• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.334-341
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• 2001

Objective : We have currently changed treatment strategies to methotrexate(MTX)-based preirradiation chemotherapy with subsequent planned radiation for the initial therapy of primary central nervous system lymphoma (PCNSL). The aim of this study was to evaluate the results of treating PCNSL with chemotherapy plus radiotherapy (CRT) or radiotherapy(RT) alone. Method and Material : This study involved 10 females and 3 males patients with a mean age of 54.2 years. All patients underwent surgery, open(8 cases) or stereotactic biopsy(5 cases) for histological diagnosis. Eleven tumors were diffuse large B-cell lymphomas. Tumor volume change in the follow-up images and survival time were evaluated in patients treated with CRT and RT alone. In the beginning, two patients received ProMACE-Cytabom chemotherapeutic regimen, but did not complete the course and died of progressive tumor 8 and 9 months after diagnosis, respectively. One patient died at 6 months before chemotherapy. These three were excluded from the survival analysis. Five patients(RT group) completed full courses of cranial irradiation with or without boost. For the current combined modality treatment, high-dose MTXbased chemotherapy(systemic and intrathecal MTX, IV vincristine, and oral procarbazine) followed by whole brain irrdiation to 45Gy to tumor was introduced in 5 patients of CRT group. Result : A complete response was achieved in three of five who received RT only and in all of five who received CRT. All patients in CRT groups are in disease free status at a mean 23 months following therapy. The RT group patients refused any additional salvage therapy at tumor relapse and survived at mean 20 months from diagnosis. The Karnofsky performance status improved in eight of ten patients with treatment. The treatment toxicity included leukoencephalopathy in RT group and severe leukopenia, transient hepatitis, avascular necrosis of femoral head, hearing loss, and amenorrhea in CRT group, respectively. Conclusion : The combined modality therapy of MTX-based chemotherapy plus radiotherapy for PCNSL may enhance tumor response and improve patient survival. The patients who received CRT should be carefully followed up because of the higher risk of treatment-induced late neurotoxicity.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.97-103
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• 2005

Hypereosinophilic syndrome (HES) is characterized by a sustained eosinophilia of $1,500/mm^3$ or more in the absence of any known causes or the signs and symptoms of organ involvement. We report a 64-year-old man with HES initially presenting with involvement of the liver and bone marrow. Despite controlling the eosinophilia by corticosteroid, he developed a cerebral infarction and later progressive interstitial pneumonia. Brain angiography revealed a severe stenosis of the proximal right internal carotid artery (ICA) and a complete obstruction of the intracranial ICA. An open lung biopsy revealed fibrosis and lymphoplasma cell infiltration without eosinophils, which were consistent with nonspecific interstitial pneumonia.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.72-77
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• 2015

Purpose: Deficits of the respiratory chain are reported to be the major cause of Leigh syndrome is said to be the underlying causes. The need for biochemical diagnosis to draw more accurate diagnosis or prognosis to support treatments is rapidly increasing. This study tried to analyze the aspects of clinical characteristics and biochemical diagnosis of mitochondrial respiratory chain complex (MRC) defect in Leigh syndrome, using methods of biochemical enzyme assay. Methods: We included total number of 47 patients who satisfied the clinical criteria of Leigh syndrome and confirmed by biochemical diagnosis. All those patients went through muscle biopsy to perform biochemical enzyme assay to analyze MRC enzyme in order to find the underlying cause of Leigh syndrome. Results: MRC I defect was seen in 23 (48.9%) cases taking the first place and MRC IV defect in 15 (31.9%) following it. There were 9 (19.2%) cases of combined MRC defect. Combined cases of type I and IV were detected in 7 (14.9%) patients while type I and V in 2 (4.3%). The onset age of symptom was less than 1 year old in 28 (59.6%). The most common early symptom, observed in 23 (48.9%), was delayed development, but there were other various neurological symptoms observed as well. In regard with the disease progression, 35 (74.5%) patients showed slowly progressive course, the one that progressed continuously but slowly over 2 years of period. As for Maximum motor development, 22 (46.8%) were bed-ridden state, most of them suffering serious delayed development. Patients showed various symptoms with different organs involved, though neuromuscular involvement was most prominent. Delayed development was seen in all cases. Multifocal lesion in brain MRI study was seen in 36 (76.6 %) cases, taking a greater percentage than 11 (23.4%) cases with single lesion. In MR spectroscopy study, the characteristic lactate peak of mitochondrial disease was identified in 20 (42.6%) patients. Conclusions: Further analysis of clinical and biochemical diagnosis on more extended group of patients with Leigh syndrome will enable us to improve diagnostic precision and to understand the natural course of mitochondrial disease.

• Tuberculosis and Respiratory Diseases
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• v.62 no.6
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• pp.549-553
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• 2007

Sarcoidosis is a multi-systemic syndrome of an unknown etiology, and it is characterized by the formation of multiple noncaseating granulomas that disrupt the architecture and function of the tissues in which they reside. The most commonly affected organs are lung, skin and lymph nodes. Overt clinical involvement of the nervous system is uncommon and this occurs in about 5% of all patients during the course of their disease. The most common manifestations are granulomatous leptomeningitis, cranial nerve palsy, electrolyte or other endocrinologic abnormalities, but isolated memory impairment is a rare manifestation. This is a case of 59 years-old male with recent memory impairment, and he was previously diagnosed with pulmonary sarcoidosis by transbronchial lung biopsy. The brain MRI imaging revealed the leptomeningeal and parenchymal involvement of sarcoidosis. He was treated with high dose corticosteroid and his memory function was improved to nearly a normal level. We report here on a case of successful treatment of pulmonary sarcoidosis combined with neurosarcoidosis with using high dose corticosteroid, and the patient presented with recent memory impairment.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.110-115
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• 1999

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and, occasionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.

• Radiation Oncology Journal
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• v.9 no.2
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• pp.177-184
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• 1991

From December 1984 to February 1990, 16 patients with tumors of pineal and suprasellar location were treated with radiation therapy. Tissue diagnoses were obtained before radiation therapy in 5 patients and 11 were irradiated without histologic confirmation. Initial treatments for these patients were craniospinal plus boost primary irradiation(six), whole brain plus boost primary irradiation(nine), primary tumor site irradiation(one). The 5 year actuarial survival rate is $71\%$. Three cases with elevated beta-human chorionic gonadotropin(HCG) responded favorably to radiation, but pineal tumors with elevated alpha-fetoprotein(AFP) did not respond well. Spinal metastasis developed in 2 cases(2/15) with elevated AFP : one received prophylactic spinal irradiation, another did not. Our studies suggest that more aggressive treatment would be necessary in patient with elevated AFP and in this patient, radiation therapy may be initiated without pathologic confirmation. From the result of our study, routine use of prophylactic spinal irrdiation for all patients with pineal region tumor is not indicated and use of prophylactic spinal irradiation is considered for the patients with positive craniospinal fluid cytology, meningeal seeding, disease extension along the ventricular wall and biopsy proven germinoma.

• Journal of Veterinary Clinics
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• v.30 no.2
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• pp.142-145
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• 2013

Equine motor neuron disease (EMND) is a spontaneous neurologic disorder of horses, which results from the degeneration of motor neurons in the spinal cord and brain stem. An 8-year-old Jeju pony gelding presented with weight loss, muscle tremors, frequent recumbence, low head carriage, sweating, and standing with four limbs close together. The gelding has been on the same stable and limited access to pasture for several years. The gelding has been fed with dried hays and commercial concentrated feeds. Laboratory test revealed very low serum vitamin E level (0.14 ${\mu}g/mL$; reference range > 1.5 ${\mu}g/mL$), mildly elevated creatine kinase (402 IU/L; reference range 119-287 IU/L) and aspartate aminotransferase (876 IU/L; reference range 226-336 IU/L). Oral glucose absorption test showed decreased glucose absorption. Histopathologic examination using a biopsy specimen from sacrocaudalis dorsalis medialis muscle revealed atrophic and hypertrophic muscle fiber, centralization of nucleus, degenerating and necrotic muscle fibers. Taken together, the gelding was diagnosed as EMND. After oral vitamin E administrated for 5 weeks, the gelding showed normally improved stance, decreased periods of recumbency, improved head carriage and weight gain except consistent tremors. This is the first report that successfully treated EMND case occurred in a Jeju pony in Korea.

• Radiation Oncology Journal
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• v.15 no.1
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• pp.11-18
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• 1997

Purpose : To evaluate the efficacy of combined treatment of surgery and chemoradiotherapy for supratentorial primitive neuroectodermal tumors (SPNET) and obtain the Prognostic factors and complications Materials and Methods .The a9e of 18 patients ranged from 1 to 27 years (median=5 years). There were 12 males and 6 females The extents of surgery were gross total (n:9), subtotal (n:8), biopsy only (n: 1). Craniospinal radiotherapy was delivered to all the patients except 2 patients who were treated only with the whole brain and primary lesion. Radiation dose were 3120-5800cGy (median=5460) to primary mass, 1500-4200cGy (median=3600cGy) to the whole brain and 1320-3600cGy (median= 2400 cGy) to the spinal axis. Chemotherapy was done in 13 patients. Median follow-up period was 45 months ranged from 1 to 89 months. Results : Patterns of failure were as follows; local recurrence (1), multiple intracranial recurrence (2), spinal seeding (3), craniospinal seeding (2) and multiple bone metastasis (1). Two of two patients who did not received craniospinal radiotherapy failed at spinal area. All the relapsed cases died at 1 to 13 months after diagnosis of progression. The 2- and 5-rear overall survival rates were $61\%\;and\;49\%$, respectively The a9e, sex, tumor location did not influence the survival but aggressive resection with combined chemotherapy showed better outcome. Among 9 survivors, complications were detected as radiation necrosis (n=1), hypopituitarism (n=2), cognitive defect(n=1), memory deficit (n=1), growth retardation (n=1). Conclusion : To improve the results of treatment of SPNET, maximal surgical resection followed by radiation therapy and chemotherapy is necessary. The extended radiation field including craniospinal axis may reduce the recurrence in spinal axis.

• Radiation Oncology Journal
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• v.20 no.3
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• pp.193-198
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• 2002

Purpose : Oligodendrogliomas (ODG) are a rare, slow growing, tumor in the brain, which can be cured by complete surgical resection, but as yet it is not known if postoperative adjuvant radiation therapy (RT) is essential, We analyzed the treatment results of patients with irradiated ODG to investigate the efficacy of RT in terms of survival rates and other influencing prognostic factors. Methods and Materials : Between March 1983 and December 1997, 42 patients with ODG were treated with RT at our hospital. The RT was peformed dally at a dose of $1.8\~2.0\;Gy$, at 5 fractions per week, to a total dose of between 39.6 Gy and 64.8 Gy (mean 53.3 Gy). The ages of the patients ranged between 5 and 62 years, with a median age of 39 years. The mean follow-up period was 63.4 months (8-152 months). The Kaplan-Meier method was used to assess the survival, and 5 year survival rates (5-YSR). Log rank tests and Cox regression analyses were used to define the significance of prognostic factors. Results : The majority of ODG in this study were located in the cerebral hemisphere $(83.3\%)$. ODG are slightly more common in men than women, and commonly occurs in middle age, between the 3rd and 4th decades. It has been recommended that RT is commenced within 4 weeks following surgery (5-YSR; $86\%\;vs.\;49\%;\;p<0.03$). Histologically well differentiated, as opposed to poorly differentiated, tumors were found to have a more favorable prognosis (p<0.02). The actuarial 5-YSR was $65.3\%$ (median survival 90 months). 5-YSR for the various extents of surgical excision, followed by external RT, was superior to that of biopsy only followed by external RT $(69.9\%\;vs.\;25.6\%,\;p<0.01)$. Tumor size and location, overall elapsed irradiation days, age, sex, whole brain irradiation as a course of treatment and chemotherapy, had no influence on the 5-YSR (p>0.05). Conclusion : A local involved field irradiation with conventional fractionation, commencing within 4 weeks following surgical excision of the tumor, was beneficial for the 5-YSR, but a total radiation dose exceeding 60 Gy did not improve the 5-YSR.

• Radiation Oncology Journal
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• v.12 no.2
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• pp.151-158
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• 1994

Since Jan. 1992, authors have conducted a pilot study to treat malignant glioma with multiple daily fractionated(MDF) radiation therapy and this paper presents the outcome compared MDF to conventional factionated(CF) radiation therapy Between Sep. 1989 and Jan. 1993, forty three patients with high grade glioma of brain except brain stem glioma were treated: nineteen patients were treated with CF radiation therapy and 24 patients were treated with MDF radiation therapy. In CF radiation therapy, total dose was 6300cGy/35fx in 7 weeks, which 5040cGy was delivered to the initial target volume and 1260cGy to reduced target volume. And in MDF radiation therapy, total dose was 6400cGy/40fx in 4 weeks, which 3200cGy was delivered to the initial target volume as 160cGy 2 times daily 6hr apart. All patients had histologically confirmed anaplastic astrocytoma(AA) of glioblastoma multiforme (GBM) with stereotactic biopsy or craniotomy for subtotal or gross tumor resection. The range of follow-up was 7 months to 4 years with a median follow-up of 9 months. The Median survival from surgery was 9 months for all patients. The median survival was 9 months and 10 months for MDF group and CF group and 10 months and 9.5 months for glioblastoma multiforme and anaplastic astrocytoma, respectively. In 36 patients with follow-up CT scan or MRI scan, disease status was evaluated according to treatment groups, Four patients(GBM:3, AA:1) of 21 patients in MDF group, were alive with no evidence of disease, while none of patient was alive with no evidence of disease in CF group. The progression of disease had occurred in 20 patients, 11 patients and 9 patients in MDF group and CF group, respectively All of these patients showed in-field progression of disease, Four of 11 patients($27\%$) in MDF group showed the new leasion outside of the treatment field, while 5 of 9 patients($56\%$) in CF group. In our study the prognosis was not influenced by age, KPS, grade, extent of surgery and different fractional scheduled radiation therapy. Authors concluded that MDF regimen was well tolerated and shortened the treatment period from 7 weeks to 4 weeks without compromising results. We believe that further follow-up is needed to assess the role of MDF.