentanucleotide sequence immediately upstream of the TATA sequence functions as a cell-specific silencer in the JC virus transcription. In vitro binding studies showed that synthetic oligonucleotides spanning a pentanucleotide sequence, designated "oligo 2", interacts with nuclear proteins from non-glial cells in a cell-specific manner. Furthermore, the sequence preferentially repressed the heterologous thymidine kinase promoter activity in non-glial cells. We also tested whether JC virus transcription is controlled by DNA methylation. Transient transfection of in vitro methylated JC virus promoter abolished transcription in both the glial and non-glial cells. The repression fold was much larger in glial cells than in non-glial cells. Taken together, this finding suggests that glial cell-specific expression of the JC virus is controlled by DNA methylation as well as cell-specific silencers.