• Journal of Korean Neurosurgical Society
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• v.49 no.6
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• pp.334-338
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• 2011

Objective : The incidence of leptomeningeal dissemination from malignant glioma is rare, so the clinical features of this are not well documented yet We attempted to determine the clinical features of leptomeningeal dissemination from malignant gliomas. Methods : We retrospectively analyzed 11 cases of leptomeningeal dissemination of malignant glioma, who were treated at our institution between 2006 and 2009. We investigated the clinical features of these patients by considering the following factors : tumor locations, the events of ventricular opening during surgery and the cerebrospinal fluid (CSF) profiles, including the cytology. Results : The group was composed of 9 males and 2 females. The histological diagnosis of their initial intracranial tumors were 4 primary glioblastoma, 3 anaplastic astrocytoma, 1 anaplastic oligoastrocytoma, 2 ganglioglioma and 1 pleomorphic xanthoastrocyotma with anaplastic features. The mean age of the patients at the time of the initial presentation was $42.8{\pm}10.3$ years. The mean time between surgery and the diagnosis of spinal dissemination was $12.3{\pm}7.9$ (3-28) months. The mean overall survival after dissemination was $2.7{\pm}1.3$ months. All our patients revealed a history of surgical opening of the ventricles. Elevated protein in the CSF was reported for eight patients who had their CSF profiles checked. Conclusion : We propose that in the malignant gliomas, the surgical opening of ventricles can cause the spinal leptomeningeal dissemination and the elevated protein content of CSF may be a candidate marker of leptomeningeal dissemination.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.321-326
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• 2020

There has been confusion in the classification of terminal myelocystocele (TMCC) due to its diverse morphology and vague pathoembryogenesis. TMCC could be summarized as having the essential features of an elongated caudal spinal cord extruding out of the dorsal extraspinal space that fuses with the subcutaneous fat, which is in the shape of a trumpet-shaped cerebrospinal fluid-filled cyst. The extraspinal portion of the extruded spinal cord is nonfunctional. The morphological features suggest that TMCC is formed during secondary neurulation, specifically the failure of the degeneration of the secondary neural tube near the time of the terminal balloon. This review discusses the definition, as well as the clinical and surgical features, of TMCC with special emphasis on its pathoembryogenesis.

• Korean Journal of Radiology
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• v.21 no.5
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• pp.537-540
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• 2020

Recently, some global cases of 2019 novel coronavirus (COVID-19) pneumonia have been caused by second- or third-generation transmission of the viral infection, resulting in no traceable epidemiological history. Owing to the complications of COVID-19 pneumonia, the first symptom and imaging features of patients can be very atypical and early diagnosis of COVID-19 infections remains a challenge. It would aid radiologists and clinicians to be aware of the early atypical symptom and imaging features of the disease and contribute to the prevention of infected patients being missed.

• Annals of Clinical Neurophysiology
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• v.25 no.2
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• pp.84-92
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• 2023

Background: Clinical spectrum of immunoglobulin M (IgM) monoclonal gammopathy varies from IgM monoclonal gammopathy of unknown significance (IgM-MGUS) to hematological malignancies. We evaluated the clinical features, electrophysiological characteristics, and prognosis of patients with peripheral neuropathy associated with IgM monoclonal gammopathy (PN-IgM MG). Methods: We retrospectively evaluated 25 patients with PN-IgM MG. Peripheral neuropathy was classified as axonal, demyelinating, or undetermined, based on electrophysiological studies. We classified the enrolled patients into the IgM-MGUS and malignancy groups, and compared the clinical and electrophysiological features between the groups. Results: Fifteen patients had IgM-MGUS and 10 had hematologic malignancies (Waldenström's macroglobulinemia: two and B-cell non-Hodgkin's lymphoma: eight). In the electrophysiological evaluation, the nerve conduction study (NCS) criteria for demyelination were met in 86.7% of the IgM-MGUS group and 10.0% of the malignancy group. In particular, the distal latencies of the motor NCS in the IgM-MGUS group were significantly prolonged compared to those in the malignancy group (median, 9.1 ± 5.1 [IgM-MGUS], 4.2 ± 1.3 [malignancy], p = 0.003; ulnar, 5.4 ± 1.9 [IgM-MGUS], 2.9 ± 0.9 [malignancy], p = 0.001; fibular, 9.3 ± 5.1 [IgM-MGUS], 3.8 ± 0.3 [malignancy], p = 0.01; P-posterior tibial, 8.3 ± 5.4 [IgM-MGUS], 4.4 ± 1.0 [malignancy], p = 0.04). Overall treatment responses were significantly worse in the malignancy group than in the IgM-MGUS group (p = 0.004), and the modified Rankin Scale score at the last visit was higher in the malignancy group than in the IgM-MGUS group (2.0 ± 1.1 [IgM-MGUS], 4.2 ± 1.7 [malignancy], p = 0.001), although there was no significant difference at the initial assessment. Conclusions: The risk of hematological malignancy should be carefully assessed in patients with PN-IgM MG without electrophysiological demyelination features.

• Korean Journal of Radiology
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• v.23 no.9
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• pp.921-930
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• 2022

Objective: To identify epidermal growth factor receptor (EGFR) mutations in lung adenocarcinoma based on ¹⁸F-fluorodeoxyglucose (FDG) PET/CT radiomics and clinical features and to distinguish EGFR exon 19 deletion (19 del) and exon 21 L858R missense (21 L858R) mutations using FDG PET/CT radiomics. Materials and Methods: We retrospectively analyzed 179 patients with lung adenocarcinoma. They were randomly assigned to training (n = 125) and testing (n = 54) cohorts in a 7:3 ratio. A total of 2632 radiomics features were extracted from the tumor region of interest from the PET (1316) and CT (1316) images. Six PET/CT radiomics features that remained after the feature selection step were used to calculate the radiomics model score (rad-score). Subsequently, a combined clinical and radiomics model was constructed based on sex, smoking history, tumor diameter, and rad-score. The performance of the combined model in identifying EGFR mutations was assessed using a receiver operating characteristic (ROC) curve. Furthermore, in a subsample of 99 patients, a PET/CT radiomics model for distinguishing 19 del and 21 L858R EGFR mutational subtypes was established, and its performance was evaluated. Results: The area under the ROC curve (AUROC) and accuracy of the combined clinical and PET/CT radiomics models were 0.882 and 81.6%, respectively, in the training cohort and 0.837 and 74.1%, respectively, in the testing cohort. The AUROC and accuracy of the radiomics model for distinguishing between 19 del and 21 L858R EGFR mutational subtypes were 0.708 and 66.7%, respectively, in the training cohort and 0.652 and 56.7%, respectively, in the testing cohort. Conclusion: The combined clinical and PET/CT radiomics model could identify the EGFR mutational status in lung adenocarcinoma with moderate accuracy. However, distinguishing between EGFR 19 del and 21 L858R mutational subtypes was more challenging using PET/CT radiomics.

• Sleep Medicine and Psychophysiology
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• v.1 no.1
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• pp.60-67
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• 1994

Objectives: The Obstructive sleep apnea syndrome is characterized by snoring, observed apnea during sleep and excessive daytime sleepiness. The overnight polysomnographic recording is used to investigate patients with possible obstructive sleep apnea syndrome. But the overnight polysomnographic recording is time consuming, expensive, and labor-intensive. Recently in diagnosis of obstructive sleep apnea syndrome, several simple screening tests have been suggested. Methods: This study was performed to assess the probability of application of clinical features and the degree of oxygen desaturation as a screening test for the obstructive sleep apnea syndrome. The sensitivity and specificity of the self-report of clinical features including snoring, observed apnea during sleep, excessive daytime sleepiness and insomnia were tested. And the degree of oxygen desaturation measured by oximetry in 42 subjects were compared with the overnight polysomnographic recording results. Results: In the prediction of apnea index more than 5, the sensitivity of observed apnea during sleep, snoring, excessive daytime sleepiness and insomnia were 96.8%, 93.5%, 38.7%, 25.8% and the specificity of those clinical features were 182%, 36.4%, 100%, 72.7%, respectively. In the prediction of apnea index more than 5, the sensitivity and specificity of the combination of more than three self-report clinical features were 54.8% and 90.9%. The degree of oxygen de saturation and maximal apnea duration in the group of apnea index more than 5 were significantly different from those in the group of apnea index below 5(P<0.001). And the apnea index was significantly correlated with the degree of oxygen desaturation and maximal apnea duration(P<0.001). Conclusion: These results suggest that application of clinical features alone as a screening test for the obstructive sleep apnea syndrome is inadequate because of it's high rate of false positive and false negative results. The degree of oxygen desaturation measured by oximetry is possibly applicable to screening test and follow up evaluation of treatment efficacy for the obstructive sleep apnea syndrome.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.16
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• pp.7195-7200
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• 2015

Background: Since seventeen employees of an offset printing company in Osaka, Japan developed cholangiocarcinoma it has become recognized as an occupational cancer. This study investigated the differences of clinical features between occupational cholangiocarcinoma and sporadic young-onset cholangiocarcinoma. Materials and Methods: Thirty-four young adults (<50 years old) with sporadic cholangiocarcinoma were extracted from the Rosai Hospital Group database (sporadic group) and their clinical features were compared with those of 17 patients with occupational cholangiocarcinoma (occupational group). Results: The 34 patients in the sporadic group were treated for cholangiocarcinoma at 16 different Rosai hospitals. There were significant differences of age (p<0.01), gender (p<0.01), abnormal laboratory tests (p<0.01), and tumor location (p<0.01) between the two groups. The percentage of patients with abnormal laboratory tests was significantly higher in the occupational group than in the sporadic group (p<0.001). Regional dilation of bile ducts, which is a characteristic of occupational cholangiocarcinoma, was not observed in the sporadic group. Conclusions: No cluster of cholangiocarcinoma cases was identified in the Rosai Hospital database. There were differences of clinical features between occupational and sporadic cholangiocarcinoma, which might be helpful for diagnosing occupational cholangiocarcinoma in the future.

• Imaging Science in Dentistry
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• v.47 no.2
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• pp.135-140
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• 2017

Squamous cell carcinoma (SCC) arising within the lining of an odontogenic keratocyst (OKC) is a rare occurrence. Although potentially locally destructive, OKC is a benign odontogenic process that typically presents with clinical and radiographic features characteristic of a benign intraosseous neoplasm. We present the clinical and radiographic features of a maxillary mass that demonstrated SCC arising from the lining of an OKC. Although the initial clinical and radiographic presentation suggested an infection or malignant neoplasm, biopsies revealed an infiltrative well-differentiated SCC contiguous with and arising from the focus of a pre-existing OKC. The patient subsequently underwent a type II hemi-maxillectomy with neoadjuvant chemoradiation. This report discusses the clinical and radiographic features associated with intraosseous malignancies, especially those arising from an otherwise benign odontogenic lesion. While the majority of OKCs are benign, the current report illustrates the potential for carcinomatous transformation within the lining of an OKC.

• Journal of Oral Medicine and Pain
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• v.41 no.3
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• pp.118-125
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• 2016

Purpose: The aims of this study were to evaluate the clinical characteristics of patients with altered sensation and/or pain, and to determine outcome predictors affecting persistent neuropathy. Methods: Patients who complained an altered sensation or pain following trigeminal nerve trauma were involved in this study. To determine outcome predictors affecting persistent neuropathy, the patients were divided into two groups; transient vs. persistent, and the clinical phenotypes are compared between groups. Data were analyzed with t-tests, chi-square, and multiple regression analyses with 95% confidence interval and p<0.05 significance level. Results: A total of 111 patients were included: 23 with transient and 88 persistent groups. The panoramic result and pin-prick test score were statistically different between the groups. Radiating symptoms after blunt and pinprick stimuli were also significantly different between groups. The results revealed that the presence of a neurologic lesion in the panoramic view result, reduced sensation in the pinprick test, and radiation in the pinprick test could affect the persistent group. Conclusions: The presence of a neurologic lesion in panoramic view result and reduced sensation and radiating symptoms in the pin prick test would be defining features of one of the main clinical features of persistent neuropathy. These features could serve as outcome predictors diagnosing the permanent nerve injury in trigeminal nerve.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1140-1151
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• 2006

Inherited metabolic disorders are individually rare but as a whole, they are nor rare. Since Archibald Garrod introduced a concept of "inborn error of metabolism" or "chemical individuality", more than 500 diseases are currently known, affecting approximately one in 500 newborns cumulatively. They frequently manifest with acute, life-threatening crisis that require immediate specific intervention or they present with insidious diverse symptoms and signs involving multiple visceral organs or tissues as well as central nervous system, hampering a correct diagnosis. In addition, many pediatricians are not familiar with all diagnostic and therapeutic strategies for diverse inherited metabolic disorders. However, the prognosis of affected children are heavily dependent on rapid and effective treatment. In this lecture, practical guidelines for the specific diagnosis based on diverse clinical features of inherited metabolic disorders will be described. Many sophisticated laboratory tests are available for confirmatory diagnosis of each disease, which challenge to general pediatricians with respect to knowledge about biochemical metabolite assay test, enzymatic test and DNA diagnostic tests. Sample collections, indications, methods and interpretation of results in varying laboratory tests will be listed as well.