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.
The precise role of radiotherapy for low grade gliomas including the optimal radiation dose and timing of treatment remains unclear. The information given by a retrosepctive analysis may be useful in the design of prospective randomized studies looking at radiation dose and time of surgical and radiotherapeutic treatment. The records of 56 patients (M:F = 29:27) with histologically verified cerebral low grade gliomas (47 cases of grade 1 or 2 astrocytomas and 9 oligodendrogliomas) diagnosed between 1979 and 1989 were retrospectively reviewed. The extent of surgical tumor removal was gross total or radical subtotal in 38 patients ($68\%$) and partial or biopsy only in the remaining 18 patients ($32\%$). Postooperative radiation therapy was given to 36 patients ($64\%$) of the total 56 patients with minimum dose of 5000 cGy (range=1250 to 7220 cGy). The 5-and 10-year survival rates for the total 56 patients were $44\%$ and $32\%$ respectively with a median survival of 4.1 years. According to the histologic grade the 5- and 10-year survivals were $52\%$ and $35\%$ for the 24 patients respectively with grade I astrocytomas compared to $20\%$ and $10\%$ for the 23 patients with grade II astrocytomas. Survival of oligodendroglioma patients was greater than those with astrocytoma ($65\%$ vs $36\%$ at 5 years), and the difference was also remarkable in the long term period of follow up ($54\%$ vs $23\%$ at 10 years). Those who received high-dose radiation therapy ($\geq$5400 cGy) had significant better survival than those who received low-dose radiation (< 5400cGy) or surgery alone (p<0.05). The 5- and 10-year survival rates were, respectively $59\%$ and $46\%$ for the 23 patients receiving high-dose radiation, $36\%$ and $24\%$ for the 13 patients receiving low-dose radiation, and $35\%$ and $26\%$ for the 20 patients with surgery alone. Survival rates by the extent of surgical resection were similar at 5 years ($46\%$ vs $41\%$), but long term survival was quite different (p<0.01) between total/subtotal resection and partial resection/biopsy ($41\%$ and $12\%$, resepctively). Previously published studies have identified important prognostic factors in these tumor: age, extent of surgery, grade, performance status, and duration of symptoms. But in our cases statistical analysis revealed that grade I histology (p<0.025) and young age (p<0.001) were the most significant good prognostic variables.
To predict the tumor grading, various imaging modalities have been applied clinically. This study determines clinical usefulness of perfusion MRI, using relative cerebral blood volume in grading of the gliomas. We did a retrospective review of 17 patients (mean age, 57.5 years; 11 male, 6 female) who underwent perfusion MR and conventional MRI, and then correlated pathologically after operation. Statistical analysis of regional cerebral blood volume and relative cerebral blood volume(rCBV) was performed by using softwares such as PAT by SIEMENS and Xmap ver 2.0 developed by ourselves. Six patients out of 13 were low-grade gliomas while eleven patients were the high-grade gliomas. Mean relative CBV (m_rCBV/white matter) in the low-grade gliomas was 1.62, and mean relative CBV(m_rCBV/cortex) was 0.12. In the high-grade gliomas, mean relative CBV(m_rCBV/white matter) and mean relative CBV(m_rCBV/cortex) were 33.53 and 0.96. Mean relative CBV of gliomas were elevated with a statistical difference(P<.05), compared with contralateral white matter(P=.019) or cortex(P=.025). Furthermore mean relative CBV(m_rCBV/white matter) was much higher than mean relative CBV(m_rCBV/cortex). Perfusion MRI using regional cerebral blood volume and rCBV is very useful imaging modality for grading the glioma.
Yang Kang Mo;Chang Hye Sook;Ahn Seoung Do;Choi Eun Kyung
Radiation Oncology Journal
/
v.12
no.2
/
pp.151-158
/
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.
Kim, Jeong-Kwon;Jung, Tae-Young;Lee, Kyung-Hwa;Kim, Seul-Kee
Journal of Korean Neurosurgical Society
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v.57
no.4
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pp.307-310
/
2015
We report a case of a paradoxical response of a tuberculoma in the brain mimicking a brain tumor. A 76-year-old woman presented with a 2 week history of headache, dysarthia, and orthopnea. Brain magnetic resonance images (MRI) revealed two rim-enhancing lesions on the pons and occipital lobe, and chest computed tomography showed randomly distributed miliary nodules. The tentative diagnosis was tuberculosis (TB) of the brain and lung. She complained of right hemiparesis and worsening general weakness after taking the anti-TB medication. On the monthly follow-up images, the enhanced lesions were enlarged with increased perfusion and choline/creatinine ratio, suggesting a high grade glioma. A surgical resection was completed to diagnose the occipital lesion, and the tuberculoma was pathologically confirmed by a positive TB-polymerase chain reaction. The anti-TB medication was continued for 13 months. A follow-up MRI showed decreased size of the brain lesions associated with perilesional edema, and the clinical symptoms had improved. Brain tuberculoma could be aggravated mimicking brain malignancy during administration of anti-TB medication. This paradoxical response can be effectively managed by continuing the anti-TB drugs.
Purpose: The purpose of this narrative study was to understand how family caregivers interpreted themselves life during caring for dying patients with gliobalstoma, and how they integrated these experiences into their personal biographies. Methods: Three family caregivers were recruited for the study. Data were collected through a series of audio-taped unstructured interviews and conversations with participants. The interviews and observation were conducted between October and November, 2011. Data were analyzed using psychosocial analytical methods that combined case based, in-depth staged analysis of narratives. Results: The life experiences of the family caregivers with a dying family member were summarized as, in their own voices, 'the repetition of gliobalstoma,' 'a smart patient,' 'being obsessed with rehabilitation treatment,' 'the frustration from nothing but just looking at the suffering of the patient,' and 'a stubborn son'. Conclusion: Caregiving was characterised by various roles and life changes from the moment of diagnosis. Family caregivers of brain tumor reported experiences similar to those described by caregivers of people with other cancers. What differed for this group was the rapidity of change and the need for immediate information and support to assist with caring for a person with high-grade glioma.
Glioblastoma (GBM) is the most common and aggressive form of human adult brain malignancy. The identification of the cell of origin harboring cancer-driver mutations is the fundamental issue for understanding the nature of GBM and developing the effective therapeutic target. It has been a long-term hypothesis that neural stem cells in the subventricular zone (SVZ) might be the origin-of-cells in human glioblastoma since they are known to have life-long proliferative activity and acquire somatic mutations. However, the cell of origin for GBM remains controversial due to lack of direct evidence thereof in human GBM. Our recent study using various sequencing techniques in triple matched samples such as tumor-free SVZ, tumor, and normal tissues from human patients identified the clonal relationship of driver mutations between GBM and tumor-free SVZ harboring neural stem cells (NSCs). Tumor-free SVZ tissue away from the tumor contained low-level GBM driver mutations (as low as 1% allelic frequency) that were found in the dominant clones in its matching tumors. Moreover, via single-cell sequencing and microdissection, it was discovered that astrocyte-like NSCs accumulating driver mutations evolved into GBM with clonal expansion. Furthermore, mutagenesis of cancer-driving genes of NSCs in mice leads to migration of mutant cells from SVZ to distant brain and development of high-grade glioma through the aberrant growth of oligodendrocyte precursor lineage. Altogether, the present study provides the first direct evidence that NSCs in human SVZ is the cell of origin that develops the driver mutations of GBM.
Tae Woong Hwang;Dong Hun Kim;Da Bi Kim;Tae Won Jang;Gun-Hwa Kim;Minho Moon;Kyung Ah Yoon;Dae Eun Choi;Jae Ho Park;Jwa-Jin Kim
International Journal of Molecular Medicine
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v.43
no.3
/
pp.1478-1486
/
2019
Temozolomide (TMZ) is an alkylating agent commonly used as a first-line treatment for high-grade glioblastoma. However, TMZ has short half-life and frequently induces tumor resistance, which can limit its therapeutic efficiency. In the present study, it was hypothesized that combined treatment with TMZ and acteoside has synergistic effects in glioblastoma therapy. Using cell viability and wound-healing assays, it was determined that this treatment regimen reduced cell viability and migration to a greater extent than either TMZ or acteoside alone. Following previous reports that TMZ affected autophagy in glioma cells, the present study examined the effects of TMZ + acteoside combination treatment on apoptosis and autophagy. The TMZ + acteoside combination treatment increased the cleavage of caspase-3 and levels of B-cell lymphoma 2 (Bcl-2)-associated X protein and phosphorylated p53, and decreased the level of Bcl-2. The combination treatment increased microtubule-associated protein 1 light chain 3 and apoptosis-related gene expression. It was also determined that TMZ + acteoside induced apoptosis and autophagy through the mitogen-activated protein kinase signaling pathway. These findings suggest that acteoside has beneficial effects on TMZ-based glioblastoma therapy.
Purpose : This study was conducted to assess how effective the permeability ratio and relative cerebral blood volume ratio are to tumor through perfusion MRI by measuring and reflecting the grade assessment and differential diagnosis and the permeability and relative cerebral blood volume of contrast media plunged from blood vessel into organ due to breakdown of blood-brain barrier in cerebral. Subject and Method : Subject of study was 29 patients whose diagnosis were confirmed by biopsy after surgery and 550 (11 slice$\times$50 image) perfusion MRI were used to make image of relative cerebral blood volume with the program furnished on instrument. The other method was to transmit to private computer and the image analysis was made additionally by making image of relative cerebral blood volume-reformulated singular value decomposition, rCBV-rSVD and permeability using IDL.6.2. In addition, Kruskal-wallis test tonggyein non numerical average by a comparative analysis of brain tumors Results : The rCBV ratio (Functool PF; GE Medical Systems and IDL 6.2 program by analysis) and permeability ratio of tumors were as follows; high grade glioma(n=4), (14.75, 19.25) 13.13. low grade astrocytoma(n=5) (14.80, 15.90) 11.60, glioblastoma(n=5) (10.90, 18.60), 22.00, metastasis(n=6) (11.00, 15.08). 22.33. meningioma(n=6) (18.58, 7.67), 5.58. oliogodendroglioma(n=3) (23.33, 16.33, 15.67. Conclusion : It was not easy to classify the grade with the relative cerebral blood volume ratio measured by using the relative cerebral blood image by type of tumors, however, permeability ratio measured by permeability image revealed that the higher the grade of tumor, the higher the measured permeability ratio, showing the assessment of tumor grade is more effective to differential diagnosis.
Kim, Woo-Cheol;Suh, Chang-Ok;Pyo, Hong-Ruyll;Loh, John-J.K;Kim, Gwi-Eon
Radiation Oncology Journal
/
v.10
no.1
/
pp.15-20
/
1992
A total of 20 patients (male: female=10:10, adult:children=8:12) with brainstem tumors had been received radiation therapy in the Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine between 1980 and 1990. Thirteen of 20 patients were treated with conventional radiation therapy (before 1989, 180~200 cGy per fraction, 5 days a week, total dose 4680~5400 cGy), and seven patients were treated with hyperfractionated radiation therapy (in 1990, 100 cGy per fraction, twice daily 10 fractions a week, total dose 7200 cGy). Median follow up Periods for conventional radiation therapy group and hyperfractionated radiation therapy group were 36 months and 10 months, respectively. Four of 20 Patients had histopathologic diagnosis prior to treatment; 3 cases were low grade astrocytoma and 1 case was high grade astrocytoma. Overall 2-year actuarial survival rate was 30%. The prognosis of patients with a longer duration of symptom and sign was better (60%, vs 12%), and the adult (52%) was better than children (14%). There was no significant difference between the focal (29%) and diffuse (26%) type. The initial clinical response was better in the hyperfractionated radiation therapy group. Because of the relatively small number of Patients and short follow up Period in hyperfractionated radiation therapy group, there was no comparison between two group.
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