Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

BACKGROUND/AIMS: Ulcerative colitis undergoes periods of exacerbation and remission. Fecal calprotectin levels increase with gut inflammation and correlate with endoscopic disease activity in ulcerative colitis. Intestinal blood loss and fecal immunochemical test levels also correlate with endoscopic disease activity. This study statistically evaluated the usefulness of fecal calprotectin, fecal immunochemical test, and C-reactive protein (CRP) as markers of disease activity. METHODS: A total 106 ulcerative colitis patients who underwent endoscopy and fecal calprotectin, fecal immunochemical test, and CRP testing, from March 2015 to August 2016, were retrospectively reviewed. Disease activity was assessed using a partial Mayo score and Mayo endoscopic score. The ability of fecal and serologic tests to reflect endoscopic disease severity was statistically evaluated. RESULTS: Among 106 patients, 68 underwent endoscopy and stool study within 2 weeks. In patients with mild to severe activity, fecal immunochemical test and fecal calprotectin were superior to CRP at Mayo endoscopic score detection rate. The area under the curves of fecal immunochemical test and fecal calprotectin for the detection of Mayo endoscopic score ≥1 were 0.956 and 0.942, respectively, and were superior to that of CRP (0.756). At Mayo endoscopic score, the effects of combination of fecal immunochemical test and CRP or fecal calprotectin and CRP were found to be higher than those of the independent fecal immunochemical test or fecal calprotectin. CONCLUSIONS: Fecal immunochemical test and fecal calprotectin can effectively detect active ulcerative colitis better than remission. As these markers reflect the status of mucosal inflammation, they may reduce the requirement for invasive endoscopic examination.
C-Reactive Protein ; Colitis, Ulcerative* ; Endoscopy ; Humans ; Inflammation ; Leukocyte L1 Antigen Complex* ; Retrospective Studies ; Serologic Tests ; Ulcer*

Immunoglobulin A (IgA) nephropathy in patients with the clinical findings of rapidly progressive glomerulonephritis usually demonstrate crescentic glomerulonephritis (CrGN). It is characterized by mesangial immune complex deposits containing IgA and is rarely associated with ANCA. The following case report illustrates a CrGN with mesangial & capillary wall IgA deposits by immunofluorescence (IF) and mesangial & endocapillary electron dense deposits by electron microscopy (EM) in patient with positive ANCA serology. A 54-year-old male visited due to the discomfort of right flank. Blood pressure was 150/90 mmHg. BUN and serum Creatinine was 40 mg/dL, 4.4 mg/dL respectively. Urinalysis revealed protein 2+, >30 RBC's/ HPF (dysmorphic), 24hr urine protein 1,612 mg/day and creatinine clearance 19 mL/min/1.73m2. Serological P-ANCA was positive by indirect immunofluorescence & ELISA. The histologic findings showed global scleorsis and fibrocelluar crescent without hypercellularity on LM, mesangial IgA deposition on IF, mesangial and subendothelial electrone dense material depositions on EM. After treatment (Methylprednisolon 500 mg/day for 3 days and then prednisolone 60 mg/day), Serum creatinin level, proteinuria and ANCA titer were decreased.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic* ; Antigen-Antibody Complex ; Blood Pressure ; Capillaries ; Creatinine ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Male ; Microscopy, Electron ; Middle Aged ; Prednisolone ; Proteinuria ; Urinalysis

Clinical isolates of Enterobacteriaceae (189 Klebsiella, 61 Enterobacter, 32 Serratia, 19 E. coli, 7 Proteus, and 3 Citrobacter) from one university hospital were epidemiologically analyzed by using transferable R plasmids resistant beta-lactam antibiotics including broad-spectrum cephalosporins. About 30% of E. cloacae and S. marcescens and about 5% of K. pneumoniae were resistant to one or more broad-spectrum j3-lactam antibiotics including cefotaxim, ceftazidime, aztreonam, or cefoxitin but all isolates of E. aerogenes, K oxytoca, and P. mirabilis were susceptible. Thirty-six conjugative R plasmids including 8 plasmids resistant expanded-spectrum cephalosporins were obtained from multiple resistant K. pneumoniae (19), E. cloacae (9), E. coli (4), and C. freundii (1). Thirty-one plasmids were subjected to R plasmid analysis and classified 20 different plasmid types. Among them 5, 2, and 2 plasmids belong to 3 different types respectively showed identical molecular size, endonuclease fragment pattern by Southem hybridization pattern by TEM-1 probe, pI value by isoelctric focusing, and also identical antibiogram and biotype of wild strains harboring plasmids. But all of plasmids resistant to cefotaxim, ceftazidime, aztreonam or cefoxitin showed different palsmid anlysis patterns. These results indicate that the epidemic strains of 3 clonal types had been present in this hospital and anlysis using transferable R plasmid and bla gene can be used to discriminate multi-resistant clinical isolates of Enterobacteriaceae.
Anti-Bacterial Agents* ; Aztreonam ; Cefotaxime ; Cefoxitin ; Ceftazidime ; Cephalosporins ; Cloaca ; Dermatoglyphics* ; Enterobacter ; Enterobacteriaceae* ; Klebsiella ; Microbial Sensitivity Tests ; Mirabilis ; Plasmids* ; Pneumonia ; Proteus ; R Factors ; Serratia

BACKGROUND: There has been proposing reports that the autonomic nervous system (ANS) is involved in the pathophysiological mechanism of migraine. However, previous reports suggesting ANS dysfunction during migraine attack was not consistent and were focused mainly on comparison between migraine patients in interictal period and normal controls. The current study is designed to evaluate quantitative evidence of ANS dysfunction by comparing patients in ictal period with those in interictal period. METHODS: We prospectively included 30 migraineurs in ictal period and 30 migraineurs in interictal period from August 2008 to August 2009. Sympathetic function was evaluated by blood pressure response to standing and isometric exercise. Parasympathetic function was evaluated by heart rate response to deep breathing, standing and the Valsalva maneuver. RESULTS: In orthostatic test, falling of systolic blood pressure (SBP) after standing was prominent in ictal group. Although both groups showed elevation of diastolic blood pressure (DBP) after standing, the change was smaller in ictal group than intercital group. Elevation of DBP after isometric exercise was lower in ictal group without statistical significance. Results of parasympathetic function test were not significantly different between ictal and interictal patients. CONCLUSIONS: This study suggests that subjects with migraine had sympathetic hypofunction during migraine attack.
Autonomic Nervous System ; Blood Pressure ; Exercise ; Heart Rate ; Humans ; Migraine Disorders ; Prospective Studies ; Respiration

No abstract available.
Apolipoprotein C-II* ; Apolipoproteins* ; Hep G2 Cells* ; RNA, Messenger*

INTRODUCTION: The authors present a retrospective analysis of the clinical features and prognostic factors of long-term survival in gliomatosis cerebri. METHODS: The authors reviewed the clinical features of 27 cases of gliomatosis cerebri treated between August 1988 and January 2001. Age at diagnosis ranged from 19 to 62(median 41) years and the male to female ratio was 18:9. Most cases presented as a headache or seizure and the mean duration of symptoms was 9.6 months. An ill defined, diffuse high signal intensity lesion extending two lobes or more, without a central necrotic center in T2-weighted magnetic resonance(MR) imaging was characteristic. All patients underwent histological confirmation by craniotomy(12 cases), stereotactic biopsy(14 cases) and stereotactic biopsy followed by craniotomy(1 case). External beam radiation therapy was administered in every case except two, one of which expired within a month of surgery and the other refused treatment. RESULTS: Mean survival time after diagnosis was 41.1 months. By univariate analysis, the symptom duration(lower than 12 months) and the Karnofsky performance scale at discharge(lower than 70), focal enhancement on preoperative MR imaging, postoperative increased intracranial pressure sign, pathologic grade(high grade) and p53(>5%) were correlated with the length of survival(p-values were 0.07, 0.00, 0.007, 0.001, 0.04, and 0.02 respectively). CONCLUSION: We suggest that:1) gliomatosis cerebri can be diagnosed by a combination of MR imaging and histopathological examination:2) confusion with extensive glioma is possible due to vague diagnostic criteria, and so survival might be better than expected had discrete diagnosis been made. 3) Initial active management for increased ICP and further radiation therapy might be an important therapy.
Biopsy ; Diagnosis ; Female ; Glioma ; Headache ; Humans ; Intracranial Pressure ; Magnetic Resonance Imaging ; Male ; Neoplasms, Neuroepithelial ; Retrospective Studies ; Seizures ; Survival Rate

Rhabdoid meningioma is an uncommon meningioma variant, which was initially described in 1998 by Perry et al.. It is classified as a variant corresponding to grade III in the recent World Health Organization(WHO) classification of tumors of the nervous system, because of its unique histopathological features and its highly aggressive clinical course. To date there were still a lot of arguments about their developmental patterns and the treatment strategy especially for rhabdoid meningiomas lacking other histological features of malignancy. Although the exact causes of rhabdoid transformation of a benign meningioma are unknown, malignant transformation long duration after surgical resection under an underlying predilection for malignancy is suggested in our case. And we suggest that any histological rhabdoid features in recurrent meningiomas or even in primary cases seem to indicate the malignant clinical course and the need for aggressive treatments, because transformation from a benign or atypical one to a malignant one seems to occur at last.
Classification ; Meningioma* ; Nervous System Neoplasms ; World Health ; World Health Organization

Myxoma is the most common primary tumor in the heart. Cardiac myxomas can present in various manners including embolization and fever, sometimes simulating endocarditis. However, they are rarely infected. We report here a case of an infected left atrial myxoma that seeded a normal mitral valve and atypically presented with multiple embolic events in the lower extremities along with multiple splenic and a cerebellar infarction.
Endocarditis ; Fever ; Heart ; Infarction ; Lower Extremity ; Mitral Valve ; Myxoma ; Seeds

Cogan's syndrome is a rare systemic inflammatory disease and can be diagnosed on the basis of typical inner ear and ocular involvement with the presence of large vessel vasculitis. We report a case of Cogan's syndrome with stable angina resulting from coronary ostial stenosis caused by aortitis.
Angina Pectoris ; Angina, Stable ; Aortitis ; Cogan Syndrome ; Constriction, Pathologic ; Ear, Inner ; Glycosaminoglycans ; Vasculitis