Objective To elucidate the mechanism of the reported association between serum γ-glutamyl transferase (GGT) activity and cardiovascular mortality.
Methods Cross-sectional analysis of the relationship between serum GGT activity and the risk factors for cardiovascular disease was performed.
Patients and materials Middle-aged Japanese male personnel of the Self-Defense Forces who underwent retirement check-up.
Results Serum GGT activity was associated with total cholesterol, triglyceride, fasting plasma glucose, total homocysteine and systolic blood pressure. The association remained in the analysis adjusted for possible confounders including cigarette smoking, ethanol consumption and body mass index.
Conclusion The observed association between serum GGT and cardiovascular risk factors may partly explain the reported relationship between serum GGT activity and cardiovascular disease. Serum GGT activity may be regarded as a marker of cardiovascular risk factors or oxidative stress rather than a mere indicator of excessive ethanol consumption or obesity.

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Objective To investigate the association between C-reactive protein (CRP) and insulin resistance.
Materials and Methods This study included 1,624 Japanese participants (652 men and 972 women) aged 40 to 69 years who were non-diabetics or did not have medication for hypertension or dyslipidemia, a history of cardiovascular disease or CRP levels >10 mg/l. Serum CRP level, fasting glucose level, and fasting insulin level were measured, and the degree of insulin resistance was assessed using the homeostasis model assessment of insulin resistance (HOMA-IR). Categories of CRP were defined by the following tertiles: <0.25 mg/l, 0.25-0.59 mg/l, and ≥0.60 mg/l.
Results Elevated CRP levels were associated with increased fasting insulin levels, fasting glucose levels, and HOMA-IR in both men and women. Although the adjustment for body mass index in addition to age, cigarette smoking, and alcohol consumption attenuated the associations between CRP and fasting insulin, fasting glucose, and HOMA-IR, elevated CRP levels were associated with increased insulin levels and HOMA-IR in both sexes. Stratified analyses by CRP level and obesity showed that obesity status was associated with increased fasting insulin levels, fasting glucose levels, and HOMA-IR in both sexes and that fasting insulin levels, fasting glucose levels, and HOMA-IR were higher among obese individuals than among non-obese individuals at the same level of CRP.
Conclusion These results suggest a possible role of subclinical inflammation in insulin resistance and glucose intolerance in Japanese, but it only partly explains the link between obesity and impaired glucose homeostasis.

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Objective Proteinuria is a recognized complication of obesity, but the pathogenesis remains unclear. We undertook the present study to clarify the factors contributing to proteinuria associated with obesity.
Methods We studied 12 obese patients with proteinuria. Twenty-seven age-matched obese subjects without proteinuria served as controls. A glucose tolerance test and renal biopsy were performed in all patients. Fasting serum insulin and homeostasis model assessment-insulin resistance (HOMA-IR) were regarded as reflecting insulin resistance. To delineate the relation between insulin resistance and proteinuria, troglitazone, which acts an insulin sensitizer was given to 6 of 12 patients with a regular diet for 8 weeks. The 6 others were observed without receiving troglitazone.
Results The 12 patients showed the presence of a cluster of insulin resistance factors: higher blood pressure, higher body mass index, higher fasting plasma glucose, higher fasting serum insulin, and higher HOMA-IR than controls. The renal biopsy specimens exhibited no histological abnormalities in 7, focal segmental glomerulosclerosis in 3 and benign nephrosclerosis in 2. Troglitazone attenuated HOMA-IR and ameliorated proteinuria, but did not affect body weight, creatinine clearance, or blood pressure. In contrast, the parameters in the patients not given troglitazone did not change.
Conclusion Insulin resistance is a factor contributing to obesity-related proteinuria. The role of insulin resistance as a factor reducing proteinuria remains to be clarified.

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Objective Copper deficiency has been reported to cause hematological disorders. However, its clinical and hematological characteristics are not fully understood. Therefore, we investigated bedridden patients suffering from copper deficiency and tried to clarify the clinical features of hematological disorders caused by this condition.
Patients and Methods Five patients with typical copper deficiency who had been dependent upon enteral nutrition for a long period of time due to various diseases were investigated. We measured hematological parameters and observed the response to copper supplementation therapy and the recovery process of hematological disorders.
Results Their mean age was 82.6±10.4 years and the mean duration of enteral nutrition was 16.4±5.2 months. Their serum copper concentration was extremely decreased (range, 3 to 8 μg/dl). All five patients had anemia and neutropenia. On the other hand, platelet count remained within the normal range. After copper supplementation therapy, hemoglobin concentration increased from 6.8±0.7 g/dl to 9.9±0.7 g/dl within a few months (p<0.01). Neutrophil count also increased from 750±370/μl to 3,690±1,210/μl in a few weeks (p<0.01). Mean corpuscular volume (MCV) decreased from 94.3±7.3 fl to 86.0±4.8 fl (p<0.05). Elevated serum ferritin and erythropoietin (EPO) levels were normalized after the improvement of anemia.
Conclusion Bicytopenia (anemia and neutropenia) with normal platelet count is a feature of hematological disorders caused by copper deficiency. MCV tends to indicate macrocytic anemia. Serum ferritin and EPO levels are elevated. These hematological abnormalities are improved within a few months after copper supplementation therapy.

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Objective The aim of this study was to analyze the clinical and laboratory features of each subtype of multiple sclerosis (MS) (relapsing-remitting, primary progressive, and secondary progressive) in the Tokyo metropolitan area.
Methods and Patients We retrospectively analyzed the medical records of 104 consecutive patients with a diagnosis of MS, who had been admitted to our university hospital from 1988 to 2002. They all met criteria for definite MS, by clinical or laboratory standards.
Results Eighty-four (80.8%) patients were classified as having relapsing-remitting MS, while 8 patients (7.7%) and 12 patients (11.5%) were classified as having primary progressive MS and secondary progressive MS, respectively. A significant female predominance existed in the relapse-remitting MS (female : male=2.4 : 1) cohort, but this ratio was 1 : 1 in both primary progressive and secondary progressive MS. The age at onset was older in the primary progressive MS (36.6±17.1 years of old) population than in either the relapsing-remitting MS (27.9±11.1) or the secondary progressive MS (27.8±11.5) subjects. Although the duration of illness was similar among the three types of MS, the number of exacerbations in the secondary progressive (5.9±4.6) cohort was significantly higher than that in the relapsing-remitting MS subjects (3.2±2.6). Patients with primary progressive MS showed a significantly higher rate of gait disturbance (87.5%) as the initial symptom than those with relapsing-remitting MS (23.8%), and this was thought to be due to the higher incidence of brainstem and spinal cord lesions. Visual disturbance as the initial symptom was frequently noted in those with secondary progressive MS (50.0%), while it was noted only in 29.8% and 12.5% in the relapsing-remitting and primary progressive patients, respectively. Primary progressive MS subjects had a higher propensity to be wheelchair-bound (75.0%) than those suffering from relapsing-remitting MS (1.2%). Increased total protein in the cerebrospinal fluid (CSF) of the secondary progressive cohort was statistically significant compared to the relapsing-remitting cohort. The frequency of oligoclonal IgG bands was rather low in each type of MS (17.1-33.3%). Gadolinium enhancement of plaques on MRI was more frequently present in secondary progressive MS (66.7%) than in either relapsing-remitting MS (32.1%) or primary progressive MS (50.0%). Of note, the opticospinal form was found in only 16.3% of the total MS patients, a proportion less than that in previous reports from southern Japan.
Conclusion The present study confirms that while the clinical and laboratory features of the MS patients in the Tokyo metropolitan area are similar to those in Western countries in most regards, features such as proportionally fewer primary and secondary progressive MS patients as well as less oligoclonal IgG bands on CSF analysis are different from those in Western countries.

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Background and Purpose Transoral carotid ultrasonography (TOCU) has enabled the assessment of the distal portion of the extracranial internal carotid artery (ICA). We evaluated the ultrasonographic features of ICA occlusion using TOCU.
Methods We studied 50 occluded ICAs in 42 stroke patients. The mechanism of ICA occlusion was embolic (group E) in 14 arteries and thrombotic (group T) in the other 36 arteries. We used a color flow imaging system equipped with special convex array transducers, and placed the probe on the postero-lateral pharyngeal wall to identify the distal extracranial ICA. We evaluated intraluminal echodensity (lucent or opaque) and measured the diameter of the ICA. Then, we examined the relationship of these early (<1 week after onset) and chronic (>4 weeks after onset) phase TOCU findings to the mechanism of ICA occlusion and the site of occlusion.
Results In the early phase of a stroke, the intraluminal echodensity was more frequently lucent (9/11, 81.8%) in group E than in group T (5/20, 20%, p<0.05). In the chronic phase, echodensity became opaque in both groups. In the early phase, the lucent echodensity was more frequently seen in patients with distal occlusion than in those with proximal occlusion. Thus, it may represent blood or fresh thrombus formation. In patients with unilateral ICA occlusion, the occluded ICA was significantly smaller in diameter than the non-occluded contralateral artery both in the early and chronic phases.
Conclusion The echodensity and diameter of the extracranial ICA distal portion as found on TOCU can help to identify the mechanism of ICA occlusion.

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Objectives In European countries and the United States the incidence of elderly-onset myasthenia gravis (MG) has recently been increasing. To investigate whether the incidence of the elderly-onset MG has increased in Nagano Prefecture of Japan, we divided the patients into young and elderly groups, and retrospectively examined their incidence.
Patients and Methods On the basis of two-step questionnaires sent to hospitals and the patient list of the intractable disease registration system in Nagano Prefecture we studied 213 MG patients diagnosed between 1982 and 2001. This 20-year period was divided into 4 five-year terms, and the incidence of MG in young- (younger than 65) and elderly-onset (65 or older) groups was investigated separately for each term.
Results The ratio of the elderly-onset group showed a significantly positive correlation with terms irrespective of associated thymoma (r=0.98, p<0.05). There was a significant difference in the mean onset age among the 4 terms (p<0.005). The standardized incidence of MG gradually increased in both young- and elderly-onset groups as well as in the whole age range. The elderly group showed a particularly high incidence in females (12.01/million/year) and in patients without thymoma (8.78/million/year) in the final five years.
Conclusions We confirmed that the incidence of elderly-onset MG has recently been increasing in the Nagano Prefecture. Since the change of the age distribution in this district is almost identical to that of the whole country, the incidence of MG might have been increasing in Japan as a whole, particularly in the elderly population.

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We report a case of a 58-year-old man with Shoshin beriberi who demonstrated ST-segment elevation and myocardial damage without coronary artery stenosis. The patient subsequently recovered with thiamine treatment. We conclude that it is important to consider Shoshin beriberi as part of the differential diagnosis in patients with shock and ST-segment elevation.

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A 52-year-old man without a history of asthma or allergic diseases died of ventricular fibrillation early in the morning. His autopsy revealed no significant findings, except for a mild mural-thickening localized at the proximal region of the right coronary artery. Microscopic examination showed periarteritis with infiltration of numerous eosinophils in the adventitia. No significant vasculitis was found in any other organs. Based on the findings this seems to be the second reported case of isolated eosinophilic coronary periarteritis.

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An 86-year-old nondiabetic woman with an episode of transient ischemic attack two days earlier was referred to our hospital. She had a history of neurogenic bladder and chronic atrial fibrillation and had been anuric for two days. Bubbles were detected by echocardiography in the right atrium, right ventricle, and inferior vena cava. Computed tomography revealed gas accumulation in the wall and lumen of the bladder. She recovered after urinary drainage and antibiotic therapy, and bubbles were no longer detected. It was suspected that bacterial injury of the bladder wall and high intravesical pressure led gas to enter the venous system.

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We encountered complications of septic shock and retroperitoneal hemorrhage which occurred after implantation of a retrievable Günther Tulip filter. A 79-year-old woman was diagnosed as right femoral vein thrombosis, and a retrievable Günther Tulip filter was deployed prior to a total knee replacement surgery. However, the patient developed septicemia due to Citrobacter freundii, followed by thrombocytopenia and retroperitoneal hemorrhage. A large hematoma was formed along the lateral side of the filter struts. We considered that the filter should be removed to control retroperitoneal hemorrhage and prevent secondary infection of the hematoma. Removal of the retrievable filter was effective in managing these complications.

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A 49-year-old man presented with fever and uremic symptoms such as general malaise, leg edema and decreased urine output. He was diagnosed as having infective endocarditis (IE) accompanied by renal failure. Although he had been receiving hemodialysis for a long time, renal function dramatically improved after heart valve replacement. This case suggests that uremia can develop as an initial manifestation of IE and removal of an infected heart valve can improve renal function despite persistent renal failure. From the perspective of recovery of renal function, early surgery should be considered in patients with renal failure following IE.

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Physical or mental exertion is an important antecedent to dissection. A fall is one of the causes of hip fracture in the elderly population. We report the case of a 78-year-old woman who was diagnosed to have a thrombosed aortic dissection after a fall. We wish to emphasize with this case report that aortic dissection should be considered in the differential diagnosis of patients who complain of chest discomfort after a tumbling over.

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Familial cardiac myxoma is inherited as an autosomal dominant syndrome. Here, we report a Japanese case of familial cardiac myxoma identified as a genetic abnormality. The mother experienced multiple recurrence of tumors in the left atrium and left ventricle 40 months after surgical resection of a left atrial myxoma. All recurrent tumors were successfully resected. Her daughter also had a solitary myxoma in the left atrium, but she had no recurrence after the operation. Both patients had lentigines in their face but no endocrine abnormality. Molecular genetic analysis demonstrated involvement of a mutation in the PRKAR1α gene.

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Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by a leukocytoclastic vasculitis involving small vessels with the deposition of IgA immune complexes. The renal involvement is the major cause of morbidity and mortality in patients with HSP. We report here an adult patient with HSP nephritis (HSPN) accompanied by persistent proteinuria and progressive renal dysfunction despite conventional therapy. The patient was successfully treated with tonsillectomy followed by intravenous pulse methylprednisolone and oral prednisone. The combination therapy resulted in a significant decrease in proteinuria, improvement of renal function and the disappearance of microhematuria. The patient finally reached a stage of clinical remission.

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A 57-year-old man with occulocutaneous albinism was admitted to our hospital because of exertional dyspnea and an abnormal shadow on chest roentgenogram. Chest CT revealed diffuse interstitial shadows with reticulonodular opacities in the bilateral whole lung fields and his pulmonary function test was consistent with a restrictive finding. Histologically, intraluminal diffuse fibrosis and interstitial fibrosis existed and ceroid-like materials within alveolar macrophages were demonstrated in a transbronchial lung biopsy specimen. In addition, because platelet dysfunction and ceroid-like materials within the reticuloendothelial cells of urine and bone marrow aspiration were recognized, we made a diagnosis of Hermansky-Pudlak syndrome (HPS). Gene analysis of the patient’s peripheral blood cells did not reveal that he was a compound homogeneity for HPS1 gene mutations. Concerning treatment, although corticosteroid therapy was administered, his clinical symptoms and abnormal chest shadow have not changed.

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A 61-year-old woman was admitted with fever and headache of 10-day duration. She was found to have anemia, jaundice, and signs of meningitis. The erythrocyte sedimentation rate was increased and the tuberculin skin test was positive. A provisional diagnosis of miliary tuberculosis was made and antituberculous therapy was started, although no miliary lesions were seen on chest radiography. However, her condition rapidly deteriorated with diffuse opacification of both lungs and she died on the 7th hospital day. Postmortem examination revealed miliary tuberculosis in several organs but not in the lungs with acute respiratory distress syndrome accounting for the lung pathology. It should be noted that on rare occasions the lungs may not be involved by miliary tuberculosis.

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Rounded atelectasis is an unusual form of lung collapse that develops as a consequence of pleural diseases. Among a variety of conditions, asbestos inhalation has been attributed in most cases, but many other causes have also been implicated. Here, we describe the first case of rounded atelectasis and pneumothorax associated with pulmonary lymphagioleiomyomatosis.

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Two cases of pleural involvement of dialysis-related amyloidosis (DRA) with pleural effusion are presented. DRA is one of the most important complications in long-term dialysis patients and β₂-microglobulin is the principal protein component of DRA, but pleural deposition of β₂-microglobulin amyloid has not yet been reported. To the best of the authors’ knowledge, this is the first case report of pleural involvement of DRA presenting with pleural effusion. This case study suggests that pleural involvement of DRA should be considered when exudative pleural effusion is observed in patients undergoing long-term dialysis.

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Two cases of primary pulmonary osteosarcoma are presented. In both cases, chest computed tomography revealed a calcified pulmonary mass and technetium-99m methylene diphosphonate bone scintigraphy showed intense uptake in the pulmonary mass. Primary pulmonary osteosarcoma was suspected on the basis of these radiographic findings. Microscopic examination of tumor specimens obtained by needle biopsy revealed histologic features of osteosarcoma, and this diagnosis was confirmed by postmortem examination of a second specimen in each case. Radiographic and histopathological findings enabled us to diagnose primary pulmonary osteosarcoma, which is one of the rarest types of cancer.

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A 30-year-old man with bronchial asthma complained of horizontal diplopia. Partial oculomotor nerve palsy with restrictions of elevation and adduction, and mydriasis was observed in the left eye. Cranial magnetic resonance imaging demonstrated an infarct lesion in the territory of the left superior median mesencephalic branch of the posterior cerebral artery. Based on bronchial asthma, hypereosinophilia, mononeuropathy multiplex, pulmonary eosinophilia and positive perinuclear antineutrophil cytoplasmic antibody in the serum, the patient was diagnosed as having Churg-Strauss syndrome. This is the first case of oculomotor nerve palsy due to midbrain infarction associated with Churg-Strauss syndrome.

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An 85-year-old woman receiving anticoagulant therapy for transient ischemic attack suddenly developed bilateral femoral nerve palsy and severe pain in the bilateral groin and thighs. Her platelet count, prothrombin time and activated partial thromboplastin time were within the therapeutic range. Hematomas in the bilateral iliopsoas muscles were clearly detectable on CT scan. The right hematoma was larger than the left one and caused more severe femoral neuropathy, but improved gradually without surgical decompression. This case is reported here because bilateralism is exceptional, and iliopsoas hematoma should be suspected when a patient receiving anticoagulant therapy presents with pain in the groin or thigh.

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A 41-year-old man was diagnosed as having primary parotid carcinoma on the right side. After radical parotidectomy, radiation therapy and systemic chemotherapy, the primary parotid carcinoma was completely remitted. Two years later, right abducens nerve palsy and Horner syndrome appeared. Neuroimaging demonstrated a gadolinium-enhanced lesion in the posterior portion of the right cavernous sinus, and metastasis of parotid carcinoma was suspected. After radiosurgery and systemic chemotherapy, the intracavernous lesion disappeared. This is the first case of combination of abducens nerve palsy and ipsilateral Horner syndrome due to metastasis from parotid carcinoma to the cavernous sinus.

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We describe a 53-year-old man with the isolated manifestation of girdle-like tightening sensation of the trunk due to polyradiculopathy at the beginning of sarcoidosis which was first misapprehended as abdominal splanchnopathy. Late development of other neurological and systemic symptoms led to the final diagnosis of sarcoidosis. Segmental dysesthesia at the trunk in neurosarcoidosis is unique and may mimic a splanchnic pain. Such a dysesthesia may be solely manifested at the beginning of sarcoidosis and may continue for days without other symptoms. When patients complain of a girdle-like tightening with unknown etiology, sarcoidosis should be suspected as the possible cause.

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Lemierre syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein and septic metastasis to various distant organs, most often the lungs. Here we describe previously unreported clinical features of cerebral infarctions and brain abscess due to metastatic septic embolism of this uncommon but lethal syndrome, and the successful course of treatment with ampicillin, cefotaxime and metronidazole.

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A 74-year-old woman with Sjögren’s syndrome and chronic hepatitis C (CHC) was admitted to our hospital in October 2003 for treatment of diabetes mellitus. She had the past history of recurrent thrombocytopenia, which was proven to be due to peripheral destruction. Although she had been diagnosed with hypertrophic cardiomyopathy (HCM) for 2 years, she had never felt palpitation. She suddenly died probably of fatal arrhythmia related to HCM during the last hospitalization. Although hepatitis C virus (HCV) infection has been associated with Sjögren’s syndrome, thrombocytopenia, HCM, and diabetes mellitus, all these diseases rarely occur in a single patient. It will be necessary to identify similar cases to elucidate the etiopathogenesis of extra-hepatic manifestations of HCV infection.

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A 79-year-old man was admitted to hospital from his nursing home for treatment of pneumonia, but died 7 days after admission. Legionella pneumonia was diagnosed after isolation of Legionella pneumophila serogroup-5 from sputum culture. The environment of the nursing home was investigated; only water specimens from the 24-hour bath were positive by culture for Legionella pneumophila serogroup-5. Subsequent analysis by pulsed-field gel electrophoresis revealed an identical pattern in isolates from both sputum culture and 24 hour bath water culture. Among 123 inpatients and staff of the nursing home, 17 were found to be seropositive for Legionella pneumophila serogroup-5.

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A diabetic patient was admitted to our hospital for infective endocarditis with acute purulent pericarditis and diabetic ketoacidosis. Echocardiography revealed attachment of vegetation to the chordae tendineae in the left ventricle and pericaridial effusion. The vegetation was enlarged and pendulated for a few days despite maximal antimicrobial therapy. Surgical resection was desirable to decrease the risk of embolic complications and cardiovascular collapse. We could not open the heart because of accumulation of purulent pericardial fluid, and right renal infarction was complicated. We believe that the immunocompromised and hypercoagulable state due to diabetes caused these conditions.

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