• 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.

• Investigative Magnetic Resonance Imaging
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• v.24 no.3
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• pp.168-173
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• 2020

Extranodal lymphoma presents in almost one-third of all non-Hodgkin lymphoma cases. The gastrointestinal tract, skin, and central nervous system are common sites of involvement, whereas the urethra and cervix are very rare. To the best of our knowledge, this is the first report on concurrent extranodal involvement of the uterine cervix and urethra. We report imaging findings of malignant lymphoma involving urethra and cervix concurrently in a 79-year-old female patient with literature review. The magnetic resonance imaging showed huge intermediate to high signal intensity mass on T2 weighted images and strong homogeneous enhancement in uterine cervix and periurethral area, but no surrounding architectural disruption.

• Journal of Korean Neurosurgical Society
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• v.56 no.1
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• pp.61-65
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• 2014

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.344-347
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• 2001

Intramedullary spinal tuberculoma is a rare form of central nervous system tuberculosis. The authors presented a case of 41 years old male with intramedullary and intradural extramedullary tuberculoma of the thoracic spinal cord without systemic involvement. The preoperative diagnosis was a metastatic cancer. Subtotal removal of intradural extramedullary and intramedullary mass was performed and pathological diagnosis was tuberculosis granuloma. We suggest that Intramedullary spinal tuberculoma must be considered in the differential diagnosis of the spinal cord compressive lesions in our country.

• Annals of Clinical Neurophysiology
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• v.24 no.2
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• pp.59-62
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• 2022

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.

• Radiation Oncology Journal
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• v.12 no.1
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• pp.33-42
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• 1994

From 1982 to 1991, sixteen Patients with primary non-Hodgkin's lymphoma of the central nervous system(CNS) were seen at Kyung Hee University Hospital. The most common subtypes were large, noncleaved cell lymphoma and immunoblastic lymphoma of B cells. Lesions most commonly involved were the parietal lobes and/or deep nuclei. Positive cerebrospinal fluid cytology was rare at initial presentation. Sixteen patients were treated with surgical biopsy or resection followed by whole brain radiotherapy at a median dose of 40 Gy(range=30-50 Gy) with variable boost doses. Of 16 patients who underwent surgery and postoperative radiotherapy, fourteen patients died between 2 and 49 months following treatment, and two are alive with no evidence of disease at 8 and 22 months. The 1-and 2-year survival rates were 55.6$ \% $ and 34.7$ \% $, respectively with 12 months of median survival. Patterns of failure were analyzed in eleven patients of total 16 patients. Failure at the original site of involvement was uncommon after radiotherapy treatment. In contrast, failure in the brain at sites other than those originally invovled was common in spite of the use of whole brain irradiation. Failure occurred in the brain 11/16(68.7$ \% $), in spinal axis 4/16(25.0$ \% $). The age, sex, location of involvement within CNS, numbers of lesion, or radiation dose did not influence on survival. The authors conclude that Primary CNS lymphoma is a locally aggressive disease that is poorly controlled with conventional radiation therapy. The limitation of current therapy for this disease are discussed, and certain promising modality should be made in regarding the management of future patients with this disease.

• Annals of Clinical Neurophysiology
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• v.21 no.2
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• pp.94-97
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• 2019

The patients with myotonic dystrophy (MD) show ocular motor abnormalities including strabismus, vergence deficits, and inaccurate or slow saccades. Two theories have been proposed to explain the oculomotor deficits in MD. The central theory attributes the defects of eye movements of MD to the involvement of the central nervous system while the muscular theory attributes to dystrophic changes of the extraocular muscles. A 58-year-old woman with MD showed selective slowing of horizontal saccades and reduced peak velocities for both horizontal canals in head impulse tests, while smooth-pursuit eye movements and vertical head impulse responses were normal. This case suggests that the extraocular muscles-as a final common pathway of the voluntary saccade and reflexive vestibular eye movements-may better explain the defective rapid eye movements observed in MD.

• Clinical and Experimental Pediatrics
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• v.62 no.9
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• pp.334-339
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• 2019

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.307-311
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• 1986

Beh,cet's disease was originally described as a triple symptom complex of oral aphthous ulceration, genital ulceration, and hypopyon iritis. It is now known to have a wide systemic manifestations. Among them, the central nervous system involvement should be diagnosed earlier because of it's lethal potential. Recently the authors experienced a case of Behcet's disease with CNS involvement. A 51-year-old female patient was admitted due to deterioration of mentality and generalized ache since 2 years prior to admission. The findings on physical examination were compatible with Behcet's disease, but without cerebrospinal pleocytosis. The manifestations were improved with medications of prednisolone, chlorambucil, colchicine, but relapsed 2 months later during subsequent tapering of prednisolone and chlorambucil. The patient is now on medication again. A case of Beh,cet's disease with CNS manifestations is reported with review of literature.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.14-20
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• 2010

Cytomegalovirus (CMV) is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID), which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS). Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at $72{^{\circ}C}$ for 5 seconds can eliminate CMV completely.