Myotonia is repetitive firing of muscle action potentials causing prolonged muscle contractions even after mechanical stimulations to the muscles have ceased. Most common myotonic disorder is myotonic dystrophy which is now termed DM1, myotonic dystrophy type 1. In Japan, proximal myotonic myopathy, which is now called DM2 has not been reported. Both DM1 and DM2 have Cl channel abnormality which causes myotonia. Less commonly we encounter Thomsen’s disease, and autosomal recessive generalized myotonia (Becker type) which also have a Cl channel abnormality. There are other myotonic disorders related to Na channelopathy which include three disorders: paramyotonia congenita, adynamia episodica hereditaria, and myotonia fluctuans. Myotonia has been treated by various Na channel blockers, mexiletine, phenytoin, and carbamazepine, but they were originally developed for cardiac arrhythmia, or seizure disorders and they have undesirable side effects, weakness. Comprehensive treatment includes myotonia control without reducing the strength, and care for systemic manifestations of DM1.
Objective In recent years, the number of elderly patients with early gastric cancer (EGC) has steadily been increasing. In our institute, endoscopic submucosal dissection (ESD) involving the use of an insulated-tip diathermic knife (IT-ESD) was introduced for the treatment of mucosal gastric carcinoma in 1996. The purpose of this study was to evaluate the effectiveness of IT-ESD for the treatment of elderly patients with EGC. Materials and Methods A total of 144 patients with EGC were treated at Shikoku Cancer Center in the 5-year period from January 2000 to December 2004, including 53 patients over 75 years old. The performance status (PS) for all patients was less than 2. We compared patient’s backgrounds, the one-piece resection rate, complete resection (CR) rate, operation time, bleeding rate, perforation rate, blood pressure, and peripheral oxygen saturation (SpO2) between patients over 75 years of age (elderly group) and the remaining 91 younger patients (non-elderly group). Results The rate of having underlying disease was significantly higher for the elderly group (p<0.05) and we found no significant difference for the one-piece resection rate, CR rate, operation time, bleeding rate, and perforation rate between the 2 groups. There were also no significant differences for the frequency of the use of oxygen, pressor and depressor between the 2 groups. Conclusion There was no significant difference in the outcome resulting from ESD between the 2 groups. Our study proves that ESD is a feasible treatment for elderly patients with EGC PS of less than 2.
Objective Although heart-type fatty acid-binding protein (H-FABP) is a cardiac marker useful for early diagnosis of acute myocardial infarction (AMI), few data are available on its prognostic value. The objective of this study is to clarify the prognostic value of H-FABP in patients with a serious condition. Methods and Patients We conducted a prospective study of 617 patients who presented to the emergency department with a serious condition. The H-FABP levels on arrival at the emergency department were divided into four groups using their quartiles. The endpoint was death from any causes in-hospital. Results H-FABP ranged from 1.2 to 2,300 ng/ml, with a median of 19.9 ng/ml, a 25%-value of 6.7 ng/ml and 75%-value of 54.0 ng/ml. The unadjusted rate of the mortality increased progressively with increasing H-FABP quartile point (11% for quartile-I, 22% for quartile-II, 36% for quartile-III, and 38% for quartile-IV; p<0.001). After adjustment for age, gender, systolic blood pressure and the presence or absence of cardiovascular disease, H-FABP was the independent factor to predict the mortality. Conclusion H-FABP has proven to be an independent factor for prognosis in patients with a serious condition on arrival at the emergency department.
Background Intellectual deterioration occurs in 10-40% of patients with Parkinson’s disease. However, there are many conflicting studies on its relation with brain perfusion and the nature of this dementing process remains controversial. Objective To compare cortical perfusion by SPECT using 123I-IMP between Parkinson’s disease patients with dementia and those without dementia and to investigate the correlation between dementia in Parkinson’s disease and brain perfusion in various areas. Methods Fifty-two cases of Parkinson’s disease and 10 control cases were studied. The Parkinson’s disease with dementia group included 30 cases and the Parkinson’s disease without dementia group included 22 cases. ,Results By multiple logistic regression method, we demonstrated significant hypoperfusion in the occipital cortex in Parkinson’s disease with dementia. Conclusions The cause of dementia in Parkinson’s disease may vary. We demonstrated that occipital hypoperfusion was closely correlated to dementia in Parkinson’s disease compared to frontal, parietal and temporal perfusion.
A 54-year-old woman was admitted to our hospital because of acute liver injury. Since she had a history of having used a diet product, drug-induced liver injury (DILI) was initially considered. However, the patient was subsequently diagnosed as suffering from primary biliary cirrhosis (PBC) based on the findings of liver histology and serum anti-mitochondrial antibody positivity. Overlap syndrome between PBC and autoimmune hepatitis (AIH) was also suspected, however, serum levels of aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase became normal three months later, after treatment with combination therapy comprising ursodeoxycholic acid plus bezafibrate. We therefore concluded that the liver disease in this patient was actually PBC, but that it resembled overlap syndrome or DILI. In cases of PBC, a rapid onset, as frequently seen in the case of DILI, viral hepatitis or AIH, is not common. We herein report a rare case of PBC which resembled DILI.
We describe a case of infective endocarditis caused by Campylobacter fetus accompanied by pulmonary emboli. A 52-year-old man was referred to our hospital due to febrile temperatures with a history of dental treatment followed by eating raw meat. Computed tomography revealed multiple infiltrations and a nodule with low attenuation area and feeding vessels. A mobile mass, possible vegetation, attached to the tricuspid valve was detected by transthoracic echocardiography. Two blood cultures disclosed Campylobacter fetus. Long-term antibiotic therapy was given, curing the infection with valvuloplasty. We presented the possibility that infective Campylobacter fetus endocarditis after dental treatment was caused by eating raw meat.
We present a 65-year-old man with rheumatic combined valvular heart disease showing persistent fever 3 weeks after diagnostic cardiac catheterization. Infective endocarditis was strongly suspected from the clinical course, however, serial blood cultures were negative. Transesophageal echocardiography, done to investigate vegetation, revealed multiple mobile plaques in the descending aorta. Administration of both steroid and simvastatin improved both symptoms and renal function. Cholesterol embolism should be considered to be one of the possible causes of low-grade fever after cardiac catheterization especially in patients with anticoagulation.
A hyperthyroid patient with bloody pericardial effusion is presented. He was hospitalized for severe dyspnea. Pericardiocentesis yielded 1.2 liters of bloody fluid. Biochemical, cytologic, and radiologic examinations failed to identify the etiology of the effusion. Upon normalization of thyroid function using antithyroid drugs, the pericardial effusion resolved without recurrence. The patient was diagnosed as Graves’ disease, which rarely is complicated by bloody pericardial effusion. As it is rarely reported and not widely known, this association may be underdiagnosed.
A 34-year-old Japanese man diagnosed as having cat-eye syndrome (CES) with isolated idiopathic hypogonadotropic hypogonadism (IHH) was treated at our university. He showed preauricular pits/tags, downward slanting palpebral fissures, ocular hypertelorism, and strabismus. However, ocular coloboma and anal atresia, major characteristic features of CES, were negative. Chromosomal analysis revealed malformation in chromosome 22 and eunuchoid features and a low grade development of secondary sexual characteristics were also evident. Endocrinological examinations revealed that this patient was in a state of isolated IHH. Although CES with IHH is extremely rare, endocrine disorders should be given due attention.
A 45-year-old woman was admitted to our hospital because of high fever and malaise. She also presented abnormal urine findings including macrohematuria and proteinuria with transient renal insufficiency. Salmonella Typhi was isolated from her blood and stool culture, and then she was diagnosed as having typhoid fever. Salmonella enterica serotype typhi infection can be complicated by renal involvement, although rare. In Japan, few cases have been reported about acute nephritic syndrome in typhoid fever. Here, we report a case of endemic acquired typhoid fever associated with acute renal failure probably due to acute nephritic syndrome successfully treated with levofloxacin.
An 88-year-old man with a 30-year history of type 2 diabetes and a 3-year history of chronic renal failure was admitted for evaluation of anasarca. On admission, findings of nephrotic syndrome and microscopic hematuria were observed. During the course of therapy, rapid deterioration of renal function occurred with the appearance of pneumonia. Irrespective of the therapy with hemodialysis and antibiotics, he died of respiratory failure. The autopsy showed a rare case of rapidly progressive glomerulonephritis (crescentic glomerulonephritis) superimposed on membranous nephropathy. This experience highlighted the importance of the differential diagnosis of non-diabetic glomerulopathy even in elderly patients with diabetes mellitus.
A 50-year-old woman was transferred to our hospital because of acute renal failure and thrombocytopenia. Due to rapid enlargement of the kidney, we first suspected that she had diffuse renal invasion of anaplastic carcinoma or lymphoma of the kidney. Anti-bacterial treatment for complicated urinary tract infection and hemodialysis treatment resulted in recovery of both renal function and thrombocytopenia. Serial CT study demonstrated disappearance of kidney swelling and multiple masses within the kidney. We finally made a diagnosis of acute multifocal bacterial nephritis. Timely initiation of dialysis therapy and appropriate anti-bacterial treatment was essential to rescue this case.
A 50-year-old man with chronic otitis media was admitted to our hospital for investigation of lung cancer. He had received repeated instillations of fibrin sealant (Bolheal) in myringoplasty 7 times over 12 months. During the lower lobectomy, soon after fibrin sealant was applied to seal bronchopleural fistulas, systolic blood pressure decreased to 60 mmHg. Treatment with epinephrine and dopamine was required until his condition improved 2 days later. The specific IgE antibody was detected for the solution for fibrinogen and factor XIII containing bovine aprotinin. Cross-reactivity between purified aprotinin and the solution was noted by competitive ELISA inhibition tests.
We report a case of severe iron deficiency anemia with marked thrombocytosis that was complicated by central retinal vein occlusion. Platelet count was over one million per microliter and an increased number of megakaryocytes was observed in the bone marrow at the time of diagnosis of iron deficiency anemia, features that resemble those of essential thrombocythemia. However, the platelet count rapidly declined with the administration of ferrous fumarate. Accordingly, central retinal vein occlusion was improved and has not recurred. In this case, significant thrombocytosis caused by iron deficiency anemia may have been involved in the development of central retinal vein occlusion.
An 80-year-old man presented to the internist with fever, fatigue and leukocytosis up to 66.8×103/μl. Although a chronic myelogenous leukemia was initially suspected, he was diagnosed as metastatic bone marrow tumor with bone marrow necrosis from primary prostate cancer on the basis of the clinical and pathological findings. The serum concentrations of IL-6 and TNF-α were mildly elevated to 65.0 pg/ml and, 54.0 pg/ml respectively. It is probable that these humoral factors were partially responsible for the leukemoid reaction although other factors induced by the bone marrow necrosis with bone marrow metastasis of prostate cancer are also likely involved.
Hypersensitivity to mosquito bites (HMB) is known to be an allergic reaction and also a skin symptom in some cases of natural killer cell leukemia/lymphoma associated with Epstein-Barr virus (EBV) infection. We describe a patient who had suffered from HMB for 5 years, and subsequently developed mantle cell lymphoma (MCL), which resembled chronic lymphocytic leukemia in the early phase. EBV monoclonality was not detected in lymph nodes by Southern blotting. Serum levels of interleukin-4 and IgE were increased, but they decreased and HMB disappeared when MCL was predominant. We consider that HMB may be a potential sign of MCL.
Septic embolisms are rare disorders which are associated with increased mortality and morbidity. We describe a rare case of septic intramuscular embolism accompanied by asymptomatic pulmonary embolism in a neutropenic patient. Methicillin-sensitive Staphylococcus aureus (S. aureus) was detected and multiple nodules were revealed in both thighs and lung. Although he was treated with sensitive antibiotics to .S. aureus, the symptoms remained unchanged during the neutropenic period. Fever subsided rapidly and his thigh pain disappeared after neutropenia resolved. A prompt diagnosis and optimal therapeutic decisions are critical for the reduction of mortality.