• Title/Summary/Keyword: CNS involvement

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Preliminary Results of Management for Primary CNS Lymphoma (원발성 중추신경계 림프종의 치료에 관한 예비적 결과)

  • Ahn, Seung-Do;Chang, Hye-Sook;Choi, Eun-Kyong
    • Radiation Oncology Journal
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    • v.11 no.1
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    • pp.79-82
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    • 1993
  • From October 1989 to March 1992, ten patients diagnosed as primary central nervous system (CNS) lymphoma were treated with radiation therapy at Asan Medical Center. To obtain pathologic diagnosis, five patients had stereotactic biopsy and the others underwent craniotomy & tumor removal. According to the classification by International Working Formulation, seven of 10 patients showed diffuse large cell types and the remaining 3 had diffuse mixed cell types. Computed tomographic scans of the brain disclosed solitary (6 cases) or multiple (4 cases) intracranial lesions. All patients received 4000 cGy/20 fx to the whole brain followed by an additional 2000 cGy/10 fx boost to the primary lesion. Six patients with initial cerebrospinal fluid (CSF involvement were treated with whole brain irradiation and intrathecal Methotrexate (IT-MTX) chemotherapy. One of them received an additional spinal irradiation after 3 cycles of IT-MTX chemotherapy because of MTX induced arachnoiditis. One patient received 3 cycles of systemic chemotherapy prior to rodiation therapy and one received 5 cycles of salvage chemotherapy for recurrence. With a median follow up time of 8 months, all patients were followed from 7 to 26 months. Radiologically seven patients showed complete remission and the remaining three showed partial remission at one month after radiotherapy. The 1 and 2 year survival rate was $86{\%}\;and\;69{\%}$ respectively. Until now, two patients expired at 7 and 14 months. These patients developed extensive CSF seeding followed by local failure. Considering initial good response to radiation therapy and low incidence of extraneural dissemination in primary CNS lymphoma, we propose to increase total tumor dose to the primary lesion by hyperfractionated radiotherapy or stereotactic radiosurgery. For the patients with CSF involvement at diagnosis, we propose craniospinal irradiation with IT MTX chemotherapy.

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Two Cases of Microscopic Polyangiitis (현미경적 다발성 혈관염 2예)

  • Song, Kyoung Eun;Im, Seung Guan;Jo, Sook Kyoung;Jung, Youn Mu;Choi, Joon Hyuck;Sheen, Seung Soo;Oh, Yoon Jung;Park, Kwang Joo;Hwang, Sung Chul;Yim, Hyun Ee
    • Tuberculosis and Respiratory Diseases
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    • v.55 no.6
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    • pp.623-630
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    • 2003
  • Microscopic polyangiitis is a systemic small-vessel vasculitis that is primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. Lung involvement is characterized by a diffuse alveolar hemorrhage. However, rarely central nervous system involvement has been reported to be occurred with the microscopic polyangiitis. Relapse of microscopic polyangiitis are reported to be more frequent than those of polyarteritis nodosa, often after a reduction or discontinuation of the therapy. We would like to report two patients with microscopic polyangiitis. One presented with clinical manifestations of both lung and central nervous system involvements and the other was a case of recurrence during steroid tapering following the steroid pulse therapy.

Subacute Inflammatory Demyelinating Polyneuropathy Combined with Optic Neuritis (시신경염이 동반된 아급성염증탈수초다발신경병)

  • Kim, Sieun;Park, Kang Min;Park, Jinse;Ha, Sam Yeol;Kim, Sung Eun;Kim, Jong Kuk;Shin, Kyong Jin
    • Annals of Clinical Neurophysiology
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    • v.15 no.1
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    • pp.13-18
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    • 2013
  • It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.

Primary Cerebellar Hodgkin's Lymphoma

  • Hwang, Chul-Yoon;Song, Young-Jin;Kim, Dae-Chul;Choi, Sun-Seob;Choi, Young-Min;Kim, Ki-Uk
    • Journal of Korean Neurosurgical Society
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    • v.42 no.2
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    • pp.149-152
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    • 2007
  • Authors report a very rare case of primary Hodgkin's lymphoma limited to the cerebellum. A 64-year-old female patient presented with headache, nausea, and vomiting. Magnetic resonance imaging of the brain revealed a nodular enhancing mass in the left cerebellar hemisphere. Tumor was removed totally with retromastoid suboccipital approach. Diagnosis was Hodgkin's lymphoma of mixed cellularity type, consisting of a dense mixed inflammatory infiltrate containing scattered large atypical mononuclear Hodgkin's cell and multinucleated Reed-Sternberg cells. Systemic work-up for the Hodgkin's lymphoma was followed with negative result. Postoperative radiation therapy was given and the patient is disease-free at 16 months after the operation.

A Case of Fatal Acute Lupus Pneumonitis Defined by Necropsy (병리조직학적으로 확인된 급성루프스성 폐렴 1예)

  • Yoon, Ki-Heon;Yoo, Jee-Hong;Kang, Hong-Mo
    • Tuberculosis and Respiratory Diseases
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    • v.39 no.1
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    • pp.89-94
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    • 1992
  • A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

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Rapid Atypical Progression of Neuro-Behçet's Disease Involving Whole Brainstem and Bilateral Thalami

  • Lee, Sang-Kook;Choi, Sung-Jae;Kim, Sang-Dae;Lim, Dong-Jun
    • Journal of Korean Neurosurgical Society
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    • v.50 no.1
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    • pp.68-71
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    • 2011
  • We present a case of Neuro-Behçet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

A Case of Ramsay Hunt Syndrome with Aseptic Meningitis (무균성 뇌수막염을 동반한 Ramsay Hunt 증후군 1례)

  • Yi, Yeon Yul;Choi, Sung Dong;Jeung, Seung Yeon;Suh, Byung Kyu;Kang, Jin Han
    • Pediatric Infection and Vaccine
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    • v.4 no.2
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    • pp.298-302
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    • 1997
  • Ramsay Hunt syndrome is a viral associated disease with severe otalgia, vertigo, fever, herpetic eruptions on either side of the external auditory meatus and cavum concha, ipsilateral facial nerve palsy and cochleovestibular dysfunction. This syndrome may be the most common cause of unilateral facial paralysis and involvement of both the vestibular and cochlear branch of 8th cranial nerve. And loss of taste sensation may be developed in same involement site. This syndrome affects adults in most cases, and a samll number of children with herpes zoster oticus have been reported. And concomittantly CNS invlovement of this snydrome is very rare. We experienced a 7 years old aged patient of Ramsay Hunt syndrome who had evidence of aseptic meningitis, and this patient showed well reponses with Acyclovir and symptomatic therapy. So, we report this case with brief review of related literatures.

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Understanding the Pathophysiology and Magnetic Resonance Imaging of Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorders

  • Laura Cacciaguerra;Maria A. Rocca;Massimo Filippi
    • Korean Journal of Radiology
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    • v.24 no.12
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    • pp.1260-1283
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    • 2023
  • Magnetic resonance imaging (MRI) has been extensively applied in the study of multiple sclerosis (MS), substantially contributing to diagnosis, differential diagnosis, and disease monitoring. MRI studies have significantly contributed to the understanding of MS through the characterization of typical radiological features and their clinical or prognostic implications using conventional MRI pulse sequences and further with the application of advanced imaging techniques sensitive to microstructural damage. Interpretation of results has often been validated by MRI-pathology studies. However, the application of MRI techniques in the study of neuromyelitis optica spectrum disorders (NMOSD) remains an emerging field, and MRI studies have focused on radiological correlates of NMOSD and its pathophysiology to aid in diagnosis, improve monitoring, and identify relevant prognostic factors. In this review, we discuss the main contributions of MRI to the understanding of MS and NMOSD, focusing on the most novel discoveries to clarify differences in the pathophysiology of focal inflammation initiation and perpetuation, involvement of normal-appearing tissue, potential entry routes of pathogenic elements into the CNS, and existence of primary or secondary mechanisms of neurodegeneration.

Loss of Facilitation in H-reflex: Implication as an Initial Abnormality and an Evidence of Central Nervous Involvement in Guillian-Bare Syndrome (H-반사 촉진의 소실: 길랑-바레 증후군의 초기 이상과 중추신경 침범의 증거로써의 의미)

  • Sung, Jung-Joon;Lee, Yoon-Jeong;Hong, Yoon-Ho;Chung, Young-Min;Lee, Kwang-Woo
    • Annals of Clinical Neurophysiology
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    • v.4 no.2
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    • pp.119-124
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    • 2002
  • Background : Guillain-$Barr{\acute{e}}$ syndrome(GBS) is a neurologically emergent condition, leading to respiratory insufficiency without an early and appropriate treatment. Thus, the treatment of GBS requires early diagnosis but it is difficult due to the low sensitivity of laboratory tools in the initial stage. Hoffman reflex (H-reflex) and its facilitation by Jendrassik maneuver (JM) are sensitive tools evaluating the central circuit of motor system on the spinal cord level. The aim of this study is to test whether the change of H-reflex and F-wave under the JM is able to detect the early stage of GBS and whether GBS involves the central nervous system (CNS). Material and Methods : All 7 GBS patients who showed normal or nearly normal nerve conduction study were included. The facilitation of H-reflex and changes of F-wave were calculated by measuring the percent difference of H-reflex or F-wave amplitude under JM compared to basal H-reflex of F-wave amplitude. The changes of F-wave and H-reflex in the GBS patients were compared with them of 8 healthy controls. Results : The F-wave amplitudes of both healthy controls and GBS patients did not changed under the influence of JM ($102.4{\pm}24.9%$, $108.7{\pm}29.0%$ respectively). However, the facilitation of H-wave by JM in the GBS patients was absent ($98.8{\pm}5.8%$), even though the H-reflex amplitude I the healthy controls increased under the influence of JM ($124.8{\pm}12.1%$). Conclusions : The loss of H-reflex facilitation in GBS implies that this phenomenon might be the most early change detected in the electrophysiological study and support the early diagnosis, and that GBS might include lesion in the spinal motor CNS.

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Pretreatment with GPR88 Agonist Attenuates Postischemic Brain Injury in a Stroke Mouse Model (GPR88 효현제의 전처리에 의한 뇌졸중후 뇌손상 감소효과 연구)

  • Lee, Seo-Yeon;Park, Jung Hwa;Kim, Min Jae;Choi, Byung Tae;Shin, Hwa Kyoung
    • Journal of Life Science
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    • v.30 no.11
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    • pp.939-946
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    • 2020
  • Stroke is one of the leading causes of neurological disability worldwide and stroke patients exhibit a range of motor, cognitive, and psychiatric impairments. GPR88 is an orphan G protein-coupled receptor (GPCR) that is highly expressed in striatal medium spiny neurons; its deletion results in poor motor coordination and motor learning. There are currently no studies on the involvement of GPR88 in stroke or in post-stroke brain function recovery. In this study, we found a decrease in GPR88 protein and mRNA expression levels in an ischemic mouse model using Western blot and real-time PCR, respectively. In addition, we observed that, among the three types of cells derived from the brain (brain microvascular endothelial cells, BV2 microglial cells, and HT22 hippocampal neuronal cells), the expression of GPR88 was highest in HT22 neuronal cells, and that GPR88 expression was downregulated in HT22 cells under oxygen-glucose deprivation (OGD) conditions. Moreover, pretreatment with RTI- 13951-33 (10 mg/kg), a brain-penetrant GPR88 agonist, ameliorated brain injury following ischemia, as evidenced by improvements in infarct volume, vestibular-motor function, and neurological score. Collectively, our results suggest that GPR88 could be a potential drug target for the treatment of central nervous system (CNS) diseases, including ischemic stroke.