Background and Aim The incidence of ischemic colitis (IC) in Japan has been increasing due to the westernization of diet and the aging population. The aim of this study was to evaluate the relationship between endoscopic findings and clinical severity in IC.
Methods This retrospective analysis included 106 cases diagnosed with IC that were divided into two groups based on endoscopic findings in the acute stage: redness and erosion (RE) versus longitudinal and circumferential ulcers (LCU). The clinical variables were compared between the two groups. In addition, we investigated the risk factors of IC associated with the severity of the endoscopic findings by multivariate logistic regression analysis.
Results The percentage of cases presenting abdominal pain was significantly higher in the LCU group than that in the RE group (p=0.002), as were the baseline serum CRP levels (p=0.0001). The periods of hospitalization in LCU group were longer than in the RE group (p=0.0001). Multivariate logistic regression analysis indicated that ischemic heart disease (IHD) and connective tissue disease were the independent explanatory factor associated with the endoscopic severity of IC (p<0.05).
Conclusion We showed clearly that the two endoscopic classifications were accurate indicators of severity and could be used to anticipate severity of IC. Furthermore, we confirmed that IHD and connective tissue disease were the exacerbating factor associated with the severity of endoscopic findings in IC.

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Objective To clarify the clinical characteristics of patients diagnosed with chronic gastritis in Japan, a large scale clinical survey of patients with chronic upper gastrointestinal symptoms was conducted. Patients diagnosed with functional dyspepsia (FD) according to the ROME III criteria were selected from among patients with chronic gastritis and the clinical characteristics of patients with FD and those with chronic gastritis were compared.
Patients and Methods Patients with upper abdominal symptoms and diagnosed with chronic gastritis were enrolled in the study. Their main complaints, duration of symptoms, clinical characteristics, mental state, and results of endoscopic examinations, if available, were prospectively surveyed. Quality of life (QOL) impairment caused by abdominal symptoms was also surveyed using the Izumo QOL scale.
Results A total of 9,125 patients with a clinical diagnosis of chronic gastritis were enrolled in the survey. Of those, approximately 60% had more than 2 symptoms and QOL impairment was greater in cases with multiple symptoms. Endoscopic examinations were performed in 2,946 cases (32.3% of enrolled patients), during which gastric and/or esophageal carcinoma was found in only 0.2%, though organic diseases were found in 6.2%. Endoscopic examinations were not done for patients with high risks of organic diseases. Of patients with organic diseases excluded by an endoscopic examination, only 362 (12.3% of patients who underwent an endoscopy) were diagnosed with FD according to the ROME III criteria, mainly because of short symptom duration. There were no remarkable differences in regard to clinical characteristics, including symptoms and mental state, between patients with chronic gastritis and those with FD.
Conclusion Clinical characteristics of patients with chronic gastritis were similar to those with FD, except for shorter symptom duration.

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Objective Physicians have provided care to only 0.2 million of the 5.3 million Japanese over the age of 40 years old who have chronic obstructive pulmonary disease (COPD). Among such individuals, many patients with respiratory symptoms diagnosed as chronic bronchitis (CB) are prescribed mainly expectorants. To determine the current status of COPD subjects diagnosed with and treated for CB, we investigated the prevalence of airflow limitation (AFL) in CB patients diagnosed by general practitioners (GPs) and the therapies administered to them.
Methods Patients receiving treatment by GPs as CB completed a questionnaire and the FEV₁/FEV₆ ratio was measured by their GPs with a Piko-6. The prevalence of AFL (FEV₁/FEV₆ <73%) and the correlation between FEV₁/FEV₆ and FEV₁/FVC were examined. Prescription behavior and comorbid lifestyle diseases were also examined.
Results Data from 197 patients with CB were analyzed. Among those who underwent spirometry, the correlation between FEV₁/FVC and FEV₁/FEV₆ was r²=0.38 (p<0.0001), and the sensitivity and specificity of the Piko-6 were 85.7% and 61.1%, respectively. The prevalence of AFL was 47.2% and increased to 54.1% among patients aged 70-79 years. Expectorants were prescribed for 39.8% of CB patients with AFL, but inhaled bronchodilators were prescribed for only 22.6%. Smoking history and age were significantly higher in the group with AFL than in those without AFL (p<0.05). The prevalence of comorbid lifestyle diseases was 73.1% in patients with AFL.
Conclusion AFL was prevalent among patients with CB. Therefore, GPs should test pulmonary function in CB patients to ensure that the appropriate therapy is administered.

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Objective Renal dysfunction may be related to cerebrovascular disease. The aim of this study was to assess the relationship between mild renal dysfunction and carotid artery atherosclerosis detected by ultrasonography in apparently healthy subjects.
Methods A total of 2,106 persons (1,368 men and 738 women, mean age+/-S.D.: 56 +/- 10 years) with no history of stroke were enrolled. Kidney function was evaluated in terms of estimated glomerular filtration rate (eGFR), calculated by using the relationship 194Cr^-1.094×Age^-0.287×0.739 (if female), where Cr is serum creatinine concentration. Atherosclerosis on ultrasonography was defined as regional intimal thickening or nodular lesion.
Results Atherosclerotic lesions were significantly more frequent in subjects with CKD stage 3 than in CKD stage 1 or 2 (p<0.001). Odds ratios for atherosclerotic lesions were significantly increased to 1.11 (95% confidence interval: 1.09-1.12, p<0.001) for increasing age, 1.66 (1.31-2.10, p<0.001) for male sex, 1.76 (1.43-2.16, p<0.001) for systolic blood pressure ≥130 mmHg and/or diastolic blood pressure ≥85 mmHg, 1.61 (1.28-2.01, p<0.001) for LDL-cholesterol ≥140 mg/dL, and 1.59 (1.23-2.05, p=0.003) for smoking habit versus no risk factor. The odds ratio of CKD stage 3 for ≥50% carotid artery stenosis was significantly increased to 3.47 (1.09-11.08, p=0.035), although CKD stage 2 and stage 3 were not significant (0.77, 95% CI: 0.59-1.01, p=0.068; 0.99, 95%CI: 0.67-1.46, p=0.981, respectively).
Conclusion Renal dysfunction defined in terms of eGFR might be associated with early-stage carotid atherosclerosis, but traditional vascular risk factors, including increasing age or hypertension, appear to play a major role.

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Objective We clarified the prevalence of cholelithiasis/cholecystitis in long-term tracheostomy invasive ventilation (TIV) patients in our hospital.
Methods We investigated the occurrence of cholelithiasis/cholecystitis in twenty-seven TIV patients over the past eight years.
Results There were ten patients with cholelithiasis/cholecystitis. The underlying disease was amyotrophic lateral sclerosis (ALS), except for 1 patient. Mean age was 56.2 ± 4.7 years and the average period to the attack of cholelithiasis/cholecystitis from TIV was 57.6 ± 15.1 months. Two patients of acute cholecystitis had acalculous cholecystitis. Five of the ten patients developed a severe state of cholecystitis and needed aggressive therapy.
Conclusion The prevalence of gallstones, symptom prevalence of asymptomatic cholelithiasis, the percentage of acalculous cholecystitis of acute cholecystitis in TIV patients was higher than in the general population. Therefore, it may be possible that TIV is a risk for cholelithiasis/cholecystitis. Some of the ALS patients with long-term TIV develop severe cholelithiasis/cholecystitis, and those facts should be considered in our daily clinical practice.

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Background Plasma ammonia has been used in emergency departments to assess whether or not generalized convulsion attacks exist in patients who are suspected of having convulsions. However, there are few reports that have assessed the relationship between generalized convulsions and hyperammonemia. The clinical significance of plasma ammonia measurements in the diagnosis of generalized convulsions is investigated in this study.
Objective A total of 293 patients who were transported by ambulance to the emergency department of St. Luke's International Hospital, Tokyo, Japan under suspicion of convulsive seizure or disturbance of consciousness were studied.
Methods The objectives were divided into two groups -"Convulsion" and "Non-convulsion"- according to the information provided by witnesses. Bivariate and multivariate analyses were carried out for patient background, clinical course, past medical history and blood test results.
Results All 11 items showing significant differences on the bivariate analysis were included in the multivariate analysis. Of these, age, total Glasgow Coma Scale score, plasma ammonia level and arterial lactate level showed a significant difference and are recognized as independent findings for the diagnosis of generalized convulsion. The plasma ammonia level had an odds ratio of 14.8 (95% CI, 3.2 to 111.5; p<0.01), 53% sensitivity and 90% specificity when 65 μg/dL was used as the cut-off value.
Conclusion Plasma ammonia values rise during generalized convulsion. Measurement of plasma ammonia is clinically highly significant as an independent finding during the diagnosis of generalized convulsion.

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Objective To clarify the clinical features and mechanism of the transience of myoclonus in patients with a transient myoclonic state with asterixis (TMA).
Methods We investigated the clinical and eletrophysiological profiles of 6 patients with TMA (age: 84±3 years). During an asymptomatic period, somatosensory evoked potentials (SEPs) were recorded in all 6 patients and motor evoked potentials (MEPs) were examined in 1 patient. SEPs were recorded and jerk-locked back averaging (JLA) was performed in 2 patients while symptomatic. SEPs were also recorded from 8 aged control subjects (age: 68±5 years).
Results All TMA patients had mild chronic systemic diseases. During an asymptomatic period, SEP amplitudes were not significantly enlarged in comparison with control subjects, and MEPs were normal. Examination of 2 patients during symptomatic period indicated no enlargement of SEP amplitudes and JLA disclosed a positive spike preceding myoclonic jerks. In one of these patients, the amplitude of the positive spike decreased once myoclonus improved.
Conclusion TMA occurred in aged patients with mild chronic systemic diseases. JLA findings and the absence of giant SEPs further support that TMA is a cortical non-reflex myoclonus. In addition, transient hyperexcitability at the primary motor cortex disclosed by JLA correlated well with its transient symptoms.

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Objective Patients with Parkinson's disease (PD) frequently complain of a diminished sense of smell. Less frequently, they may complain of taste impairment. In the present study, we investigated the symptoms, frequency, and severity of taste impairment as well as smell impairment in PD patients and compared the results with those of age- and sex-matched healthy controls.
Patients and Methods We interviewed 285 PD patients (120 men, 165 women) without dementia or nasal problems. Control subjects comprised 61 (20 men, 41 women) healthy spouses of the PD patients. Alteration of smell and taste sense lasting more than 3 months was defined as abnormal.
Results One hundred and sixteen patients with PD complained of smell impairment and 26 complained of taste impairment. Only 5 controls complained of smell impairment, and no control subjects reported taste impairment. Taste impairment was more marked in patients with smell impairment. Impaired taste included diminished taste perception in 21 patients, altered sense of taste in 4 patients and burning mouth in 1 patient.
Conclusion Taste as well as smell perception is impaired in patients with PD. The frequency of smell and taste impairments tended to increase with disease progression.

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A hepatic tumor was found in a 57-year-old man with glycogen storage disease type 1a (GSD1a) with a mutation in exon 5 of the glucose-6-phosphatase gene (G727T). Partial hepatectomy was performed, and the tumor was histologically diagnosed as moderately differentiated hepatocellular carcinoma (HCC). On contrast-enhanced ultrasonography, the tumor had a late phase defect. Abdominal imaging with other modalities was also performed. More studies are needed to clarify the differences in imaging findings between GSD1a-associated HCC and other tumors such as adenomas.

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We report a patient who developed ulcerative colitis during the course of primary biliary cirrhosis (PBC). PBC was diagnosed by liver histology when the patient was 31 years old, at which time no symptoms were observed. At 45 years of age, the patient complained of bloody diarrhea and abdominal pain, and she was diagnosed with ulcerative colitis by colonoscopy and histological examination. The patient had HLA-DRB1^*1502 and ^*0802, which are associated with both diseases. Ulcerative colitis should be considered in the differential diagnosis of bowel disease in patients with PBC. The possible genetic associations between 2 diseases are also discussed.

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A 43-year-old man was admitted to our hospital with right hypochondriac and epigastric pain. An abdominal radiograph showed a large niveau in the right subphrenic space. An abdominal CT scan demonstrated a large liver abscess (diameter, 13 cm) with gas formation. Klebsiella pneumoniae cells were isolated from the abscess, and the patient was treated with antibiotics and percutaneous drainage. It is very important to treat gas-forming liver abscess immediately, because subsequent bacteremia and septic shock are frequently noted, and the associated mortality rate is high.

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Megaesophagus resulting from achalasia is a rare but serious cause of acute airway obstruction. We treated achalasia in a 52-year-old woman with acute respiratory distress and stridor. Chest X-ray and endoscopy showed a marked dilatation of the cervical esophagus with a large amount of undigested food. Emergency suction of the food through a nasogastric tube led to decompression of the esophagus and the immediate relief of respiratory symptoms. These findings suggest a dysfunction of the upper esophageal sphincter as a possible mechanism. As this exceptional complication of achalasia is fatal, a wider appreciation is required.

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A 74-year-old man without history of ventricular arrhythmias underwent coronary bypass surgery for 3-vessel disease. On the 4th postoperative day, he developed ventricular fibrillation (VF). His monitored ECG showed no elevation of the ST-segment and no prolongation of QT interval, but evolving J waves prior to VF were shown. These J waves gradually decreased after defibrillation. The subsequent angiography revealed patent grafts and normal left ventricular function. J waves reappeared in inferior leads when contrast medium was injected into the coronary artery. Therefore, evolving J wave can be a marker of latent ischemia and a predictor of VF.

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Severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is referred to as 'disproportionate' because the elevated pulmonary artery pressure does not match the degree of air flow limitation. We report a 41-year-old man presenting with early-onset pulmonary emphysema and pulmonary hypertension with a mean pressure of 74 mmHg. Continuous intravenous epoprostenol led to marked hemodynamic improvement, and epoprostenol was successfully replaced with bosentan. The patient has been followed for 3 years without exacerbation. This is the first report demonstrating the long-term efficacy of specific drugs for pulmonary arterial hypertension in disproportionate pulmonary hypertension in COPD.

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A 51-year-old woman with adenomyosis was admitted because of anemia with schistocytosis, thrombocytopenia, and acute renal failure (ARF). Thrombotic microangiopathy (TMA) was considered. Plasma exchange and steroid therapies improved laboratory results. However, renal biopsy specimen revealed acute tubular necrosis (ATN), but not TMA, and thrombocytopenia, diagnosed it as disseminated intravascular coagulation (DIC) but not TMA. Few cases of DIC associated with benign tumors of the uterus and, especially, adenomyosis have been reported. In adenomyosis patients, ARF is usually caused by obstructive uropathy. However, the rare case suggests that hemolytic anemia, DIC, and ARF due to ATN can occur in adenomyosis patients.

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Idiopathic basal ganglia calcification (IBGC) is a syndrome in which bilateral cerebral calcification occurs despite the absence of abnormal calcium metabolism. A 17-year-old Japanese female was admitted for investigation of intermittent proteinuria from the age of 12 years. On admission, her blood pressure was 126/60 mmHg and her serum creatinine was 0.8 mg/dL. Although computed tomography revealed bilateral striopallidodentate calcinosis, her level of intelligence and neurological findings were normal, as were the results of endocrine tests including parathyroid hormone. Asymptomatic IBGC was diagnosed. Renal biopsy showed membranoproliferative glomerulonephritis. Peritoneal dialysis was started for end-stage renal failure when she was 24 years old. Pyramidal and extrapyramidal signs started to develop at the age of 27 years and progressed, resulting in death from aspiration pneumonia at the age of 32 years. Post-mortem revealed bilateral calcification of the basal ganglia, dentate nucleus, thalamus, and centrum semiovale. On light microscopy, there was circumferential calcification of the media and intima of affected vessels in the brain, including small arteries, small veins, and capillaries, and luminal narrowing was seen. On electron microscopy, layers of differing electron density were arranged in concentric laminae. This is the first report of IBGC with bilateral and symmetrical cerebral calcification accompanied by membranoproliferative glomerulonephritis resulting in end-stage renal failure.

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A 53-year-old woman with systemic lupus erythematosus presented with a 3-day history of fever and coughing. Diagnosis of pneumococcal bronchitis was made based on symptoms and positivity of pneumococcal urinary antigen test. On day 3, severe low back pain acutely occurred. Pneumococcal vertebral osteomyelitis and psoas abscess was diagnosed 17 days later by yield of penicillin-susceptible S. pneumoniae strain in blood cultures and drainage fluid. Although pneumococcal urinary antigen test is a useful tool for the diagnosis of pneumococcal pneumonia, we should consider the possibility of pneumococcal infections other than pneumonia or overwhelming bacteremia in immunosuppressive patients when urinary antigen test is positive.

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Micropapillary carcinoma is known to be associated with a poor prognosis and high propensity for lymphovascular invasion and lymph node metastasis. Case reports on colorectal micropapillary carcinoma are relatively rare. We report here a 26-year-old woman who had sigmoid colon cancer with a micropapillary component. We made the diagnosis of pulmonary lymphangitic carcinomatosis but could not identify the primary lesion. We gave her chemotherapy as an occult primary cancer. But her respiratory condition did not improve and she died of respiratory failure. Autopsy was performed after her death. The final diagnosis was pulmonary lymphangitic carcinomatosis and multiple lymph node metastases of sigmoid colon cancer with a component of micropapillary carcinoma.

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We encountered a case of limited-disease small cell lung cancer with episodic syncope. The frequency of the syncopal attacks increased with the increase in the tumor size, thus a relationship was suspected to exist between the SCLC and syncope. Syncope was evaluated by history taking, 24-hour ECG monitoring, and coronary angiography. As orthostatic hypotension and cardiac disease could be excluded, we finally diagnosed this case as neurally mediated syncope. Serum tests for anti-Hu and anti-Yo antibodies were negative. A temporary pacemaker was inserted for sick sinus syndrome. This patient showed good response to the chemotherapy. No further syncopal attacks were observed after the second course of chemotherapy. Here, in addition we review four cases of SCLC with episodic syncope. Interestingly, in all cases, the tumor was located in the left hilum in close vicinity of the afferent vagal nerve (C-fibers) and mechano-receptor. Therefore, we thought that the mechanism underlying the syncope was mechano-receptor hypersensitivity.

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A 70-year-old woman was admitted to our hospital with pleuritis and pericarditis. Cytological examination of pleural and pericardial effusion, and pleural biopsy specimens under thoracoscopy revealed no specific pathological findings. The pleural effusion was drained continuously; however, she died of circulatory insufficiency at day 45 from admission. At autopsy, a fragile hemorrhagic mass arising from the right auricle had invaded bilateral pleura and the pericardium directly without distant metastasis. Immunohistochemical staining showed that the tumor cells expressed endothelial markers such as CD31 and CD34 antibodies, and factor VIII-related protein. These findings supported the diagnosis of a poorly differentiated angiosarcoma.

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Chylothorax, the accumulation of fatty fluid within the chest cavity, is associated with multiple etiologies including surgical injuries. A rare complication of acupuncture in a 37-year-old woman who developed left pneumothorax and pleural fluid collection after acupuncture was performed on the neck and upper back is described. Chest tube drainage resulted in complete lung expansion, and analysis of the milky fluid revealed chyle leakage. Conservative treatment with a diet low in lipids and rich in medium-chain triacylglycerols allowed extubation. Acupuncture-induced thoracic duct injury, although extremely rare, should be considered as a cause of chylothorax.

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A 41-year-old man with hairy cell leukemia developed erythroid crisis after the transfusion of red cell concentrate. He was diagnosed with Parvovirus B19 infection based upon the presence of B19-specific IgM antibody and viral DNA in the sera. The repository sample from the corresponding red cell concentrate was negative for B19 antigen by red cell hemo-agglutination method, but was found to contain B19 virus DNA. Furthermore, a genomic PCR direct sequencing showed that B19 in both patient's sera and repository sample were identical. This is the first report directly demonstrating the transmission of B19 through B19 antigen-negative red cell concentrate transfusion. Further accumulation of the cases is warranted to estimate its incidence and to reconsider the screening methods of blood products.

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A 44-year-old woman was admitted with generalized lymphadenopathy, which was diagnosed as angioimmunoblastic T-cell lymphoma (AITL). The patient showed autoimmune hemolytic anemia (AIHA), polyclonal hypergammaglobulinemia and a high antinuclear antibody titer. Moreover, a human immunodeficiency virus (HIV)-1/2 screening test using the particle agglutination method was reactive. After chemotherapy for AITL, the AIHA was eliminated, and the false-positive HIV results were no longer detected. Autoimmunity associated with AITL is the likely cause of the cross-reaction with HIV and the AIHA. It is important to recognize that the cross-reaction with HIV can be a potential complication in AITL as well as AIHA.

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A 31-year-old pregnant woman was diagnosed with chronic-phase chronic myeloid leukemia at gestational week 16. To avoid exposure of the fetus to teratogenic agents, the patient opted for a course of careful observation only for the duration of her pregnancy. We detected 9.1% of BCR-ABL-positive cells in the patient's colostrum with fluorescence in situ hybridization.

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The distribution of adult T-cell leukemia/lymphoma (ATLL) is typically systemic. In addition to peripheral blood (PB) and lymph nodes, extranodal sites such as the skin, lung, liver, gastrointestinal tract, and central nervous system are frequently involved. We report a unique case of ATLL in which the patient presented with prolonged fever. A 65-year-old man had high-grade fever lasting for 2 weeks. He showed no lymphadenopathy, hepatosplenomegaly, skin lesions, or PB involvement. Bone marrow examination showed widespread infiltration of ATLL cells. ¹⁸F-fluorodeoxy glucose positron emission tomography (FDG-PET) revealed that the disease was confined to the bone marrow.

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A 60-year-old woman with a history of symptomatic seizures secondary to a subarachnoid hemorrhage was admitted to hospital because of a generalized seizure. The following day, her electrocardiogram showed negative T waves in II, III, _aV_F, and V_2-6, and the echocardiogram showed an impaired left ventricular ejection fraction with ventricular apical akinesia. Head magnetic resonance imaging showed no acute brain injury, but single photon emission computed tomography (SPECT) showed hyperperfusion which affected the left temporal cortex in particular. Hyperactivity of the temporal lobe might cause autonomic nervous system dysfunction and might be related to takotsubo cardiomyopathy.

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A 70-year-old man presented with sudden onset of global aphasia and right hemiplegia. Brain MRI revealed occlusion of the left middle cerebral artery. He was diagnosed as having a hyperacute cerebral infarction. Intravenous thrombolytic therapy was started, and the neurological symptoms were resolved after 11 h. Echocardiography showed a mobile mass in the left atrium, suspicious of a myxoma. The tumor was resected and pathologically diagnosed as a myxoma. In this patient, intravenous thrombolytic therapy was effective, and no adverse effects were observed. This suggests that, even with complications of myxoma, thrombolytic therapy can be considered if there are no contraindications.

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A 36-year-old man with a 21-year history of Crohn's disease suddenly developed left hemiparesis. He did not have atherosclerotic risk factors on admission, but he had marked dehydration which was likely due to prolonged home intravenous hyper-alimentation. Brain MRI revealed lacunar infarction in the right anterior corona radiata. An anticoagulation drug and a free-oxide scavenger successfully reversed his neurological deficits almost completely. Stroke in young adults less than 40 years old is extremely rare; therefore, we conclude that Crohn's disease can be a risk factor for acute ischemic stroke in our case, due most probably to dehydration and other complex mechanisms.

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We report genetically confirmed heterozygote oculopharyngeal muscular dystrophy (OPMD) accompanied by dementia, suggesting a possible causal association between OPMD and dementia. The proband first noticed bilateral ptosis, dysphagia, and proximal dominant muscle weakness in the lower extremities at age 53. Ten years later, she was found to have dementia with a score of 10/30 on the mini-mental state examination (MMSE). On PABPN1 gene analysis, the GCN repeat was expanded 17 times in one allele. In addition, the proband's younger brother exhibited myopathy and dementia. To our knowledge, this is the first report of genetically confirmed heterozygote OPMD associated with dementia.

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We present the first documented case of thrombotic thrombocytopenic purpura (TTP) with severe hypertension complicated by polymyositis and systemic sclerosis sine scleroderma. TTP developed in the progressive phase of visceral fibrosis in the absence of skin thickening. ADAMTS13 activity was not useful for the diagnosis of TTP. Although TTP and scleroderma renal crisis (SRC) share similar findings of thrombotic microangiopathy, severe thrombocytopenia with multiple organ injuries and hemorrhagic manifestations suggested TTP rather than SRC. The patient's condition improved dramatically with plasmapheresis.

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Hypereosinophilic syndrome (HES) encompasses both myeloproliferative and lymphoproliferative diseases. We encountered a rare case of lymphocytic HES followed by malignant T cell lymphoma, who was diagnosed as eosinophilic pneumonia upon the first visit. During the clinical course, the transition of the chest CT findings from bilateral multifocal ground-glass opacities and consolidations to bilateral scattered multiple small nodules was impressive and suggestive. Given the increased risk of developing T-cell lymphoma, patients with HES (especially lymphocytic-HES) should be monitored on a regular basis to detect this complication as early as possible.

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We report a case of pathologically confirmed malignant syphilis in an HIV-infected patient. Physical examinations revealed ulceronecrotic skin lesions. Skin biopsies demonstrated syphilis spirochetes on immunohistochemical stain, and syphilis serological titers were positive. Treatment with intravenous penicillin G was begun, and complete resolution of the skin lesions was observed. A rapid plasma reagin titer test performed 3 months after treatment revealed a 4-fold reduction in the titer, indicating successful treatment.

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Guillain-Barré syndrome sometimes manifests as immune reconstitution inflammatory syndrome. We report a treatment-naïve male with chronic HIV-1 infection who presented with an axonal variant of Guillain-Barré syndrome. Antiretroviral therapy commenced one month later and no rapid improvement or deterioration of tetraparesis was noted. This is the first report that describes the detection and serial measurements of anti-ganglioside antibody in a patient with HIV infection. This case suggests a limited role for T-cell immunity in the production of anti-ganglioside antibody and the pathogenesis of axonal variants, since the antiretroviral therapy-induced improvement in T-cell immunity neither re-elevated anti-ganglioside antibody titer nor worsened tetraparesis.

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