Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
Volume 57 , Issue 4
Showing 1-30 articles out of 30 articles from the selected issue
EDITORIAL
ORIGINAL ARTICLES
  • Yoshitaka Takuma, Iwadou Shota, Hirokazu Miyatake, Shuji Uematsu, Ryou ...
    2018 Volume 57 Issue 4 Pages 457-468
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    Objective The purpose of this study was to construct nomograms for the disease-free survival (DFS) and overall survival (OS) of post-radiofrequency ablation (RFA) patients with hepatocellular carcinoma (HCC). Furthermore, we compared the prognostic predictive ability of these nomograms for estimating per-patient outcomes with that of traditional staging systems.

    Methods We retrospectively enrolled 298 patients in the training set and 272 patients in the validation set who underwent RFA for HCC. The nomograms for the DFS and OS were constructed from the training set using the multivariate Cox proportional hazards model. The discriminatory accuracy of the models was compared with traditional staging systems by analyzing the Harrell's C-index.

    Results The DFS nomogram was developed based on the tumor size, tumor number, aspartate aminotransferase (AST), albumin, age, and α-fetoprotein. The OS nomogram was developed based on the tumor size, the model for end-stage liver disease, AST, and albumin. Our DFS and OS nomograms had good calibration and discriminatory abilities in the training set, with C-indexes of 0.640 and 0.692, respectively, that were greater than those of traditional staging systems. The C-indexes of our DFS and OS nomograms were also greater than those of traditional staging systems in the validation set, with C-indexes of 0.614 and 0.657, respectively. RFA patients were stratified into low- and high-risk groups based on the median nomogram scores. High-risk patients receiving surgical resection (SR) were associated with a better DFS and OS than those undergoing RFA. However, the DFS and OS were similar between the low-risk RFA and SR groups.

    Conclusion We constructed reliable and useful nomograms that accurately predict the DFS and OS after RFA for early-stage HCC patients. These graphical tools are easy to use and will assist physicians during the therapeutic decision-making process.

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  • Hirokazu Saito, Yoshihiro Kadono, Kentaro Kamikawa, Atsushi Urata, Har ...
    2018 Volume 57 Issue 4 Pages 469-477
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    Objective Single-stage endoscopic stone removal for choledocholithiasis is an advantageous approach because it is associated with a shorter hospital stay; however, few studies have reported the incidence of complications related to this procedure in detail. The aim of this study was to examine the incidence of complications and efficacy of this procedure.

    Methods This retrospective study investigated the incidence of complications in 345 patients with naive papilla who underwent therapeutic endoscopic retrograde cholangiopancreatography (ERCP) for choledocholithiasis at three institutions between April 2014 and March 2016 by a propensity score analysis. The efficacy of single-stage endoscopic stone removal was assessed based on a hospital stay of within 7 days and the number of ERCP attempts.

    Results Among 114 patients who underwent single-stage endoscopic stone removal, 15 patients (13.2%) experienced complications. Among the remaining 231 patients in the two-stage endoscopic stone removal group, complications were observed in 17 patients (7.4%). The propensity score analysis, which was adjusted for confounding factors, revealed that single-stage endoscopic stone removal was not a significant risk factor for complications (p=0.52). In patients in whom >10 min was required for deep cannulation, single-stage endoscopic stone removal was not a significant risk factor for complications in the propensity score analysis (p=0.37). In the single-stage group, the proportion of patients with a hospital stay of within 7 days was significantly higher and the number of ERCP attempts was significantly lower in comparison to the two-stage group (p <0.0001 and <0.0001, respectively).

    Conclusion Single-stage endoscopic stone removal did not increase the incidence of complications associated with ERCP and was effective for reducing the hospital stay and the number of ERCP attempts.

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  • Satoshi Ito, Daisuke Kobayashi, Akira Murasawa, Ichiei Narita, Kiyoshi ...
    2018 Volume 57 Issue 4 Pages 479-485
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    Objective To analyze the neuropathic pain (NeP) components in patients with rheumatoid arthritis (RA).

    Methods The painDETECT questionnaire (PD-Q) was completed by 300 RA patients (79 men, 221 women).

    Results Nine patients (3.0%) were categorized as likely NeP, 33 (11.0%) were categorized as possible NeP, and 258 (86.0%) were categorized as unlikely NeP. When we excluded patients with diabetes mellitus, spinal diseases, neurological diseases, and herpes zoster infection (conditions associated with NeP), 5 of the patients (1.7%) had likely NeP, and 23 (7.7%) had possible NeP without any underlying conditions. Furthermore, there were no marked differences in the percentages of these underlying conditions among the patients with likely, possible, and unlikely NeP. When we compared patients with likely and possible NeP (n=42) and unlikely NeP (n=258), the body mass index (BMI), disease activity score-28 based on the erythrocyte sedimentation rate, C-reactive protein level, pain visual analogue scale (VAS), and PD-Q score were significantly higher in the patients with likely and possible NeP than in those with unlikely NeP. A multivariate analysis showed that BMI ≥22 and not being in clinical remission were associated with NeP.

    Conclusion Although RA pain has usually been classified as nociceptive pain, the present study clarified that a significant number of patients might have NeP. The present findings suggest that high disease activity and being overweight are related to NeP in RA patients.

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  • Hideyuki Horie, Isao Ito, Satoshi Konishi, Yuki Yamamoto, Yuko Yamamot ...
    2018 Volume 57 Issue 4 Pages 487-495
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: October 11, 2017
    JOURNALS OPEN ACCESS

    Objective In the past decade, extended-spectrum β-lactamase (ESBL)-producing bacteria have increasingly frequently been isolated from various kinds of clinical specimens. However, the appropriate treatment of pneumonia in which ESBL-producing bacteria are isolated from sputum culture is poorly understood. To investigate whether or not ESBL-producing bacteria isolated from sputum in pneumonia cases should be treated as the causative bacteria.

    Methods and Patients In this retrospective study, we screened for patients, admitted between January 2009 and December 2015 in whom pneumonia was suspected and for whom sputum cultures yielded Escherichia coli or Klebsiella spp. isolates. We identified patients with community-acquired pneumonia (CAP) or healthcare-associated pneumonia (HCAP) from whom ESBL-producing bacteria had been isolated from sputum culture and to whom antibiotic treatment had been given with a diagnosis of pneumonia. We analyzed the patients' backgrounds and the effect of the antibiotic treatment for the initial 3-5 days.

    Results From 400 patients initially screened, 27 with ESBL-producing bacteria were secondarily screened. In this subset of patients, 15 were diagnosed with pneumonia, including 7 with CAP (5 E. coli and 2 K. pneumoniae) and 8 with HCAP (8 E. coli). These patients exhibited an average age of 84.1 years old, and 9 of 15 were men. No patients were initially treated with antimicrobials that are effective against isolated ESBL-producing bacteria. However, 13 of 15 patients showed improvement of pneumonia following the initial antibiotic treatment.

    Conclusion ESBL-producing bacteria isolated from sputum are not likely to be the actual causative organisms of pneumonia.

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  • Mao Hagihara, Yukiko Kato, Ai Kurumiya, Tomoko Takahashi, Miki Sakata, ...
    2018 Volume 57 Issue 4 Pages 497-501
    Published: February 15, 2018
    Released: February 15, 2018
    JOURNALS OPEN ACCESS

    Objective From November 24 to December 9, 2013, an outbreak of the influenza (flu) A (H3) virus occurred in a tertiary-care university hospital (1,014 beds). We herein report the prophylactic effect of anti-flu agents for controlling the flu outbreak.

    Methods We administered pre- or post-exposure prophylaxis with anti-flu agents in flu outbreak. To test the effectiveness of prophylaxis in a flu outbreak, we used the posterior mean of the reproductive value during the pre- and post-intervention period. We also simulated the probability distribution of new flu cases. We performed an analysis to quantify the strength of the intervention effect.

    Results A total of 97 people were diagnosed with flu before the intervention, and 7 were diagnosed after the intervention. A molecular analysis of the flu virus revealed that this outbreak was due to the flu A (H3) virus. A total of 3,702 people received prophylaxis. There was a significant reduction in the reproductive value from 1.89 [95% confidence interval (CI), 1.59 to 2.24] to 0.65 (95% CI, 0.02 to 1.00) after the intervention (p<0.001).

    Conclusion Prophylaxis with anti-flu agents, along with prompt identification and isolation of infected individuals, was effective in reducing the impact of a flu outbreak in a hospital.

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  • Tsuguka Shiwa, Maho Nishimura, Masaya Kato
    2018 Volume 57 Issue 4 Pages 503-506
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    Objective The early diagnosis and treatment of microalbuminuria is important for preventing the progression of diabetic kidney disease in patients with diabetes. In this study, we assessed the accuracy of the semi-quantitative measurement of microalbuminuria by urine dipstick screening in patients with diabetes.

    Methods The semi-quantitative urinary albumin-to-creatinine ratio (QUACR) was used for microalbuminuria screening. A total of 291 diabetes patients with normoalbuminuria [urine albumin-to-creatinine ratio (UACR) <30 mg/g・Cre; n=205] or microalbuminuria (UACR 30-299 mg/g・Cre; n=86) were enrolled as study participants. Both the qualitative test of albumin (QUA) and the QUACR of early-morning or spot urine samples were performed at the same time. A receiver operating characteristic (ROC) analysis was performed to compare the diagnostic utility of the QUACR to that of the QUA in the detection of microalbuminuria.

    Results The sensitivity and specificity values of the QUACR were 84.9% and 76.6%, respectively. Those of the QUA were 53.5% and 84.4%, respectively. In the ROC analysis, the area under the curve values of the QUACR and QUA for the diagnosis of microalbuminuria were 0.807 (95% confidence interval: 0.752-0.863) and 0.689 (0.618-0.760), respectively.

    Conclusion These results suggest that the QUACR is a simple and efficient test-with high levels of sensitivity and specificity-for the detection of microalbuminuria in patients with diabetes.

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CASE REPORTS
  • Kentaro Shimizu, Mitsuru Kageyama, Hiroshi Ogura, Tomoki Yamada, Takes ...
    2018 Volume 57 Issue 4 Pages 507-510
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    Intestinal dysmotility is a major problem in critically ill patients. This report describes the successful treatment of intestinal dysmotility with rhubarb as a laxative in six critically ill patients on mechanical ventilation. Bowel movement and defecation occurred in all patients an average of 1.8 days after the administration of powdered rhubarb. In 4 patients who also had gastric reflux, the reflux volume via nasal tube was decreased an average of 3.5 days after the initiation of rhubarb treatment, and enteral nutrition was able to be started. Rhubarb may be useful for the treatment of incompetent gastric and intestinal motility in critically ill patients.

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  • Satoshi Oka, Ken Umetani, Tomoko Harama, Takuya Shimizu, Aritaka Makin ...
    2018 Volume 57 Issue 4 Pages 511-515
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    A 77-year-old Japanese woman presented with asymptomatic abdominal lymphadenopathy. Soluble interleukin-2 receptor (sIL2R) and angiotensin-converting enzyme (ACE) levels were elevated, and a pathological examination of lymph-node biopsies revealed non-caseating granulomas, which was consistent with sarcoidosis. Fluorodeoxyglucose-positron emission tomography did not show a clear accumulation in the mediastinal lymph-nodes or heart. Five months later, she presented with acute progressive heart failure that was refractory to conventional treatment. Her sIL2R and ACE levels decreased spontaneously over time, without steroid treatment. Autopsy findings revealed non-caseating granulomas. Cardiac sarcoidosis presenting as acute, progressive, treatment-refractory heart failure is rare. Steroid therapy after the resolution of inflammation did not affect the clinical outcome.

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  • Hideyuki Hayashi, Masahiro Kimura, Takao Kato, Kazutaka Nakasone, Yuta ...
    2018 Volume 57 Issue 4 Pages 517-522
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    Recently, it has been reported that spontaneous left ventricular wall thickening occurs among patients with takotsubo syndrome, which affects the long-term prognosis of such patients due to cerebral and cardiac complications. We herein report two cases of transient left ventricular wall thickening with takotsubo syndrome in which sequential cardiac magnetic resonance imaging revealed the existence of edematous changes in the thickened wall. Notably, a left ventricular aneurysm was detected during the course of ventricular wall thickening and may have played a role in the development of serious complications accompanied by takotsubo syndrome. This is the first case report of left ventricular aneurysm occurring with ventricular wall thickening due to takotsubo syndrome.

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  • Takahiro Kanda, Kei Tawarahara, Gaku Matsukura, Masayoshi Matsunari, R ...
    2018 Volume 57 Issue 4 Pages 523-526
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    Spontaneous coronary artery dissection (SCAD) is rare, but it frequently presents as acute myocardial infarction. It is frequently fatal and most cases are diagnosed at autopsy. We herein present the case of a 65-year-old woman with ST-elevation and myocardial infarction due to SCAD. Optical coherence tomography (OCT) helped us to confirm the diagnosis. The information on the intravascular morphology provided by OCT imaging is much more detailed in comparison to that provided by coronary angiography (CAG) and intravascular ultrasound (IVUS).

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  • Nobumasa Ohara, Kazumasa Ohashi, Toshiya Fujisaki, Chiyumi Oda, Yohei ...
    2018 Volume 57 Issue 4 Pages 527-535
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    A 63-year-old Japanese woman with advanced lung adenocarcinoma developed isolated adrenocorticotropin deficiency caused by immune checkpoint inhibitor (ICI)-related hypophysitis following 8 months of nivolumab therapy. Prompt corticosteroid replacement therapy effectively relieved her secondary adrenal insufficiency symptoms and allowed her to pursue nivolumab therapy, which had been effective for the control of lung adenocarcinoma. Human leukocyte antigen (HLA) typing revealed the presence of the DRB1*04:05-DQA1*03:03-DQB1*04:01 haplotype, which is associated with susceptibility to autoimmune polyglandular syndrome with pituitary disorder in the Japanese population. This case suggests that genetic factors, such as HLA, contribute to the development of endocrinopathies induced by ICIs.

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  • Rieko Nakatani, Takashi Murata, Takeshi Usui, Koki Moriyoshi, Toshiki ...
    2018 Volume 57 Issue 4 Pages 537-543
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    Glycated hemoglobin (HbA1c) is a widely used marker of glycemic control but can be affected by hemolytic anemia. Glycated albumin (GA) is also affected in patients with liver cirrhosis. We herein report the assessment of glycemic control in a 41-year-old man with dehydrated hereditary stomatocytosis and a PIEZO1 gene mutation complicated by diabetes mellitus and liver cirrhosis due to hemochromatosis. The estimated HbA1c calculated from the average glucose level obtained by continuous glucose monitoring or by self-monitoring of blood glucose was useful for evaluating the glycemic control in this patient, as HbA1c and GA were unreliable due to the coexisting conditions.

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  • Erina Yuasa-Shibasaki, Sumiyasu Ishii, Shunichi Matsumoto, Takuya Toma ...
    2018 Volume 57 Issue 4 Pages 545-549
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    A 39-year-old woman was admitted to our hospital with symptoms of general fatigue, nausea, and vomiting that appeared three months after she stopped seven years of medroxyprogesterone acetate (MPA) medication for endometrial stromal sarcoma. Laboratory tests demonstrated moderate hypercalcemia. Several tests demonstrated that she was suffering from adrenal insufficiency. Glucocorticoid supplementation decreased her calcium level to a normal range, indicating that hypercalcemia was induced by adrenal insufficiency. It was suggested that she was suffering from MPA-induced adrenal insufficiency, but hypocortisolemia was being compensated by a high dose of MPA; hypocortisolemia and hypercalcemia then became evident after MPA treatment was discontinued.

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  • Kazuyoshi Toda, Sumiyasu Ishii, Hidetoshi Yasuoka, Masaki Nishioka, Ta ...
    2018 Volume 57 Issue 4 Pages 551-555
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    Ewing's sarcoma usually arises in the bones of children and adolescents. We herein report a 74-year-old man with Ewing's sarcoma in the adrenal gland. The diagnosis was confirmed by a genetic test, pathological studies, and several imaging studies. He already had multiple liver metastases when he was transferred to our hospital and died on the 37th day. The diagnosis was further confirmed by autopsy studies. Adrenal Ewing's sarcoma is very rare, and our patient was older than other reported cases. Ewing's sarcoma should be considered even in elderly patients with adrenal tumors.

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  • Chihiro Sato, Kenichi Okuda, Hiroyuki Tamiya, Kota Yamamoto, Katsuyuki ...
    2018 Volume 57 Issue 4 Pages 557-561
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    A malignant tumor can cause hypercoagulation and it also often coexists with thrombosis. Cisplatin-based chemotherapy can also induce adverse vascular effects, including arterial thrombosis. We herein report a case of acute arterial thrombosis in a patient undergoing postoperative adjuvant cisplatin-based chemotherapy for completely resected lung cancer. The patient complained of acute leg pain after chemotherapy, and computed tomography revealed multiple thrombi from the thoracic to popliteal arteries. Arterial thrombosis during adjuvant chemotherapy is extremely rare; however, careful clinical observation of patients receiving cisplatin-based chemotherapy is important, because arterial thrombosis, even in the absence of the primary malignant tumor, is possible.

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  • Kenji Tokunaga, Ayako Yamamura, Shikiko Ueno, Yoshitaka Kikukawa, Shun ...
    2018 Volume 57 Issue 4 Pages 563-568
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    No valid treatment for isolated myeloid sarcoma (IMS) has yet been established, and no thorough genetic examinations have been performed because of its low incidence and unique manner of development. We herein report a 34-year-old man with pancreatic IMS with t(8;21)/RUNX1-RUNX1T1 rearrangement. He was treated with high-dose cytarabine followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT). This is the first report of pancreatic IMS with t(8;21). Positron emission tomography/computed tomography and genetic study are useful for the diagnosis, and allo-HSCT achieved complete remission in this patient.

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  • Yoshitaka Adachi, Yohei Yamaguchi, Ken Sagou, Yusuke Yamaga, Nobuaki F ...
    2018 Volume 57 Issue 4 Pages 569-574
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    A 64-year-old man with acute myeloid leukemia underwent umbilical cord blood transplantation (UCBT). After 11 months of complete remission (CR) following UCBT, the bone marrow showed 7.5% myeloblasts. CR was obtained after a single course of azacitidine monotherapy, but the myeloblasts gradually increased in the blood. We made a diagnosis of acute megakaryoblastic leukemia derived from donor cell with a fluorescence in situ hybridization (FISH) analysis of the sex chromosomes and an immunophenotypic analysis. Azacitidine was administered again and produced a therapeutic effect of stable disease. This case suggests that azacitidine may be a useful therapy for patients with acute megakaryoblastic leukemia in situations in which intensive chemotherapy and transplantation are not indicated.

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  • Sachiko Furukawa, Kazunari Oobu, Masafumi Moriyama, Shintaro Kawano, S ...
    2018 Volume 57 Issue 4 Pages 575-581
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    Long-term methotrexate (MTX) treatment can cause MTX-related lymphoproliferative disorder (MTX-LPD). We experienced a case of MTX-LPD that was associated with severe osteonecrosis of the jaw mimicking medication-related osteonecrosis of the jaw. The patient was an 81-year-old woman with rheumatoid arthritis (RA) who was treated with MTX and bisphosphonate. After 7 years, she was referred to our department for the assessment of giant ulcer and exposure of the alveolar bone of the left maxilla. Histopathological and immunological analyses confirmed a diagnosis of MTX-LPD. At seven months after the cessation of MTX treatment, the ulcerative and necrotic lesions had markedly decreased in size. A 1-year follow-up examination showed no evidence of recurrence and good RA control.

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  • Kensuke Matsuda, Kazuhiro Toyama, Takashi Toya, Masako Ikemura, Fumihi ...
    2018 Volume 57 Issue 4 Pages 583-586
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    A 16-year-old boy with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) underwent allogeneic hematopoietic stem cell transplantation after conditioning with fludarabine, melphalan, total body irradiation, and rabbit antithymocyte globulin (ATG). A severe, persistent infusion reaction occurred after the initial administration of ATG. Investigations showed a rapid increase in the levels of liver enzymes and ferritin, and the reactivation of HLH was confirmed by marked hemophagocytosis in the bone marrow. Treatment with pulse glucocorticoid therapy resulted in the improvement of HLH. This is the first case of HLH reactivation triggered by ATG. Physicians should therefore be cautious of HLH reactivation, especially when a severe and prolonged infusion reaction occurs.

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  • Junji Takasugi, Munehisa Shimamura, Toru Koda, Toshihiro Kishikawa, At ...
    2018 Volume 57 Issue 4 Pages 587-590
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 01, 2017
    JOURNALS OPEN ACCESS

    Paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS) are usually associated with small-cell lung carcinoma (SCLC). PCD-LEMS with extrapulmonary non-SCLC tumors; however, has not been previously reported. A 78-year-old man presented with dysarthria, dysphagia, staggering gait, and lower extremity muscle fatigue. He was diagnosed with PCD-LEMS associated with neuroendocrine carcinoma of the oropharynx, based on the histological findings of the biopsy, the existence of antibodies against P/Q-type voltage-gated calcium channels, and an incremental response of the compound muscle action potentials during repetitive nerve stimulation tests. Thus, PCD-LEMS should be included in the differential diagnosis of neurological dysfunction, even in extrapulmonary non-SCLC patients.

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  • Hisashi Takahashi, Taiki Ueshima, Daiki Goto, Tadashi Kimura, Natsuko ...
    2018 Volume 57 Issue 4 Pages 591-594
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    A 63-year-old man developed vomiting, paraparesis, dysuria, bulbar palsy, and orthostatic hypotension over a period of 5 months. Neuroradiological examinations showed a swollen lower brainstem with a dural arteriovenous fistula at the craniocervical junction (DAVF-CCJ). A steroid was administered intravenously in the hospital to relieve brainstem edema. A few hours later, however, the patient developed acute tetraparesis with respiratory failure. Recently, there have been several reports describing the acute worsening of paraparesis in patients with a spinal dural arteriovenous fistula after steroid treatment. In addition to these reports, the present case suggests the risk of administering steroids to patients with DAVF-CCJ, especially those with brainstem dysfunction.

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  • Satoko Oka, Kazuo Ono, Masaharu Nohgawa
    2018 Volume 57 Issue 4 Pages 595-600
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    Sweet disease may occur in several organs, and central nervous system involvement, known as Neuro-Sweet disease (NSD), is rare. The clinical features of NSD include recurrent encephalomeningitis accompanied by fever and erythematous plaques; systemic corticosteroid therapy is highly effective. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is an important electrolyte abnormality because it can be life-threatening. We describe the first case of SIADH and NSD associated with low-risk myelodysplastic syndrome that was successfully treated with corticosteroids and cyclosporine. The patient has remained stable for 1 year without any recurrence.

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  • Masahiro Ohara, Kokoro Ozaki, Takuya Ohkubo, Akane Yamada, Yoshiyuki N ...
    2018 Volume 57 Issue 4 Pages 601-604
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    Myasthenia gravis (MG), a neuromuscular junction autoimmune disease, sometimes complicates second malignancies; however, T-cell lymphoproliferative disorders have rarely been reported. A 55-year-old man, who received oral tacrolimus and prednisolone for MG for 16 years after thymectomy, presented with left abdominal pain, lymphadenopathy, and splenomegaly. A lymph node biopsy revealed peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). This is the first report of oral tacrolimus leading to a T-cell lymphoproliferative disorder in patient without a history of transplantation. Physicians should be aware of the possibility of rare T-cell lymphoproliferative disorders, such as PTCL-NOS, occurring as complications in MG patients on immunosuppressive regimens after thymectomy.

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  • Hiroshi Nishida, Yuichi Hayashi, Naoko Harada, Takeo Sakurai, Kenji Wa ...
    2018 Volume 57 Issue 4 Pages 605-611
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    A 61-year-old woman was admitted to our hospital due to memory difficulties, visual hallucinations, and slowly progressing motor difficulties in the limbs. A clinical examination revealed bradykinesia, gait disturbance, left-side-dominant rigidity, ideomotor apraxia, dressing apraxia, left-sided spatial agnosia, impaired visuospatial ability, and executive dysfunction. Her symptoms were unresponsive to levodopa, and corticobasal syndrome (CBS) was diagnosed. One year later, amyloid positron emission tomography revealed amyloid beta accumulation in the bilateral cerebral cortices; at this point, CBS with underlying Alzheimer's disease pathology (CBS-AD) was diagnosed. Visual hallucinations may help differentiate CBS with corticobasal degeneration (CBS-CBD) from other pathologies, including CBS-AD.

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  • Kazushi Minami, Shinichi Takahashi, Yoshihiro Nihei, Koichi Oki, Shige ...
    2018 Volume 57 Issue 4 Pages 613-615
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 21, 2017
    JOURNALS OPEN ACCESS

    Seipinopathy is an autosomal dominant neurodegenerative disease caused by mutations of the Berardinelli-Seip Congenital Lipodystrophy 2 (BSCL2) gene. We report the first Japanese case of seipinopathy with a heterozygous mutation of p.N88S in the BSCL2 gene. The patient showed bilateral hyperreflexia of the biceps, triceps, brachioradialis, and knee, as well as the pes cavus and distal dominant weakness and atrophy of both arms and legs, suggesting the involvement of both upper and lower motor neurons. Mutations of the BSCL2 gene have been known to cause motor neuron degeneration through endoplasmic reticulum stress. Seipinopathy should be considered in patients with symptoms mimicking amyotrophic lateral sclerosis.

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  • Yoji Komiya, Makoto Soejima, Daisuke Tezuka, Hitoshi Kohsaka
    2018 Volume 57 Issue 4 Pages 617-622
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: November 20, 2017
    JOURNALS OPEN ACCESS

    A 59-year-old man with swollen submandibular glands developed an aortic aneurysm requiring aortic prosthesis implantation. Echocardiography performed to evaluate the cardiac function before the surgery incidentally revealed masses around the coronary arteries. The serum IgG4 levels were increased. A post-operational pathological examination of the abdominal aneurysms revealed infiltration of plasma cells, with the ratio of IgG4/IgG-positive cells being >80%. The patient was diagnosed with IgG4-related disease (RD) with coronary artery involvement. He was treated successfully with corticosteroid before any associated cardiovascular events occurred. Given the poor prognosis of IgG4-RD-associated coronary artery involvement, this case emphasizes the importance of the early assessment with echocardiography, even if patients have no cardiovascular symptoms.

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  • Shoko Mizoguchi, Toshiki Sawai, Atsuya Hirota, Shigetada Yamamoto, Hir ...
    2018 Volume 57 Issue 4 Pages 623-626
    Published: February 15, 2018
    Released: February 15, 2018
    [Advance publication] Released: December 08, 2017
    JOURNALS OPEN ACCESS

    A 66-year-old man, who had been diagnosed with deep venous thrombosis (DVT), and who was treated with a vitamin K antagonist (VKA) and who had undergone the implantation of an inferior vena cava filter, was admitted due to an exacerbation of DVT. VKA was administered again; however, the patient's DVT worsened. Further examinations revealed colon cancer, which led to a diagnosis of Trousseau's syndrome. The regression of the thrombi was confirmed after the administration of heparin and the resection of the tumors. Trousseau's syndrome should always be kept in mind when patients present with refractory venous thrombosis. The administration of heparin, and cancer control are necessary for the effective treatment of thrombosis in such cases.

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