• Annals of Clinical Neurophysiology
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• 제25권2호
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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.

• 수면정신생리
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• 제1권1호
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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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• 제16권16호
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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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• 제47권2호
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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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• 제41권3호
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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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• 제49권11호
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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.

• 대한견주관절학회:학술대회논문집
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• 대한견주관절학회 2005년도 제13차 춘계학술대회
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• pp.1-1
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• 2005

• 한국임상약학회지
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• 제30권4호
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• pp.270-278
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• 2020

Background: Although the identification of clinical and laboratory features in pediatric COVID-19 patients is essential in establishing an appropriate treatment plan, a systematic review and meta-analysis on the topic has yet to be reported. Methods: We searched MEDLINE, Embase, and Web of Science to access clinical and laboratory characteristics as well as clinical outcomes of children with COVID-19 infection. A meta-analysis using random-effect model was performed to estimate pooled prevalence and 95% confidence intervals. Results: Among the 532 studies initially collected, 12 articles were finally included in the meta-analysis. Among the investigated 320 pediatric patients with COVID-19, fever (48.2%) and cough (39.3%) were the most common symptoms. Almost one third of patients (30.4%) were asymptomatic. In laboratory findings, only 11.4% of pediatric patients experienced lymphocytopenia. Increased inflammatory markers including c-reactive protein (18.6%) and procalcitonin (32.4%) were observed. Only a few patients needed mechanical ventilation and intensive care support, and only one death was reported. Conclusion: Pediatric patients with COVID-19 infection exhibited milder symptoms and more favorable outcomes compared to adults. However, considering the high rate of asymptomatic pediatric patients, close monitoring is required to prevent community infection in asymptomatic conditions and hidden disease progression.

• Journal of Interdisciplinary Genomics
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• 제2권1호
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• pp.1-4
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• 2020

Recent advances in magnetic resonance imaging and identification of causative genes led to the recognition of a new group of disorders named neurodegeneration with brain iron accumulation (NBIA). NBIA is a group of inherited disorders characterized by abnormal iron deposition in the brain, usually in the basal ganglia. The disorder shares the clinical features of movement disorders and is accompanied by varying degrees of neuropsychiatric abnormalities. In this review, the causative genes, clinical presentations, neuroimaging features, and pathological findings are summarized.

• Annals of Clinical Neurophysiology
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• 제4권2호
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• pp.98-107
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• 2002

Multifocal motor neuropathy (MMN) is a chronic immune-mediated peripheral myelinopathy. The major clinical features include slowly progressive, painless, and asymmetric weakness, usually of distal limb muscle. Early in the course of the disease, weakness is not necessarily associated with muscle atrophy, owing to the initial primary involvement of peripheral myelin. Chronic progressive weakness is often associated with some degree of concurrent axonal loss and subsequent muscle atrophy. Sensory symptoms are usually mild or absent, and involvement of cranial and respiratory muscles is rare. The findings of multifocal motor conduction block, abnormal temporal dispersion, and focal conduction slowing at segments not at risk for common entrapment or compression injury, associated with normal sensory conduction studies along the same segments, are the hallmark electrophysiologic features of MMN. The slow progression and absence of upper motor neuron signs are the major clinical points that separate MMN from amyotrophic lateral sclerosis. The role of GM1 antibodies, found in high titers in 22~84% of MMN patients, remains uncertain. The contention that MMN is an autoimmune disorder is largely based on the often dramatic improvement in symptoms following the administration of intravenuos immunoglobulin or cyclophosphamide.